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Child and Adolescent
Psychiatry
Editedby

Michael Rutter
CBE,MD, FRCP,    FRCPsych, FMedSci
                          FRS,
Professorof Developmental Psychopathology
Social, Genetic and Developmental Psychiatry ResearchCentre
Institute of Psychiatry
London


Eric Taylor
MA, MB, FRCP,   FRCPsych,
                        FMedSci
Professorof Child and AdolescentPsychiatry
Department of Child and AdolescentPsychiatry
Institute of Psychiatry
London


FOURTH     EDITION




Blackwell
Science
r9
                                                                                                                                                 --




                                      Speechand LanguageDifficulties
 I,;) .-,                             Dorothy ~M. Bishop




 Introduction                                                                 so that the bulk of phonology and syntax is acquired by around
                                                                              4 years of age. There are, however, frequent exceptions, and
   The ability to communicate through language distinguishes                  theseare the topic of this chapter.
   humans from all other animals. Spoken language allows us to                  Delay in learning to talk is a common reason for a parent to
  convey information, expressour feelings and demonstrate so-                seek advice from a .family doctor or paediatrician. Because
  cial affiliations. It also provides a vehicle for organizing our           human communication is complex, assessment         and diagnosis
  thoughts and memories,enabling us to construct complex lines               of speechand languagedifficulties in children is a particularly
  of reasoning, and to contemplate past, future and hypothetical             challenging problem that requires expertise in several differ-
  events,rather than remaining grounded in present reality. The              ent areas, including linguistics, audiology, child development,
  developmentof written languageprovides evenmore dramatic                   neuropsychology, paediatric neurology and psychiatry. This
  releasefrom the here-and-now,making it possible to transcend               chapter will usethe diagnostic flow chart shown in Fig. 39.1 to
  spaceand time.                                                             introduce a range of different conditions that can lead to speech
     All known human cultures have language,but there is huge                and languagedifficulties in children. This depicts the sequence
  diversity in how languagesare structured, both in terms of the             of decisions the clinician needsto make when first assessing    a
  sounds used to expressmeaning (phonemes)and the ways in                   child who presentswith poor communication. However, recent
  which linguistic elementsare combined (syntax). For instance,             researchsuggests    that this diagnostic processshould not becon-
  Frenchhastwo different vowels that sound like '00' to a speaker           fined to thosecases   wherecommunicative impairment is the pre-
  of English, but which arephonemically distinct; they signal con-          senting complaint, but should be extendedmuch more broadly
  trasts in meaning, so that 'rue' and 'roux' mean different things.        to all children referred to psychiatric services.
                                                                                                                            The reasonis sim-
  In English, on the other hand, we make a phonemic contrast be-            pie: surveysof children attending psychiatric clinics revealthat a
 tween the sounds 'th' and 'z' (e.g. 'bathe' vs. 'baize'), which are        high proportion of them have somekind of communicative im-
 not distinguished in French.In tone languages,such as Chinese              pairment, and in many cases goesunrecognizedunlessa for-
                                                                                                            this
 Mandarin, the pitch at which a word is spoken signalsmeaning,              mal assessment made. Cohen (1996) summarized findings
                                                                                               is
 so that 'ba' has four completely different meanings depending              from a Canadian study in which 399 consecutivepsychiatric re-
 on whether the pitch is rising ('to uproot'), falling ('a harrow'),        ferrals of children agedfrom 4 to 12 yearswere given a detailed
 changing from fall to rise ('to hold') or at a level high pitch            language assessment.     Children with autistic disorder, general
 ('eight')."                                                                developmentaldelay,neurolog.icaldamage,hearing impairment
    Moving to grammar; in English, relationships between enti-              or a non-English-speakinghome background had beenexclud-
 ties are indicated by a mixture of word order and grammatical              ed from this sample. Around one-quarter of the children had
 morphemes(e.g. inflectional endingssuch as '-ing' or '-ed', and            previously identified languageimpairments. Of the remainder,
 small function words such as 'by'). Thus it is the boy who is             none of whom was thought to be language-impaired,34% met
 doing the kissing in 'the boy kissesthe girl', but is the recipient of    criteria for language impairment. There appeared to be two
 the kiss in 'the girl kissesthe boy' or 'the boy is kissedby the girl'.   reasonswhy communicative difficulties had been overlooked
In somelanguages,suchasTurkish, word order generally obeys                 in thesechildren. First, they did not have such overt expressive
the sequencesubject-object-verb, and inflectional suffixes do              language difficulties as children with previously identified
all the work of expressingrelationships. Other languages,such              problems, although their receptivelanguageskills were aspoor
as ChineseMandarin, havevirtually no inflections. Word order,              as that group. Secondly,they were more likely to haveexternal-
particles and prepositions are usedto indicate how the elements            izing psychiatric disorders, which may have diverted attention
of a sentence    interrelate.                                              from communication. Cohen et al. (1998) suggested that
    Clearly, languageacquisition involves far more than learning           languagefunction should be incorporated routinely into the as-
labels for things. The child must work out which speechsound               sessmentand treatment process for children with psychiatric
contrasts are meaningful in the ambient lang~age,and how to                impairments. Somesuggestions how to implement this rec-
                                                                                                                for
combine words and grammatical morphemes to expressrela-                    ommendation are given below in the sectionon Assessment.
tionships betweenthings and events.Most children master this                   In the next section, different diagnostic entities will be re-
complex skill with no explicit instruction and with relative ease,         viewed, with a main focus on specificdevelopmental language

664



                                                                                                                                                 "~
~

                                                                                                                                       SPEECHAND lANGUAGE DIFFICULTIES

                                                           START

                                                  Islangu                                              Does
                                                                                                          child
                                                                 h
                                                                 age        .               Yes        produce                            Yes    Refer    to    speech    and   language

                                                  compre           enslon
                                                                                . t     ,
                                                                                                       .
                                                                                                       Immature
                                                                                                                          /d evlan t
                                                                                                                                .                th eraplst,.     query    expressive
                                                                                                                                                                                        .
                                                  age-approprla                       e.               utterances?                               language         disorder

                                                                                                                  No
                                                           No                                              Isspeech
                                                                                                           unintelligible or
     Language  impairmentsecondary      Yes       ~ens?r                                                   poorly
     to hearingloss                                earln                                                   articulated?
                                                  40 dB                                                              Yes
                                                                                                                 Any indication of
                                                                                                                       I .
                                                           No                                                    neuro oglcaI or                 Referto speechand language
                                                                                                                 ~tru~ural                       therapist, query developmental
      Referfor neurologicalopinion,     Yes       Any evi.denceof                                                Impairment?                     phonological disorder
            acquiredepileptic aphasia             re.9resslon
                                                            or                                                               No
      query                                       seizures?                                       No                 Are speech
                                                                                                       Yes           ~rrors.          Yes
                                                           No                                                        Inconsistent
                                                                                                                                and
                                                                                                                     worsewith longer     Refert? speechand language
                                                                                 ..                                  utterances                  therapist, query developmental
      GobaI deveopmentaI deIay
       I        I                        No       well above ability
                                                  Non-verbal                                                        ..                           verbaldyspraxia
                                                         h .                                                 Dysarthria/anarthria
                                                  compre enSlon
                                                  level?
                                                           Yes

      Detailedevaluationto consider     Yes     Impairmentsin                                          Ischild m.ute                     Yes             Selectivemutism
      autisticdisorder/ PDDNOS                  non-verbalsocial                                       exceptwIth close
                                                communication/play?                                    family/friends?
                                                                                                              No
                                                                                                       Isspeechfluent?                           Referto speechand language
                                                      '.                                                                                  No     therapist, query developmental
                                          Assessexpressive language.                                                                             fl      d'   d     t tt . ,
                                                                                                                                                                          {,

                                          considermixed receptive-                                                                                  uency Isor er ,s u erlng,
                                          expressive
                                                   languagedisorder
                                                                                                                    Yes
                                                                                                       Is voice quality                          Refer to otolaryngology, query
                                                                                                       normal?                            No     voice disorder


                                                                                                                    Yes
                                                                                                       Does child say                            Consider Asperger syndrome.
                                                                                                       things that are                   Yes     pragmatic language
                                                                                                       bizarre or                                impairment
                                                                                                       tangential?

    Fig.39.1Decision for diagnosing
                   tree           speech language
                                       and      disorders children.
                                                        in                                                           ..




    disorder.Assessment
                      procedures will briefly be reviewed in a                                    of this chapter, but it is worth noting here that assessment  can
    later section.                                                                                be difficult in children with major behavioural difficulties. It is
                                                                                                  tempting to assumethat the child can understand but is unco-
                                                                                                  operative, but it is at least as likely that the behavioural difficul-
    A decision tree for diagnosis                                                                 ties stem from fear and frustration in a child who comprehends
                                                                                                  very little. If the child doesnot co-operatewith formal compre-
    In Fig. 39.1, the question of whether comprehension is age-                                   hensiontesting, proceeddown the decision tree on the left-hand
    appropriate is placed at the top of the decision tree. There are                              side of the diagram.
    good reasonsfor this. First, whereasproblems with expressive                                     As shown in Fig. 39.1, results from a comprehensionassess-
    speech languageare usually fairly easyto detecton the basis
            and                                                                                   ment are not sufficient for a diagnosis,but they determinewhich
    of informal observation, comprehensionis much harder to esti-                                 diagnosesshould be considered, and also help the clinician to
    matethis way. Secondly,different diagnosesneedto be consid-                                   adjust his or her languagelevel to the child's level of understand-
    ered for the child with comprehension problems than for one                                   ing, for instance when conducting a psychiatric interview.
    whose problems are confined to speech output or sentence                                      Where comprehension is unimpaired, we can exclude autistic
    formulation.                                                                                  disorder and mental handicap. It is also unlikely that hearing
      Methods for testing comprehensionare discussedat the end                                    lossor acquired epileptic aphasiais implicated.


                                                                                                                                                                                            665
--

           CHAPTER                   39



           Hearing loss                                                                                                                                            loss.       It is often           cited      as a cause                of speech           and       language              difficul-

    ""                                                                                                                                                             ties, but recent                  research suggests    that the effect may have been
    !      The       first     diagnosis               to consider               in a child           with        comprehension                          dif-      overestimated                   in the past by relying     on clinical samples (Bishop

           ficulties          is hearing           loss. Note                that         in Fig.         39.1      evaluation                     of hear-        &      Edmundson                     1986;        Bishop               1988).         Epidemiological                       studies

           ing       comes           before        assessment                    of non-verbal                      ability.           It is all         too       have        found          only         weak       influences,                if any,        on      long-term             speech,

           easy       to assume                 that        if a child           has low             IQ      then      the language                      im-       language             and       literacy          outcomes               (Grievink             et at. 1993;                Peters       et

           pairment                has     been         explained.                   However,                impaired                 hearing            is a      at.     1994).        It is also             important                 to    be aware               just     how          common
           common              correlate               of many             syndromes                  that       affect         general             ~ntelli-       OME          is. A Dutch                  epidemiological                    study         of children               screened          at

           gence,            and     an audiological                       evaluation                 should          always               be under-               3-monthly              intervals                between           2 and             4 years          of age          found          that

           .taken       in a child              with        poor          understanding,                     regardless                 ofIQ          level        55%          of    children               had     at      least        one      episode             of     bilateral           OME

           Furthermore,                    one should                    beware           of relying              on hearing                 tests      car-       during        this     period             (Zielhuis         et at. 1990).
           ried      out      some        years         previously:                  some        conditions                 lead to progres-                             For    simplicity,              progress            through             the flow         chart         is halted         when

           sive       hearing             loss.        It        is salutary              to    note         that          on        follow-up                in   a primary            diagnosis              (in italic          type)        is arrived        at. However,                  it is, of

           adulthood,                  Mawhood                      et     at.       (2000)           found           bilateral                    hearing         course,           entirely           possible            that     more          than        one          pathology           is pre-
"          losses           exceeding              40 dB             in    three          out        of     23      children                who         had        sent.       The      question             the clinician                needs        to consider                 is whether           the

           been        identified               as having                 severe       receptive               language                 disorder              in   primary            diagnosis               can     adequately                  explain            the      child's        commu-

        '. childhood.                                                                                                                                             nicative          profile,           or whether             there           are some          features             that     are not
                 A   vexed           question               is    what       level        of     hearing            loss        is    sufficient             to    accounted              for.       For       instance,             we        would          expect           a    child       with          a


           explain            language             impairment.                       The        research            literature                suggests             severe        sensorineural                  hearing            loss to be slow                in acquiring                 spoken

           that      most          children            with         severe        and          profound             hearing               losses        will       language,             but       to      make        excellent               progress           in        mastering            a sign

           have       major          problems                    acquiring           oral       language             and literacy                    skills,       language,             if      exposure              to     this         mode          of     communication                          was
           even        if     they        are     diagnosed                  early             and        given       hearing                aids       and        provided              early          in     development                      (Petitto          2000).             Even if no
           auditory             training               (Conrad               1979).            Most of              these            children           will       signed        input           is available,               we      would             expect          to     see good           use of
           demonstrate                    normal                 communicative                       ability        in the            visuomotor                   non-verbal                 communication                          (gesture            and         facial         communica-

           modality   if exposed early to a sign   language  (Orlansky   &                                                                                         tion). Thus, if a hearing-impaired child shows little sign of com-
           Bonvillian 1985), and there is no evidence that learning to sign                                                                                        municating non-verbally, this is an indication that the hearing
           interferes
                    with acquisitionof spoken language
                                                     (Bishop1983).                                                                                                 lossis not the whole story,and further diagnoses
                                                                                                                                                                                                                  needto be
           Recently, dramatic gains in spoken language acquisition have                                                                                            considered.
           been seen in some children who have received cochlear implants                                                                                             The flow chart in Fig. 39.1 explicitly recommends continuing
           early in life (Miyamoto et at. 1997; Tomblin et at. 1999) and it                                                                                        through the decision tree if a child has a conductive hearing loss
           seems likely that this intervention will become increasingly                                                                                            or a mild sensorineural loss (under 40dB). This does not mean
           widespread. Nevertheless, outcome can be very variable, and                                                                                             that mild or intermittent losses should be ignored, nor that they
           some children make disappointing progress. It is also worth not-                                                                                        are irrelevant in the aetiology of speech and language problems,
           ing that there is strong resistance to cochlear implants from                                                                                           but they are unlikely to be the whole explanation for a child's di f-
           some members of the deaf community, who maintain that if the                                                                                            ficulties. In so far as detrimental long-term effects of OME have
           child learns sign language, deafness need not be a handicap                                                                                             been reported, they tend to occur in samples with nther risk
           (Lane 1990).                                                                                                                                            factors present, e.g. low birth weight and/or socioeconomic
                 Much lessis known about the impact of mild and moderate                                                                                           disadvantage (Gravel etat. 1996).
           sensorineural hearing loss on language development. The hand-
           ful of studies that include children with mild or moderate hear-                                                                                        A            . d               .1 t .                    h .
           ,    '"                 , 11  fi d 1          1 1 ,        d'                                                                                                 cqulre epl ep ICap asia
           mg Impairmenttyplca y n                                                        anguage eves mterme late
                                                                                      .                                                                 "an        (l          dau- KIeff nersyndrome)
           between those of normally hearIng and more severely heanng-
           impaired children (Brannon & Murry 1966). However, the                                                                                                  Acquired epileptic aphasia (AEA) should be suspected when
           average results may mask substantial variation. A recent small                                                                                          language regresses after a period                                           of normal development. Typi-
           studyof children with sensorineural hearing losses in the range                                                                                         cally, the child becomes increasingly unresponsive to spoken
           of 20-70dB HL, showed age-appropriate levels of language                                                                                                language, sometimes over a period of months but sometimes
           comprehension and expression in 78% of children (Norbury                                                                                                within a matter of days. Deterioration in expressive language
           et at., 2001). All of these were attending regular classrooms,                                                                                          typically follows. Deafness may be suspected, but normal hear-
           most had mild losses (20-40dB),                                                  and most wore hearing aids.                                            ing thresholds are obtained. In classic Landau-Kleffner                                                                             syn-
           None used sign language. This suggests that mild hearing loss                                                                                           drome, the clinical picture is one of severe and selective receptive
           can act as a risk factor for language impairment, but that, given                                                                                       aphasia, with the child retaining good non-verbal intelligence
           appropriate intervention, many children compensate well for                                                                                             (Landau & Kleffner 1957). This has also been described as an
           their hearing difficulties.                                                                                                                             auditory agnosia, which may extend to affect perception of
            Otitis media with effusion (OME) is a common childhood                                                                                                 non-verbal as well as verbal sounds.
          complaint that is often associated with mild conductive hearing                                                                                            The epileptic basis of the disorder may be overlooked because

           666
r                                                                                                                                              -
                                                                                             SPEECHAND lANGUAGE DIFFICULTIES

    over 50% of thesechildren do not presentwith frank seizures.         no consistentcausehasbeendemonstrated.A pair of discordant
    However, abnormal electrical activity, usually involving the         monozygotic twins has been reported, ruling out a purely
    temporal lobes, is evident on sleep electroencephalography           geneticaetiology (Feekeryet al. 1993).
    (EEG),although this abnormality typically diesdown byadoles-            Medical interventions typically involve use of anticonvul-
    cence,making retrospectivediagnosisdifficult.                        santsto control the epileptic activity, but although this is often
      The developmentalcourseof AEA is highly variable. In some          effective in controlling seizures,it doesnot necessarilynormal-
    children, the diseasefollows a fluctuating course, with periods      ize the underlying EEG abnormality and doesnot always lead to
    of improvements followed by regression. On average, the              improvement in language.Someauthorities haverecommended
    younger the child at onset, the worse the outcome for language,      aggressive  treatment with corticosteroids (Lerman et al. 1991),
    but this generalization hides a great deal of variability (Bishop    or neurosurgical intervention in caseswhere it is possible to
    1985). Many children with onsetbefore5 yearsof agehaveseri-          isolate the epileptic focus (Morrell et al. 1995). For both treat-
    ous and lifelong difficulties in understanding spoken language.      ments, some casesof dramatic improvement have beenreport-
    Nevertheless, a long-term case study suggeststhat gradual            ed, but suchsuccess not invariable, making it difficult to weigh
                                                                                               is
    improvement of language skill can continue over many years           the risks of adverseeffects against the possibility of recovery,
    (Van Dongen et al. 1989). Regarding seizures,the outcome is          especiallyin a disorder that may, in any case,follow a fluctuat-
    much more favourable, with these usually disappearing by             ing course. It is generally agreedthat an educational approach
    adolescence.                                                         that relies on developing visual forms of language (written or
       It is particularly important that child psychiatrists are aware   signed)is more effectivethan attempting to overcomethe child's
    of this rare disorder, because appearanceof communicative
                                    the                                  auditory impairment.
    difficulties in a previously normal child often prompts a psychi-       There has been some debate in the liter~ture as to whether,
    attic referral, especiallyif there are associatedbehavioural dis-    even after excluding those with Landau-Kleffner syndrome,
    turbances, as is not uncommonly the case (Appleton 1995).            there is an unusually high rate of EEG abnormality in children
    Differentialdiagnosis
                        from deafness
                                    shouldbeunproblematic
                                                        if               with languageimpairments(Echenne al. 1992; Parry-
                                                                                                         et
    proper audiological assessment carried out. AEA differs from
                                       is                                Fielder et al. 1997), and whether a similar pathophysiological
    selectivemutism in that languagecomprehensionis usually in-          processmight be present in children with more typical forms
    tact in the latter condition, and the child can be observedto talk   of developmental language disorder. For the present, this re-
    normally under certain restricted conditions. Neither is true for    mains a speculation without firm evidence (Deonna 2000).
    AEA. As noted by Genton & Guerrini (1993), it is essentialto         Where a child presents with language disorders and seizures
    conduct an EEG recording for one full sleepcycle in any child        but does not have the clinical picture of AEA, it is recom-
    who develops an unexplained language disorder, as this will          mended that the diagnostic process continues through the
    clearly demonstrate the underlying functional abnormality in         decisiontree.
    the brain of the child with AEA.
      As with so many of the conditions reviewed in this chapter, the    Global developmentaldelay
    boundaries of AEA are not clear-cut, and diagnosis of atypical
    casesposesparticular problems. Caseshave been described in           It is customary to make a diagnostic distinction betweencases
    which only expressivelanguaseis disturbed. In other children,        wherenon-verbal ability and ve,bal ability areequally impaired,
    the regressionaffectssocial.interaction and adaptive behaviour       and those where poor verbal skills are discrepant with normal
    as well as language,making it difficult to draw a sharp line be-     non-verbal ability. Terminology in this area is something of a
    tweenAEA and autistic regression(Deonna2000).                        minefield. Clinically, the term 'global developmental delay' is
      The prevalenceof AEA is hard to determine as it is a rare dis-     usedfrequently, although rather imprecisely,to refer to children
    order,which is often misdiagnosed either deafness selective
                                        as              or               who function well below agelevel in a rangeof domains, includ-
    mutism (seebelow). Appleton (1995) noted that over 200 cases         ing verbal and non-verbal ability, adaptive skills and motor de-
    have been reported since the condition was first described in        velopment. Terms such as 'mental handicap' and 'intellectual
    1957, and this number is increasing as the availability of new       retardation' are still used in some quarters to refer to children
    methods of brain imaging makes it possible to discover more          with an overall IQ below 70 (2 SD below the mean), but these
    about the underlying abnormality (Morrell & Lewine 1994;             labels have fallen out of favour because negativesocial con-
                                                                                                                    of
    Guerreiro et al. 1996). Most clinicians, however,can expect to       notations. In the UK, the preferred term in many clinical and
    see only one or two cases during a lifetime.                         educational contexts is 'learning difficulty' or 'learning disabil-
      The aetiology of AEA remainsa mystery.No structural brain          ity', but this has enormous potential for confusion, because
    lesion has beendemonsttated,and magneticresonanceimaging             outside the UK people tend to restrict the use of theseterms to
    (MRI) and computed tomography (CT) scan are usually nor-             children with normal intelligenceand a specificlearning disabil-
    mal, but metabolic abnormalities, predominantly in the tempo-        ity in one domain, such as specific reading disability. The term
    rallobes, are apparent on functional imaging (Guerreiro et al.       'global developmentaldelay' is usedhere,while recognizingit is
    1996; Da Silva et al. 1997). A variety of diseases,
                                                      ranging from       far from ideal (especiallyas 'delay' implies, unrealistically, that
    cerebralarteritis to subacuteencephalitis,have beenmooted but        there may be subsequent   catch-up).

                                                                                                                                      667
-                                                                                                               -
 CHAPTER39

    Sadly,a diagnosis of global developmental delay is often the             A core characteristic of autism is lack of social sensitivity. It is
  prelude to relative neglect of the child's language difficulties.       sometimesthought that all autistic children live in a world of
  There is a tendencyto assume  that the level of non-verbal ability      their own, ignoring all other people.This is far too extremea pic-
  sets some kind of limit on the level of language that can be            ture: many children with autism will enjoy cuddles and rough-
  achieved. There is evidence against this viewpoint from two             and-tumble play, but they may neither seeknor offer comfort or
 sources.First, somesyndromesare associatedwith a phenotype               affection. In older, verbal, high-functioning children, one may
 in which intelligence is impaired but languageis an area of rela-        find a strong desire to interact with other people, but a severe
 tive strength.The most well-known caseis that of Williams syn-           lack of understanding of how to do this. The concept of friend-
 drome. This is sometimes misleadingly described as though                ship as a reciprocal emotionally supportive relationship is hard
 language is normal. and other skills imp~ired. The reality for           for a child with autism to grasp.
 most children is that both verbal and non-verbal abilities are              Diagnosis dependson historical information about early de-
 well below averagebut, nevertheless,   skills suchas verbal mem-        velopment as well as observation and assessment the child's
                                                                                                                                of
 ory, vocabulary and syntax arefar better than thoseseen other
                                                          in             current behaviour and abilities. Specificinstruments developed
 children with different aetiologieswho have similar levelsof IQ         for the diagnosis of autistic disorder include the Autism
 (Morris & Mervis 1999). Furthermore, there may be relative              Diagnostic Interview-Revised (ADI-R), which is a parental in-
 sparing of aspectsof syntax and morphology that give especial           terview, and the Autism Diagnostic Observation Schedule
 difficulty to children with developmental language disorders            (ADOS-G), which hasfour modules,to cover the agerangefrom
 (Clahsen& Almazan 1998). The secondpoint is that interven-              infancy to adulthood, each involving direct observation of the
 tion studies suggestthat in many caseschildren of low IQ can            child or young person in situations designedto elicit autistic be-
 benefit from language intervention just as much as those of             haviours (Lord et al. 1994, 2000). Autistic disorder is covered
averageIQ(Feyetal.1994).                                                thoroughly by Lord & Bailey (Chapter 38), so in this section I
  Although the flow chart shows global developmental delay              shall focus just on areasof diagnostic difficulty.
and autistic disorder as separateentities, these disorders com-           Textbook casesof autistic disorder or developmental lan-
monly co-occur, and so it is import~nt to evaluate social com-          guagedisorder are easyenough to recognize,but many children
munication, play and repetitive behaviour in children with a            presentwith a pattern of symptoms that doesnot fit unambigu-
global developmentaldelay.                                              ously in either category, while showing some features of both.
                                                                        Thus their difficulties extend beyond the highly selectiveimpair-
Autistic disorder and related cond'tion S
                                  I
                                                                        ment of language structure seen in developmental language dis-
                                                                        order, but they do not have the full triad of autistic impairments

 Delayed language development and poor comprehension are                 in severeenough form to merit a diagnosisof autistic disorder.
 hallmarks of autistic disorder, and the issueof differential diag-      O'Hare et al. (1998) carried out an audit of 103 children re-
 nosis between autistic disorder and specific developmental              ferred to a speechclinic at Edinburgh Children's Hospital, and
 language disorder frequently crops up in clinical settings. A           found that eight of them met diagnostic criteria for autism, but a
 diagnosis of autistic disorder should be suspectedif the child's        further 14 had autistic symptomatology that fell short of meet-
 comprehensiondifficulties are accompaniedby more pervasive              ing diagnostic criteria, in most casesbecause  only two elements
 difficulties affecting social interaction, non-verbal communica-        of the triad of autistic impairments were present.All but one of
 tion and play, or if the child shows unusual repetitive or ritualis-    thesechildren was rated as having abnormal receptivelanguage               .
 tic behaviours, or restricted interests. The clinician needs to         on a speech therapy assessment.Although the diagnosis of
 consider whether language development is merely delayed, or             Asperger syndrome is sometimesusedin such cases,this is not
 whether thereare deviant featuresthat would not be regardedas           appropriate if the child's languagemilestonesare delayed.
 normal at any age, such as repetitive use of stereotypedcatch-            In the UK, the term 'autistic spectrum disorder' is usedquite
phrases,unusualand exaggeratedintonation, pronoun reversal,              widely, although often without clear diagnostic criteria. In the
and a frequent failure to respond when the parent attempts to            USA, and increasingly elsewhere,the DSM-IV (American Psy-
attract the child's attention. An intriguing observation in some         chiatric Association 1994) term 'pervasive developmental dis-
children with autism is that scoreson tests of expressivelan-            order not otherwise specified' (PDDNOS) is frequently applied.
guage(suchas picture naming) may be higher than those on re-             However, this is not very satisfactory,asthis was clearly intend-
ceptive tests (suchas selectinga named picture). Whereasmost            ed as a default category to be used in rare caseswhen a child
children with communicative problems will use non-verbal                showed autistic symptomatology but diagnostic criteria for
meansof expression,children with autism often have difficulty           autistic disorder were not met. Furthermore, it provides little in-
in both interpreting and producing appropriate non-verbal               formation about symptomatology and does not help decisions
communication. Imaginative play doesnot developnormally in              about educational placement. Bishop (2000) suggestedthat
children with autism; instead there may be repetitive routines,         thesedifficulties probably reflect the fact that diagnostic labels
suchasforming long lines of toy cars, or the child may be preoc-        impose a categorical structure on what is in reality a multidi-
cupied with everyday artefacts such as lights or switches, and          mensionalspace,    with children varying in terms of the severityof
disregardstoys that most children would find attractive.                impairments in language,social interaction and range of inter~

668
~
CHAPTER
      39

Researchhas shown that measures       obtained from spontaneous       (1994b) offered guidelinesthat still seemrelevant in the light of
speech samples are useful in identifying children who are             current research:children whose expressivevocabularies con-
deemedlanguageimpaired but who do not meet conventional               sist of lessthan 50 words at the ageof 24 months should becare-
psychometric criteria (Dunn et al. 1996). Unfortunately, such         fully monitored, but long-term problems are unlikely in those
measuresare time-consuming and not always practicable in              with vocabularies of more than eight words who have good
clinical settings. Furthermore, adequate normative data are           comprehension.
often lacking.                                                           There are a number of longitudinal studies showing that the
   In the final analysis,the specificcriteria adopted for identify-   child whose language is significantly impaired at 4-5 years of
ing languageimpairment will dependpartly on one'sgoalswhen            age is at high risk of developing literacy problems (Bishop &
making the diagnosis. Stringent criteria developed in research        Adams 1990; Tallal et al. 1997; Stothard et al. 1998; Johnson
contexts are not always appropriate in clinical settings, where       et al. 1999). Although it is commonly believed that oral lan-
the goal is to provide a diagnosis that ensures the child has         guage problems disappear with age to be replaced by literacy
access appropriate services.
        to                      Here, one wants to usemeasures        problems, this is seldom seen.Rather, the oral languageprob-
that haveecological validity-that have relevancefor function-         lemsbecomelessobvious in casualinteractions, but can beread-
ing in everyday communicative and social settings-and to              ily demonstrated on formal testing. It would be wrong to give
identify those children who will benefit from intervention. If        the impression that all language-impaired4 year olds are des-
one requires a large discrepancy between verbal and non-              tined for academic failure: some children do show marked
verbal ability before children can be considered for special          improvement. However, these tend to be children with pre-
educational services,  then many children with poor verbal skills     dominantly expressivedifficulties that have resolved by the age
are denied access,even though their linguistic problems may           of 51/2years (Bishop & Edmundson 1987; Bishop & Adams
be identical to those of other children who do meet diagnostic        1990).
criteria.                                                                DLD is associatedwith increasedrisk for psychiatric as well
                                                                      as language and literacy problems. The underlying nature of
D I     t I       d                                                   this associationhas beenthe causeof much speculation (Rutter
 eveopmena course prognosIs
                an
                                    "
                                                                                        .
                                                                      & Lor d 1987) Comor b1 lty cou ld reflect t he m fl uence 0 f
                                                                                                 .d.                     °

A number of longitudinal studies have thrown light on the de-         common aetiological risk factors, or the causal effect of one
velopmental course of DLD. There is general agreement that            condition on the other (Beitchmanet al. 1996). For instance,for
the child with significantly impaired receptive language skills       some children affective disorders and low self-esteem may
hasa poor prognosis,evenif this diagnosisis madeat a very early       be a consequenceof growing awareness of communicative
age.Comprehensionproblems do not usually appear to resolve            inadequacy.
spontaneously.On the contrary, the range of impairments seen
in a child with receptive language difficulties often increases
  .h             d.                       "I d       h. ° d.               I
                                                                      Prevaence
wIt age, exten mg to encompasssocIa an psyc IatriC Isor-
der (Rutter & Mawhood 1991), and impairment on non-verbal             Two recentepidemiological surveys,in the USA and Canada,es-
as well as verbal measures(Stothard et al. 1998; Johnson et al.       timated the prevalenceof specificlanguageimpairment (SLI) in
1999). There is muc;h more debate about prognosis for pre-            5 year olds at around.7% (Tomblin et al. 1997; Johnson et al.
school expressive   languagedisorders,with someproposing that         1999). However, it should be noted that neither study adopted
outcome is generallygood and intervention is seldomwarranted          the stringent 'discrepancy' criteria of DSM-IV and ICD-I0, but
(Paul 2000), while others maintain that these children are at         rather diagnosedSLI if the child scored below cut-off on stan-
high risk for persistingdifficulties that may only becomeappar-       dardized languagetests,but had a non-verbal IQ of 80 or above
ent on detailed testing (Scarborough& Dobrich 1990; Rescorla          and no other exclusionary criteria. Furthermore, in the study by
etal.1997). At leastsomeof this controversy arisesbecause      dif-   Tomblinetal., only 29% of thosediagnosedascases         ofSLIwere
ferent studies have used different follow-up periods to assess        already known by parents to have any speechor languagediffi-
prognosis. In the longitudinal study conducted by Paul et at.         culties. This estimateis likely therefore to be higher than would
(1996), the initial impressiongiven by the first wavesof follow-      be the caseif it were basedon a definition such as DSM-IV or
up was that 2 year olds with expressivelanguagedelayswere at          ICD-10, which requires both that there be a substantial dis-
high risk of persisting communicative problems. However, the          crepancy between verbal and non-verbal ability, and that the
longer the follow-up, the smallerthe proportion of children who       language impairment interferes with everyday or academic
had marked languageimpairment. It appearsthat the numbers             functioning.
of children with clinically significant language difficulties do
shrink dramatically as children mature. It seemsreasonableto          R kf t
                                                                       "            d t
                                                                                         I
                                                                                          o


                                              . .                .     IS ac ors an ae 10ogy
conclude that a good long-term prognosIs IS usually seen m
children identified as'late talkers' before the ageof 3 years,pro-    The principal risk factors for DLD are:
vided the problem is restricted to expressivelanguageand the.           male gender-in clinical samples, sex ratio of affected
delay is not too severe(Whitehurst & Fischel 1994). Bishop            males:femalesisaround30r4:1 (Robinson 1991);

670
~                                                                                                                                                                                                                                                                                                                                                                                                                                   -,




                                                                                                                                                                                                                                                                                       SPEECH                     AND                 lANGUAGE                                     DIFFICULTIES



    .        family                 history                    of         DLD-around                                                  30%               of        affected                   children                    should                 not            lead          us       to       conclude                     that            environmental                                          factors              are

    have             an        affected                   first-degree                                relative,                       compared                            with          3%             of        the     unimportant,                                or        that          nothing                  can            be     done              to        alleviate                     language

    general                   population                             (Stromswold                                       1998);                 and                                                                        difficulties.                         Provisional                         evidence                     suggests                     genes                may               act        as      risk

    .          being               a later-born                               child          in        a large                    family                (Bishop                   1997a).                                factors                that             increase                      the        probability                            that              a     child                 will            have            a

    Although                         much                 has             been              written                         about                language                        outcomes                         of     languag~                        disorder,                    but         the          severity                and            persistence                             of      language

    medical                    risk            factors,                       such           as otitis                      media                  and            low           birth           weight,                  disorder                    can         be highly                     variable,                    even            within                 a pair                of        genetically

    thereisnostrongevidencethattheseactasmajorriskfactorsfor                                                                                                                                                             identical                       monozygotic                              twins.               A        study                 by          Bishop                  et        al.        (1999)

    specific                  DLD,                  although                      they                may              act           synergistically                              to       cause                im-      suggested                        that            environmental                                    factors                could                 impair                      the        child's

    pairment                         in         a child                   who                is        already                        at     risk            from                other               causes              ability            to process                          non-verbal                       auditory                       stimuli,                 with             a subsequent

    (Bishop                    1987).                 Although                         there                  is an              association                            between                   low            so-     small             knock-on                             effect             on          language                         development.                                   In         children

    cioeconomic                                 status               and              DLD                  (Fundudis                             et al.               1979),             this          is not            who          were                    not         at         genetic               risk,            this           negative                      effect                 was             not        of

    strong,                    and             there            is little                   support                         for         the        commonly                                held             view         clinical                significance,                                 but         in        those                who               were                  at      genetic                     risk,

    that            parents                    can        cause                  their             child                   to     become                     language                       impaired                     for        whom                       language                        learning                    was              a     more                  difficult                     task,              the

    by          inadequate                            verbal                    stimulation,                                    except                  in        the       most               extreme                   combination                                of     environmental                                   and            genetic                 risk            factors                 was          suf-

    cases            of        abuse                and            neglect.                                                                                                                                              ficient             to           lead             to         clinically                     important                          problems                              in       language

              Over             the        past            decade,                     there                 has            been             an      explosion                         of       research                  learning.

    concerned                             with             the                genetic                   basis                   of         DLD                (see          Bishop,                       2001

    for              a   review).                          Three     twin                                    studies                   have     obtained                                       closely                   I t                     t   o


    slml "      "
                    I ar fi n d mgs        "              0 f h Ig h h enta
                                                                          "                       o         b I l Ity
                                                                                                                  o    o          f or t h IS d Isor d er
                                                                                                                                                    o             "                        (L ewls     &    o              n erven                   Ion

    Thompson                                   1992;               Bishop                    et         al.           1995;                  Tomblin                       &       Buckwalter                            Intervention                                is      usually                    carried                  out             by         speech                     and             language

    1998).                    A     molecular                          study                 of         a three-generational                                                    family                 show-             therapists,                          who          use           a wide                range             of       techniques                          to        stimulate                      lan-

    ing             an        autosomal                             dominant                               pattern                      of        inheritance                            for           severe            guage              learning.                        In       the         past,          there               was          a vogue                      for            drilling                chil-

    speech                    and         language                            disorder                      found                 clear            evidence                      of        linkage                 to    dren          in        grammatical                                   exercises,                    using                imitation                            and           elicitation

    a site               on         chromosome                                   7 (Fisher                             et       al.        1998),                 although                      it     is un-            methods,                         in        an          attempt                   to       have               the         child                extract                     the          salient

    clear            how                 far        these            results                  will               generalize                        to        other               cases            of        heri-        grammatical                                regularities.                         Such             methods                         fell        into            disuse              when                it

    table            language                         disorder.                                                                                                                                                          became                  apparent                         that         there            was          little         generalization                                     to      everyday

              Currently,                         there              is considerable                                         interest                in        two           aspects                  of         lan-     situations.                           Contemporary                                     approaches                             to          enhancing                              develop-

    guage                  functioning                                that             have                   been                   postulated                           as      behavioural                            ment              of         language                        structure                      are         more                 likely              to           adopt               'milieu'

    markers                         of         heritable                       SU.            The                first            is       phonological                                 short-term                       methods,                         in        which                the          intervention                          is     interwoven                                 into          natural

    memory,                          typically                      assessed                          by         asking                     the         child              to      repeat                   non-         episodes                        of      communication,                                        and             the            therapist                         builds                 on        the

    sense                words                 of      increasing                           length,                        such            as 'hampent'                            or        'blonter-                   child's                utterances,                            rather                  than             dictating                         what                 will           be        talked

    staping'                       (Gathercoleetal.1994).                                                                  Poor            performance                                on       this             task     about.                 In        addition,                      there             has         been               a move                       away               from                 a focus

    characterizes                                   many              children                         with                SU,             even          those              who             had             early        solely             on            grammar                          and          phonology                           toward                      interventions                                  that

    difficulties                          that           appear                   to        have                 resolved.                        Furthermore,                                 deficient                 develop                      children's                         social                use         of         language,                          often                 working                         in

    performance                                     on         non-word                                repetition                            showed                       very           high               heri-        small             groups                     that           may              include               normally                         developing                               as       well            as

    .tability                  in        a twin                study              (Bishop                         et al.               1996).                The            second                   area           in   l~nguage-impaired                                             peers             (Gallagher                         1996;                 Hayden                       &          Pukonen

    which                  many                 children                       with           DLD                      have             disproportionate                                        difficul-                 1996).

    ties            concerns                        certain                    aspects                      of         grammar.                          Children                       with                DLD                 Another                        way         in which                     modern                  approaches                              to     remediation                              dif-

    can             have             major                problems                            in        adding                         appropriate                              verb           endings,                   fer      from              the        past            is that               parents               are           more              likely             to        be        directly               in-

        such         as past                    tense               '-ed',             when                   given                   an     eliciting                     sentence                    frame             volved,                 particularly                              with          preschool-aged                                     children                      (Girolametto

        (e.g.        Q:            'Here                 the         boy              is      raking                        the            leaves.                What                 has           he         just     et     al.        1996).                     Methods                         such            as        the         Hanen                      approach"                               involve

        done?'                A:     'Raked                    the            leaves')                  (Rice2000).                                Rice               &     Wexler                   (1996)              videoing                        interactions                          between                     parent                 and             child                and          then            using

        have             postulated                        an         underlying                                 impairl11ent                                of       an        innate               system               these            when                 working                        with             groups                 of        parents                     in        a constructive

        that         has            evolved                    to             handle                  specific                       types              of        grammatical                                rule.        way         to        help            them              facilitate                   communication.

    With                 the        exception                        of        non-word                               repetition,                        the           measures                      used           to          A     radically                          different                    approach                        has        been              developed                              by     Tallal

        assess             such                hypotheses                             are             not             available                     as        standardized                                  tests,        et al.       (1996),                       who             have              devised               a computer-based                                             intervention,

        making                    them              1nsuitable                        for            clinical                   use         at present.                        Nevertheless,                             FastForword,                                   that         involves                  prolonged                         and             intensive                        ttaining                on

        the         work                 is      promising                             in         suggesting                                that             we           may           be           able           to    specific               components                                  of        language                      and         auditory                         processing.                          The

        develop                     more                 selective                      language                                 measures                         that           will             identify                theory                underlying                            this         approach                          maintains                         that            language                       diffi-

        homogeneous                                    groups                     of          children                            with             a         common                         aetiology.                    culties               are            caused                  by         a     failure                 to        make               fine-grained                                 auditory

        That              would                     be         a     considerable                                          improvement                                     on         the         current                 discriminations                                       in       the           temporal                       dimension,                              and              the         training

        position,                    where                 the            same               child                    mayor                   may             not           receive                  a diag-              materials                       are        designed                     to     sharpen                     perceptual                         acuity,                 in        much            the

        nosis            of       DLD               depending                           on            which                     tests        are         used              to    assess                verbal

        ability.
               The             fact             that               there                is         sttong                        genetic                     influence                       on             DLD           "See        website                   at http://hanen.velocet.ca/programs_parentoshtml



                                                                                                                                                                                                                                                                                                                                                                                                                        671
CHAPTER39

sameway as has been demonstratedin animal experiments. By              phonological teststhat require no speechfrom the child. Some
embeddingtraining in attractive computerized games,children            children with phonological problems have difficulties in dis-
can be persuadedto participate in thousands of training trials,        criminating between similar speechsounds, such as 'pat' vs.
in a way that would simply not be possible with a standard             'cat', when asked,for instance,to selecta picture to match what
therapist-basedinteraction.                                            they haveheard. However, the most common difficulty is not so
   For all thesetypes of intervention, there are few adequately        much in telling soundsapart, asin recognizingthat different ex-
contl;olled trials that allow one to assessclinical efficacy.In gen-   emplars of the same sound are indeed the same sound. So if
eral, one does not seemiracle cures, but this is not to say that       asked to say whether 'bag' or 'boat' rhymes with 'rag', or to
gains are negligible (Bryne-Saricks 1987; Law et at. 1998). A          judge whether 'soup' or 'coat' begins with the same sound as
clinical trial assessing FastForword approach showed sig-
                          the                                          'Sam', the child with phonological problems may perform at
nificant gainsrelative to a control group (Merzenich et at. 1996),     chancelevels (Bird et at. 1995). Suchobservations suggestthat
but questionsremain about the persistence      and generalizability    the difficulty is one of categorization of speech,rather than poor
of theseeffects.Sincethe initial controlled trial, the authors have    acuity for differencesbetweenspeech      sounds.
gathered a large amount of data on pre- and post-intervention             The prognosis of pure phonological disorder is much better
language test scoresof children enrolled in FastForword, but           than that of languagedisorder (Bishop & Adams 1990;Johnson
this is difficult to evaluate without controls for practice and        et at. 1999), especiallyif the phonological difficulties resolveby
placebo effects (TallaI2000). Furthermore, it is not possible to       the time the child starts school (Bird et at. 1995). It is difficult
know which specific components of this complex intervention            to estimate the prevalenceof phonological problems, because
are most effective,or whether the whole gamut of different exer-       studies typically do not discriminate betWeen    different types of
cisesis essentialto achievetherapeutic benefits.                       speech   problem; lisping and other deviations,specificphonolog-
                                                                       ical impairments, and speech    problems accompanyinglanguage
D I       t I h I . I d. d                                             impairment all tend to be included together. Furthermore,
 eve opmen a p ono oglca Isor er                                                                                         .
                                                                       prevalenceappearsstrongly age-dependent,        with speechprob-
It is customary to draw a distinction betWeen   speech, physi-
                                                        the            lems declining sharply betWeen3 and 6 years of age (Morley
cal act of articulating speechsounds, and language,the whole            1972). After excluding children with additional handicaps,
complex system of combining elements of sound at different             Johnsonetat. (1999) obtained a prevalence     estimateof6.1 % for
levelsof complexity to expressmeaning. It is possibleto have a         specific speech-onlyimpairments at 5 years of age.This figure
languageimpairment with normal speech(e.g.in cases DLD    of           excludes the children from this sample who had comorbid
where the child speaksclearly but doesnot comprehendor pro-            speechand languageimpairment. Shriberg et at. (1999) report-
ducecomplex syntactic constructions). The conversesituation is         ed a prevalenceof speechdelay in US 6-year-oldsof 3.8%, with
also seen,when the child has somedifficulty in producing clear         comorbid languageimpairment in around 12% of thesecases.
speechbut the underlying languageskills are intact, e.g. in cases      Little is known about risk factors and aetiology of phonological
of dysarthria (seebelow). The child who persistsin using imma-         disorders, although, as with other communication disorders,
ture or deviant sound patterns but who has no physical b~sis           boys are at greater risk than girls (Shriberg et at. 1999). Inter-
for this disorder does not fit so neatly into this dichotomous         vention is carried out by speech-languagetherapists, and
view. Speechis undoubtedly the prese~ting problem, but the             typically involves games and exercisesto develop the child's
underlying impairment appearsto be linguistic rather than one          awareness phonemic contrasts (Deanet at. 1995).
                                                                                    of
of motor control: a failure to learn which speech soundsare dis-
tinctiv.ein the am.bientlanguage.Often th~ speech   errors involve     Developmental       verbal dyspraxia
a persistenceof Immature patterns. For Instance, sounds pro-
ducedin the back of the mouth, suchas 'k' and 'g' are not distin-      Developmental verbal dyspraxia is a controversial diagnostic
guishedin the child's output from those produced in the front of       category that is defined differently by different experts,and not
the mouth, suchas't' and 'd', so that 'cat' may be pronounced as       usedat all by someauthorities (fora review seeCrary 1993). The
'tat' and dog as'dod'. The terms 'phonological disorder' (DSM-         central characteristicin most definitions is that there aredifficul-
IV) and 'phonological impairment' have superseded      such labels     ties in speechproduction that suggestan impairment of motor
as 'functional articulation disorder' to refer to such problems.       programming, because is the length and complexity of what is
                                                                                                it
The term 'phonological' implies that the child's difficulties are      uttered, rather than the specific speechsounds used,that is the
linguistic rather than motoric, perhapsakin to thoseof an adult        main factor determining accuracy.In children with this diagno-
mastering a foreign language. Most of us have difficulty in            sis, one is likely to seespeecherrors that are inconsistent from
learning to use a new set of speechsounds, not becauseour              one occasion to the next, that are particularly evident in poly-
articulators are in any way defective, but because have not
                                                     we                syllabic words, and that involve transpositions of speech
internalized the sound distinctions that are critical in the           soundsrather than simple substitution of one soundfor another.
language.                                                              For instance, Bradford & Dodd (1996) reported a dyspraxic
    Evidencethat a phonological disorder is not just a problem in      child whoserenderingsof 'elephant' on three separateoccasions
articulating soundsaccuratelycan be obtained using specialized         were 'ewint', 'wuwit' and 'uwit'.

672
r"                                                                                              SPEECHAND lANGUAGE DIFFICULTIES




        Debate continues over the question of whether problems in           Drought syndrome.However, Clark et at. (2000.),in a recentre-
     sequencingnon-verbal movementsshould be part of the diag-              view of 47 cases,noted that most children with this condition
     nostic criteria: someauthorities maintain that to be regardedas        have additional complex impairments, including mild pyrami-
     dyspraxic, the child should be impaired in imitating sequences         dal tetraplegia, learning difficulties, behaviour problems and
     of non-speech   movementsof the tongue and mouth. Not all chil-        epilepsy.Crary (1993) noted that remarkably little clinical or re-
     dren who make inconsistentphonological errors haveextensive            searchattention has beenpaid to developmental dysarthria, al-
     difficulties producing non-speech    movements,raising the ques-       though it was well describedby Morley et at. (1954) more than
     tion of where they should beclassified(Bradford & Dodd 1996).          40 yearsago. It appearsto be a strongly familial condition.
     In addition, there is the question of whether dyspraxia should be        Dysarthria and anarthria involve difficulties affecting speech
     diagnosedin a child who hasbroader difficulties with expressive        rather than language,and so one would expect to find normal
     language, or only in those with a relatively pure problem in           language comprehension and normal literacy skills in pure
     speech   output. In practice, many children who receivethis diag-      cases.However, quite often the aetiological factors that cause
     nosis do have associatedproblems affecting language, literacy          articulation difficulties also lead to problems in other areas,in-
     and phonological awareness     (Stackhouse1992).                       cluding hearing and language.
        The lack of agreeddiagnostic criteria make it impossible to
     ma~e generalizations about risk factors, prevale?ce or .prog-          Selective mutism
     nosls.The causeof developmentalverbal dyspraxia remains an
     enigma, but it appearsto be strongly familial (Morley 1972). It        Selectivemutism is diagnosedwhen a child is able to speakbut
     is also worth noting that although their grammatical difficulties      fails to do so except in very restricted situations, such as with
     have beenemphasizedin published accounts, the phenotype in             close family. This disorder was previously known as 'elective
     the three-generationalfamily mentioned in the section on DLD           mutism', but the terminology was modified in DSM-IV to avoid
     also involved severely dyspraxic speech (Hurst et at. 1990).           the connotation of volitional behaviour.The diagnosisis strong-
     Crary (1993) provides'an overview of approachesto interven-          . lysuggestedwhen one finds mutism in a child whohasnoneuro-
     tion for developmentalverbal dyspraxia.                                logical or structural abnormalities of the articulators, and who
                                                                            has normal languagecomprehension,as well as a normal early
     A th .     dd  th .                                                    historyof usinglanguage.
                                                                                                   However, crucialpoint that needs
                                                                                                          the
      nar ria an ysar ria                                                     b     bl h d . h h hold d
                                                                                       o                      k.         ..
                                                                           to eesta IS e 1St att ec I           oesspea In somesituations.
     Anarthria or dysarthria is diagnosed when speech problems                As Dummit et at. (1997) noted, this condition is more pro-
     arise becauseof structural or neurological abnormalities of           perly regardedasa form of anxiety disorder rather than a speech
     articulatory control. Anarthria is the term used when there is        and languagedisorder. Rates of comorbid anxiety and phobic
     no ability to produce speech,whereas dysarthria refers to dis-        disorders are high, both in affected children and in their first-
     ordered articulation caused by weakness, incoordination or            degreerelatives. However, differential diagnosiscan be compli-
     structural abnormalities of the articulators. It is important to      cated by the fact that some children with selectivemutism do
     distinguish these articulation problems, where speech is im-          have developmentallanguagedisorders (Kristensen2000), sug-
     paired because problems producing articulatory movements,
                      of                                                   gesting that self-consciousness   about inadequate communica-
     from developmentalphonological disorders, where the child is          tion skills may play a.part in maintaining mutism.
     neurologically normal and capable of producing articulatory              Persistent selectivemutism affects less than 1 in 1000 chil-
     movements(seeabove). Neurological conditions that can cause           dren, although the frequency of transient mutism in children
     anarthria or dysarthria include cerebral palsy and Mobius             starting school is much higher.Girls are two to three times more
     syndrome,in which there is agenesis cranial nerve nuclei and
                                           of                              likely to be affectedthan boys.The causes selectivemutism re-
                                                                                                                       of
     associated  facial immobility. Structural abnormalities of articu-    main unknown. Although conventional wisdom maintains that
     lators that can lead to dysarthric speech include cleft palate and    physical or sexual abuseor other kinds of trauma may precipic
     TreacherCollins syndrome.There are a number of other genetic          tate selectivemutism, there is little evidenceof this (Black &
     syndromesthat are associatedwith unusual proportions of the           Uhde 1995), and the strong familial component to the disorder
     articulators and/or hypotonicity which affects tongue control,        suggests  that Dummit et at. (1997) may be correct in regarding
     e.g.Down syndrome.                                                    this disorder as the extreme end of a biologically basedcon-
        Where no specificsyndromeis detected,one should bealert to         tinuum of temperamentand social behaviour.
     the possibility of dysarthria when there is poor co-ordination or        Behaviour modification methods have been shown to be ef-
     weaknessof facial muscles,as evidencedby drooling, feeding            fective in re-establishing speech(Sluckin et at. 1991), but the
     problems, or difficulties imitating simple oral movementssuch         long-term prognosis of selectivemutism is neverthelesspoor.
     as moving the tongue from side to side or pursing the lips.           There is a high rate of personality disorder and psychiatric prob-
     Worster-Drought (1974) stressedthat anarthria can occur in            lems associatedwith a history of selectivemutism (Kolvin &
     children in the absenceof any other neurological impairment,          Fundudis 1981). Dummit et at. (1997) argued that therapeutic
     and he gave detailed descriptions of this condition, which is         interventions should focus on alleviating anxiety, but there has
     known as both congenital suprabulbar paresis and Worster-             beenno systematicresearchon the efficacyof this approach.

                                                                                                                                        673
Speech and language difficulties (2002)
Speech and language difficulties (2002)
Speech and language difficulties (2002)
Speech and language difficulties (2002)
Speech and language difficulties (2002)
Speech and language difficulties (2002)
Speech and language difficulties (2002)
Speech and language difficulties (2002)

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Speech and language difficulties (2002)

  • 1. Child and Adolescent Psychiatry Editedby Michael Rutter CBE,MD, FRCP, FRCPsych, FMedSci FRS, Professorof Developmental Psychopathology Social, Genetic and Developmental Psychiatry ResearchCentre Institute of Psychiatry London Eric Taylor MA, MB, FRCP, FRCPsych, FMedSci Professorof Child and AdolescentPsychiatry Department of Child and AdolescentPsychiatry Institute of Psychiatry London FOURTH EDITION Blackwell Science
  • 2. r9 -- Speechand LanguageDifficulties I,;) .-, Dorothy ~M. Bishop Introduction so that the bulk of phonology and syntax is acquired by around 4 years of age. There are, however, frequent exceptions, and The ability to communicate through language distinguishes theseare the topic of this chapter. humans from all other animals. Spoken language allows us to Delay in learning to talk is a common reason for a parent to convey information, expressour feelings and demonstrate so- seek advice from a .family doctor or paediatrician. Because cial affiliations. It also provides a vehicle for organizing our human communication is complex, assessment and diagnosis thoughts and memories,enabling us to construct complex lines of speechand languagedifficulties in children is a particularly of reasoning, and to contemplate past, future and hypothetical challenging problem that requires expertise in several differ- events,rather than remaining grounded in present reality. The ent areas, including linguistics, audiology, child development, developmentof written languageprovides evenmore dramatic neuropsychology, paediatric neurology and psychiatry. This releasefrom the here-and-now,making it possible to transcend chapter will usethe diagnostic flow chart shown in Fig. 39.1 to spaceand time. introduce a range of different conditions that can lead to speech All known human cultures have language,but there is huge and languagedifficulties in children. This depicts the sequence diversity in how languagesare structured, both in terms of the of decisions the clinician needsto make when first assessing a sounds used to expressmeaning (phonemes)and the ways in child who presentswith poor communication. However, recent which linguistic elementsare combined (syntax). For instance, researchsuggests that this diagnostic processshould not becon- Frenchhastwo different vowels that sound like '00' to a speaker fined to thosecases wherecommunicative impairment is the pre- of English, but which arephonemically distinct; they signal con- senting complaint, but should be extendedmuch more broadly trasts in meaning, so that 'rue' and 'roux' mean different things. to all children referred to psychiatric services. The reasonis sim- In English, on the other hand, we make a phonemic contrast be- pie: surveysof children attending psychiatric clinics revealthat a tween the sounds 'th' and 'z' (e.g. 'bathe' vs. 'baize'), which are high proportion of them have somekind of communicative im- not distinguished in French.In tone languages,such as Chinese pairment, and in many cases goesunrecognizedunlessa for- this Mandarin, the pitch at which a word is spoken signalsmeaning, mal assessment made. Cohen (1996) summarized findings is so that 'ba' has four completely different meanings depending from a Canadian study in which 399 consecutivepsychiatric re- on whether the pitch is rising ('to uproot'), falling ('a harrow'), ferrals of children agedfrom 4 to 12 yearswere given a detailed changing from fall to rise ('to hold') or at a level high pitch language assessment. Children with autistic disorder, general ('eight')." developmentaldelay,neurolog.icaldamage,hearing impairment Moving to grammar; in English, relationships between enti- or a non-English-speakinghome background had beenexclud- ties are indicated by a mixture of word order and grammatical ed from this sample. Around one-quarter of the children had morphemes(e.g. inflectional endingssuch as '-ing' or '-ed', and previously identified languageimpairments. Of the remainder, small function words such as 'by'). Thus it is the boy who is none of whom was thought to be language-impaired,34% met doing the kissing in 'the boy kissesthe girl', but is the recipient of criteria for language impairment. There appeared to be two the kiss in 'the girl kissesthe boy' or 'the boy is kissedby the girl'. reasonswhy communicative difficulties had been overlooked In somelanguages,suchasTurkish, word order generally obeys in thesechildren. First, they did not have such overt expressive the sequencesubject-object-verb, and inflectional suffixes do language difficulties as children with previously identified all the work of expressingrelationships. Other languages,such problems, although their receptivelanguageskills were aspoor as ChineseMandarin, havevirtually no inflections. Word order, as that group. Secondly,they were more likely to haveexternal- particles and prepositions are usedto indicate how the elements izing psychiatric disorders, which may have diverted attention of a sentence interrelate. from communication. Cohen et al. (1998) suggested that Clearly, languageacquisition involves far more than learning languagefunction should be incorporated routinely into the as- labels for things. The child must work out which speechsound sessmentand treatment process for children with psychiatric contrasts are meaningful in the ambient lang~age,and how to impairments. Somesuggestions how to implement this rec- for combine words and grammatical morphemes to expressrela- ommendation are given below in the sectionon Assessment. tionships betweenthings and events.Most children master this In the next section, different diagnostic entities will be re- complex skill with no explicit instruction and with relative ease, viewed, with a main focus on specificdevelopmental language 664 "~
  • 3. ~ SPEECHAND lANGUAGE DIFFICULTIES START Islangu Does child h age . Yes produce Yes Refer to speech and language compre enslon . t , . Immature /d evlan t . th eraplst,. query expressive . age-approprla e. utterances? language disorder No No Isspeech unintelligible or Language impairmentsecondary Yes ~ens?r poorly to hearingloss earln articulated? 40 dB Yes Any indication of I . No neuro oglcaI or Referto speechand language ~tru~ural therapist, query developmental Referfor neurologicalopinion, Yes Any evi.denceof Impairment? phonological disorder acquiredepileptic aphasia re.9resslon or No query seizures? No Are speech Yes ~rrors. Yes No Inconsistent and worsewith longer Refert? speechand language .. utterances therapist, query developmental GobaI deveopmentaI deIay I I No well above ability Non-verbal .. verbaldyspraxia h . Dysarthria/anarthria compre enSlon level? Yes Detailedevaluationto consider Yes Impairmentsin Ischild m.ute Yes Selectivemutism autisticdisorder/ PDDNOS non-verbalsocial exceptwIth close communication/play? family/friends? No Isspeechfluent? Referto speechand language '. No therapist, query developmental Assessexpressive language. fl d' d t tt . , {, considermixed receptive- uency Isor er ,s u erlng, expressive languagedisorder Yes Is voice quality Refer to otolaryngology, query normal? No voice disorder Yes Does child say Consider Asperger syndrome. things that are Yes pragmatic language bizarre or impairment tangential? Fig.39.1Decision for diagnosing tree speech language and disorders children. in .. disorder.Assessment procedures will briefly be reviewed in a of this chapter, but it is worth noting here that assessment can later section. be difficult in children with major behavioural difficulties. It is tempting to assumethat the child can understand but is unco- operative, but it is at least as likely that the behavioural difficul- A decision tree for diagnosis ties stem from fear and frustration in a child who comprehends very little. If the child doesnot co-operatewith formal compre- In Fig. 39.1, the question of whether comprehension is age- hensiontesting, proceeddown the decision tree on the left-hand appropriate is placed at the top of the decision tree. There are side of the diagram. good reasonsfor this. First, whereasproblems with expressive As shown in Fig. 39.1, results from a comprehensionassess- speech languageare usually fairly easyto detecton the basis and ment are not sufficient for a diagnosis,but they determinewhich of informal observation, comprehensionis much harder to esti- diagnosesshould be considered, and also help the clinician to matethis way. Secondly,different diagnosesneedto be consid- adjust his or her languagelevel to the child's level of understand- ered for the child with comprehension problems than for one ing, for instance when conducting a psychiatric interview. whose problems are confined to speech output or sentence Where comprehension is unimpaired, we can exclude autistic formulation. disorder and mental handicap. It is also unlikely that hearing Methods for testing comprehensionare discussedat the end lossor acquired epileptic aphasiais implicated. 665
  • 4. -- CHAPTER 39 Hearing loss loss. It is often cited as a cause of speech and language difficul- "" ties, but recent research suggests that the effect may have been ! The first diagnosis to consider in a child with comprehension dif- overestimated in the past by relying on clinical samples (Bishop ficulties is hearing loss. Note that in Fig. 39.1 evaluation of hear- & Edmundson 1986; Bishop 1988). Epidemiological studies ing comes before assessment of non-verbal ability. It is all too have found only weak influences, if any, on long-term speech, easy to assume that if a child has low IQ then the language im- language and literacy outcomes (Grievink et at. 1993; Peters et pairment has been explained. However, impaired hearing is a at. 1994). It is also important to be aware just how common common correlate of many syndromes that affect general ~ntelli- OME is. A Dutch epidemiological study of children screened at gence, and an audiological evaluation should always be under- 3-monthly intervals between 2 and 4 years of age found that .taken in a child with poor understanding, regardless ofIQ level 55% of children had at least one episode of bilateral OME Furthermore, one should beware of relying on hearing tests car- during this period (Zielhuis et at. 1990). ried out some years previously: some conditions lead to progres- For simplicity, progress through the flow chart is halted when sive hearing loss. It is salutary to note that on follow-up in a primary diagnosis (in italic type) is arrived at. However, it is, of adulthood, Mawhood et at. (2000) found bilateral hearing course, entirely possible that more than one pathology is pre- " losses exceeding 40 dB in three out of 23 children who had sent. The question the clinician needs to consider is whether the been identified as having severe receptive language disorder in primary diagnosis can adequately explain the child's commu- '. childhood. nicative profile, or whether there are some features that are not A vexed question is what level of hearing loss is sufficient to accounted for. For instance, we would expect a child with a explain language impairment. The research literature suggests severe sensorineural hearing loss to be slow in acquiring spoken that most children with severe and profound hearing losses will language, but to make excellent progress in mastering a sign have major problems acquiring oral language and literacy skills, language, if exposure to this mode of communication was even if they are diagnosed early and given hearing aids and provided early in development (Petitto 2000). Even if no auditory training (Conrad 1979). Most of these children will signed input is available, we would expect to see good use of demonstrate normal communicative ability in the visuomotor non-verbal communication (gesture and facial communica- modality if exposed early to a sign language (Orlansky & tion). Thus, if a hearing-impaired child shows little sign of com- Bonvillian 1985), and there is no evidence that learning to sign municating non-verbally, this is an indication that the hearing interferes with acquisitionof spoken language (Bishop1983). lossis not the whole story,and further diagnoses needto be Recently, dramatic gains in spoken language acquisition have considered. been seen in some children who have received cochlear implants The flow chart in Fig. 39.1 explicitly recommends continuing early in life (Miyamoto et at. 1997; Tomblin et at. 1999) and it through the decision tree if a child has a conductive hearing loss seems likely that this intervention will become increasingly or a mild sensorineural loss (under 40dB). This does not mean widespread. Nevertheless, outcome can be very variable, and that mild or intermittent losses should be ignored, nor that they some children make disappointing progress. It is also worth not- are irrelevant in the aetiology of speech and language problems, ing that there is strong resistance to cochlear implants from but they are unlikely to be the whole explanation for a child's di f- some members of the deaf community, who maintain that if the ficulties. In so far as detrimental long-term effects of OME have child learns sign language, deafness need not be a handicap been reported, they tend to occur in samples with nther risk (Lane 1990). factors present, e.g. low birth weight and/or socioeconomic Much lessis known about the impact of mild and moderate disadvantage (Gravel etat. 1996). sensorineural hearing loss on language development. The hand- ful of studies that include children with mild or moderate hear- A . d .1 t . h . , '" , 11 fi d 1 1 1 , d' cqulre epl ep ICap asia mg Impairmenttyplca y n anguage eves mterme late . "an (l dau- KIeff nersyndrome) between those of normally hearIng and more severely heanng- impaired children (Brannon & Murry 1966). However, the Acquired epileptic aphasia (AEA) should be suspected when average results may mask substantial variation. A recent small language regresses after a period of normal development. Typi- studyof children with sensorineural hearing losses in the range cally, the child becomes increasingly unresponsive to spoken of 20-70dB HL, showed age-appropriate levels of language language, sometimes over a period of months but sometimes comprehension and expression in 78% of children (Norbury within a matter of days. Deterioration in expressive language et at., 2001). All of these were attending regular classrooms, typically follows. Deafness may be suspected, but normal hear- most had mild losses (20-40dB), and most wore hearing aids. ing thresholds are obtained. In classic Landau-Kleffner syn- None used sign language. This suggests that mild hearing loss drome, the clinical picture is one of severe and selective receptive can act as a risk factor for language impairment, but that, given aphasia, with the child retaining good non-verbal intelligence appropriate intervention, many children compensate well for (Landau & Kleffner 1957). This has also been described as an their hearing difficulties. auditory agnosia, which may extend to affect perception of Otitis media with effusion (OME) is a common childhood non-verbal as well as verbal sounds. complaint that is often associated with mild conductive hearing The epileptic basis of the disorder may be overlooked because 666
  • 5. r - SPEECHAND lANGUAGE DIFFICULTIES over 50% of thesechildren do not presentwith frank seizures. no consistentcausehasbeendemonstrated.A pair of discordant However, abnormal electrical activity, usually involving the monozygotic twins has been reported, ruling out a purely temporal lobes, is evident on sleep electroencephalography geneticaetiology (Feekeryet al. 1993). (EEG),although this abnormality typically diesdown byadoles- Medical interventions typically involve use of anticonvul- cence,making retrospectivediagnosisdifficult. santsto control the epileptic activity, but although this is often The developmentalcourseof AEA is highly variable. In some effective in controlling seizures,it doesnot necessarilynormal- children, the diseasefollows a fluctuating course, with periods ize the underlying EEG abnormality and doesnot always lead to of improvements followed by regression. On average, the improvement in language.Someauthorities haverecommended younger the child at onset, the worse the outcome for language, aggressive treatment with corticosteroids (Lerman et al. 1991), but this generalization hides a great deal of variability (Bishop or neurosurgical intervention in caseswhere it is possible to 1985). Many children with onsetbefore5 yearsof agehaveseri- isolate the epileptic focus (Morrell et al. 1995). For both treat- ous and lifelong difficulties in understanding spoken language. ments, some casesof dramatic improvement have beenreport- Nevertheless, a long-term case study suggeststhat gradual ed, but suchsuccess not invariable, making it difficult to weigh is improvement of language skill can continue over many years the risks of adverseeffects against the possibility of recovery, (Van Dongen et al. 1989). Regarding seizures,the outcome is especiallyin a disorder that may, in any case,follow a fluctuat- much more favourable, with these usually disappearing by ing course. It is generally agreedthat an educational approach adolescence. that relies on developing visual forms of language (written or It is particularly important that child psychiatrists are aware signed)is more effectivethan attempting to overcomethe child's of this rare disorder, because appearanceof communicative the auditory impairment. difficulties in a previously normal child often prompts a psychi- There has been some debate in the liter~ture as to whether, attic referral, especiallyif there are associatedbehavioural dis- even after excluding those with Landau-Kleffner syndrome, turbances, as is not uncommonly the case (Appleton 1995). there is an unusually high rate of EEG abnormality in children Differentialdiagnosis from deafness shouldbeunproblematic if with languageimpairments(Echenne al. 1992; Parry- et proper audiological assessment carried out. AEA differs from is Fielder et al. 1997), and whether a similar pathophysiological selectivemutism in that languagecomprehensionis usually in- processmight be present in children with more typical forms tact in the latter condition, and the child can be observedto talk of developmental language disorder. For the present, this re- normally under certain restricted conditions. Neither is true for mains a speculation without firm evidence (Deonna 2000). AEA. As noted by Genton & Guerrini (1993), it is essentialto Where a child presents with language disorders and seizures conduct an EEG recording for one full sleepcycle in any child but does not have the clinical picture of AEA, it is recom- who develops an unexplained language disorder, as this will mended that the diagnostic process continues through the clearly demonstrate the underlying functional abnormality in decisiontree. the brain of the child with AEA. As with so many of the conditions reviewed in this chapter, the Global developmentaldelay boundaries of AEA are not clear-cut, and diagnosis of atypical casesposesparticular problems. Caseshave been described in It is customary to make a diagnostic distinction betweencases which only expressivelanguaseis disturbed. In other children, wherenon-verbal ability and ve,bal ability areequally impaired, the regressionaffectssocial.interaction and adaptive behaviour and those where poor verbal skills are discrepant with normal as well as language,making it difficult to draw a sharp line be- non-verbal ability. Terminology in this area is something of a tweenAEA and autistic regression(Deonna2000). minefield. Clinically, the term 'global developmental delay' is The prevalenceof AEA is hard to determine as it is a rare dis- usedfrequently, although rather imprecisely,to refer to children order,which is often misdiagnosed either deafness selective as or who function well below agelevel in a rangeof domains, includ- mutism (seebelow). Appleton (1995) noted that over 200 cases ing verbal and non-verbal ability, adaptive skills and motor de- have been reported since the condition was first described in velopment. Terms such as 'mental handicap' and 'intellectual 1957, and this number is increasing as the availability of new retardation' are still used in some quarters to refer to children methods of brain imaging makes it possible to discover more with an overall IQ below 70 (2 SD below the mean), but these about the underlying abnormality (Morrell & Lewine 1994; labels have fallen out of favour because negativesocial con- of Guerreiro et al. 1996). Most clinicians, however,can expect to notations. In the UK, the preferred term in many clinical and see only one or two cases during a lifetime. educational contexts is 'learning difficulty' or 'learning disabil- The aetiology of AEA remainsa mystery.No structural brain ity', but this has enormous potential for confusion, because lesion has beendemonsttated,and magneticresonanceimaging outside the UK people tend to restrict the use of theseterms to (MRI) and computed tomography (CT) scan are usually nor- children with normal intelligenceand a specificlearning disabil- mal, but metabolic abnormalities, predominantly in the tempo- ity in one domain, such as specific reading disability. The term rallobes, are apparent on functional imaging (Guerreiro et al. 'global developmentaldelay' is usedhere,while recognizingit is 1996; Da Silva et al. 1997). A variety of diseases, ranging from far from ideal (especiallyas 'delay' implies, unrealistically, that cerebralarteritis to subacuteencephalitis,have beenmooted but there may be subsequent catch-up). 667
  • 6. - - CHAPTER39 Sadly,a diagnosis of global developmental delay is often the A core characteristic of autism is lack of social sensitivity. It is prelude to relative neglect of the child's language difficulties. sometimesthought that all autistic children live in a world of There is a tendencyto assume that the level of non-verbal ability their own, ignoring all other people.This is far too extremea pic- sets some kind of limit on the level of language that can be ture: many children with autism will enjoy cuddles and rough- achieved. There is evidence against this viewpoint from two and-tumble play, but they may neither seeknor offer comfort or sources.First, somesyndromesare associatedwith a phenotype affection. In older, verbal, high-functioning children, one may in which intelligence is impaired but languageis an area of rela- find a strong desire to interact with other people, but a severe tive strength.The most well-known caseis that of Williams syn- lack of understanding of how to do this. The concept of friend- drome. This is sometimes misleadingly described as though ship as a reciprocal emotionally supportive relationship is hard language is normal. and other skills imp~ired. The reality for for a child with autism to grasp. most children is that both verbal and non-verbal abilities are Diagnosis dependson historical information about early de- well below averagebut, nevertheless, skills suchas verbal mem- velopment as well as observation and assessment the child's of ory, vocabulary and syntax arefar better than thoseseen other in current behaviour and abilities. Specificinstruments developed children with different aetiologieswho have similar levelsof IQ for the diagnosis of autistic disorder include the Autism (Morris & Mervis 1999). Furthermore, there may be relative Diagnostic Interview-Revised (ADI-R), which is a parental in- sparing of aspectsof syntax and morphology that give especial terview, and the Autism Diagnostic Observation Schedule difficulty to children with developmental language disorders (ADOS-G), which hasfour modules,to cover the agerangefrom (Clahsen& Almazan 1998). The secondpoint is that interven- infancy to adulthood, each involving direct observation of the tion studies suggestthat in many caseschildren of low IQ can child or young person in situations designedto elicit autistic be- benefit from language intervention just as much as those of haviours (Lord et al. 1994, 2000). Autistic disorder is covered averageIQ(Feyetal.1994). thoroughly by Lord & Bailey (Chapter 38), so in this section I Although the flow chart shows global developmental delay shall focus just on areasof diagnostic difficulty. and autistic disorder as separateentities, these disorders com- Textbook casesof autistic disorder or developmental lan- monly co-occur, and so it is import~nt to evaluate social com- guagedisorder are easyenough to recognize,but many children munication, play and repetitive behaviour in children with a presentwith a pattern of symptoms that doesnot fit unambigu- global developmentaldelay. ously in either category, while showing some features of both. Thus their difficulties extend beyond the highly selectiveimpair- Autistic disorder and related cond'tion S I ment of language structure seen in developmental language dis- order, but they do not have the full triad of autistic impairments Delayed language development and poor comprehension are in severeenough form to merit a diagnosisof autistic disorder. hallmarks of autistic disorder, and the issueof differential diag- O'Hare et al. (1998) carried out an audit of 103 children re- nosis between autistic disorder and specific developmental ferred to a speechclinic at Edinburgh Children's Hospital, and language disorder frequently crops up in clinical settings. A found that eight of them met diagnostic criteria for autism, but a diagnosis of autistic disorder should be suspectedif the child's further 14 had autistic symptomatology that fell short of meet- comprehensiondifficulties are accompaniedby more pervasive ing diagnostic criteria, in most casesbecause only two elements difficulties affecting social interaction, non-verbal communica- of the triad of autistic impairments were present.All but one of tion and play, or if the child shows unusual repetitive or ritualis- thesechildren was rated as having abnormal receptivelanguage . tic behaviours, or restricted interests. The clinician needs to on a speech therapy assessment.Although the diagnosis of consider whether language development is merely delayed, or Asperger syndrome is sometimesusedin such cases,this is not whether thereare deviant featuresthat would not be regardedas appropriate if the child's languagemilestonesare delayed. normal at any age, such as repetitive use of stereotypedcatch- In the UK, the term 'autistic spectrum disorder' is usedquite phrases,unusualand exaggeratedintonation, pronoun reversal, widely, although often without clear diagnostic criteria. In the and a frequent failure to respond when the parent attempts to USA, and increasingly elsewhere,the DSM-IV (American Psy- attract the child's attention. An intriguing observation in some chiatric Association 1994) term 'pervasive developmental dis- children with autism is that scoreson tests of expressivelan- order not otherwise specified' (PDDNOS) is frequently applied. guage(suchas picture naming) may be higher than those on re- However, this is not very satisfactory,asthis was clearly intend- ceptive tests (suchas selectinga named picture). Whereasmost ed as a default category to be used in rare caseswhen a child children with communicative problems will use non-verbal showed autistic symptomatology but diagnostic criteria for meansof expression,children with autism often have difficulty autistic disorder were not met. Furthermore, it provides little in- in both interpreting and producing appropriate non-verbal formation about symptomatology and does not help decisions communication. Imaginative play doesnot developnormally in about educational placement. Bishop (2000) suggestedthat children with autism; instead there may be repetitive routines, thesedifficulties probably reflect the fact that diagnostic labels suchasforming long lines of toy cars, or the child may be preoc- impose a categorical structure on what is in reality a multidi- cupied with everyday artefacts such as lights or switches, and mensionalspace, with children varying in terms of the severityof disregardstoys that most children would find attractive. impairments in language,social interaction and range of inter~ 668
  • 7.
  • 8. ~ CHAPTER 39 Researchhas shown that measures obtained from spontaneous (1994b) offered guidelinesthat still seemrelevant in the light of speech samples are useful in identifying children who are current research:children whose expressivevocabularies con- deemedlanguageimpaired but who do not meet conventional sist of lessthan 50 words at the ageof 24 months should becare- psychometric criteria (Dunn et al. 1996). Unfortunately, such fully monitored, but long-term problems are unlikely in those measuresare time-consuming and not always practicable in with vocabularies of more than eight words who have good clinical settings. Furthermore, adequate normative data are comprehension. often lacking. There are a number of longitudinal studies showing that the In the final analysis,the specificcriteria adopted for identify- child whose language is significantly impaired at 4-5 years of ing languageimpairment will dependpartly on one'sgoalswhen age is at high risk of developing literacy problems (Bishop & making the diagnosis. Stringent criteria developed in research Adams 1990; Tallal et al. 1997; Stothard et al. 1998; Johnson contexts are not always appropriate in clinical settings, where et al. 1999). Although it is commonly believed that oral lan- the goal is to provide a diagnosis that ensures the child has guage problems disappear with age to be replaced by literacy access appropriate services. to Here, one wants to usemeasures problems, this is seldom seen.Rather, the oral languageprob- that haveecological validity-that have relevancefor function- lemsbecomelessobvious in casualinteractions, but can beread- ing in everyday communicative and social settings-and to ily demonstrated on formal testing. It would be wrong to give identify those children who will benefit from intervention. If the impression that all language-impaired4 year olds are des- one requires a large discrepancy between verbal and non- tined for academic failure: some children do show marked verbal ability before children can be considered for special improvement. However, these tend to be children with pre- educational services, then many children with poor verbal skills dominantly expressivedifficulties that have resolved by the age are denied access,even though their linguistic problems may of 51/2years (Bishop & Edmundson 1987; Bishop & Adams be identical to those of other children who do meet diagnostic 1990). criteria. DLD is associatedwith increasedrisk for psychiatric as well as language and literacy problems. The underlying nature of D I t I d this associationhas beenthe causeof much speculation (Rutter eveopmena course prognosIs an " . & Lor d 1987) Comor b1 lty cou ld reflect t he m fl uence 0 f .d. ° A number of longitudinal studies have thrown light on the de- common aetiological risk factors, or the causal effect of one velopmental course of DLD. There is general agreement that condition on the other (Beitchmanet al. 1996). For instance,for the child with significantly impaired receptive language skills some children affective disorders and low self-esteem may hasa poor prognosis,evenif this diagnosisis madeat a very early be a consequenceof growing awareness of communicative age.Comprehensionproblems do not usually appear to resolve inadequacy. spontaneously.On the contrary, the range of impairments seen in a child with receptive language difficulties often increases .h d. "I d h. ° d. I Prevaence wIt age, exten mg to encompasssocIa an psyc IatriC Isor- der (Rutter & Mawhood 1991), and impairment on non-verbal Two recentepidemiological surveys,in the USA and Canada,es- as well as verbal measures(Stothard et al. 1998; Johnson et al. timated the prevalenceof specificlanguageimpairment (SLI) in 1999). There is muc;h more debate about prognosis for pre- 5 year olds at around.7% (Tomblin et al. 1997; Johnson et al. school expressive languagedisorders,with someproposing that 1999). However, it should be noted that neither study adopted outcome is generallygood and intervention is seldomwarranted the stringent 'discrepancy' criteria of DSM-IV and ICD-I0, but (Paul 2000), while others maintain that these children are at rather diagnosedSLI if the child scored below cut-off on stan- high risk for persistingdifficulties that may only becomeappar- dardized languagetests,but had a non-verbal IQ of 80 or above ent on detailed testing (Scarborough& Dobrich 1990; Rescorla and no other exclusionary criteria. Furthermore, in the study by etal.1997). At leastsomeof this controversy arisesbecause dif- Tomblinetal., only 29% of thosediagnosedascases ofSLIwere ferent studies have used different follow-up periods to assess already known by parents to have any speechor languagediffi- prognosis. In the longitudinal study conducted by Paul et at. culties. This estimateis likely therefore to be higher than would (1996), the initial impressiongiven by the first wavesof follow- be the caseif it were basedon a definition such as DSM-IV or up was that 2 year olds with expressivelanguagedelayswere at ICD-10, which requires both that there be a substantial dis- high risk of persisting communicative problems. However, the crepancy between verbal and non-verbal ability, and that the longer the follow-up, the smallerthe proportion of children who language impairment interferes with everyday or academic had marked languageimpairment. It appearsthat the numbers functioning. of children with clinically significant language difficulties do shrink dramatically as children mature. It seemsreasonableto R kf t " d t I o . . . IS ac ors an ae 10ogy conclude that a good long-term prognosIs IS usually seen m children identified as'late talkers' before the ageof 3 years,pro- The principal risk factors for DLD are: vided the problem is restricted to expressivelanguageand the. male gender-in clinical samples, sex ratio of affected delay is not too severe(Whitehurst & Fischel 1994). Bishop males:femalesisaround30r4:1 (Robinson 1991); 670
  • 9. ~ -, SPEECH AND lANGUAGE DIFFICULTIES . family history of DLD-around 30% of affected children should not lead us to conclude that environmental factors are have an affected first-degree relative, compared with 3% of the unimportant, or that nothing can be done to alleviate language general population (Stromswold 1998); and difficulties. Provisional evidence suggests genes may act as risk . being a later-born child in a large family (Bishop 1997a). factors that increase the probability that a child will have a Although much has been written about language outcomes of languag~ disorder, but the severity and persistence of language medical risk factors, such as otitis media and low birth weight, disorder can be highly variable, even within a pair of genetically thereisnostrongevidencethattheseactasmajorriskfactorsfor identical monozygotic twins. A study by Bishop et al. (1999) specific DLD, although they may act synergistically to cause im- suggested that environmental factors could impair the child's pairment in a child who is already at risk from other causes ability to process non-verbal auditory stimuli, with a subsequent (Bishop 1987). Although there is an association between low so- small knock-on effect on language development. In children cioeconomic status and DLD (Fundudis et al. 1979), this is not who were not at genetic risk, this negative effect was not of strong, and there is little support for the commonly held view clinical significance, but in those who were at genetic risk, that parents can cause their child to become language impaired for whom language learning was a more difficult task, the by inadequate verbal stimulation, except in the most extreme combination of environmental and genetic risk factors was suf- cases of abuse and neglect. ficient to lead to clinically important problems in language Over the past decade, there has been an explosion of research learning. concerned with the genetic basis of DLD (see Bishop, 2001 for a review). Three twin studies have obtained closely I t t o slml " " I ar fi n d mgs " 0 f h Ig h h enta " o b I l Ity o o f or t h IS d Isor d er o " (L ewls & o n erven Ion Thompson 1992; Bishop et al. 1995; Tomblin & Buckwalter Intervention is usually carried out by speech and language 1998). A molecular study of a three-generational family show- therapists, who use a wide range of techniques to stimulate lan- ing an autosomal dominant pattern of inheritance for severe guage learning. In the past, there was a vogue for drilling chil- speech and language disorder found clear evidence of linkage to dren in grammatical exercises, using imitation and elicitation a site on chromosome 7 (Fisher et al. 1998), although it is un- methods, in an attempt to have the child extract the salient clear how far these results will generalize to other cases of heri- grammatical regularities. Such methods fell into disuse when it table language disorder. became apparent that there was little generalization to everyday Currently, there is considerable interest in two aspects of lan- situations. Contemporary approaches to enhancing develop- guage functioning that have been postulated as behavioural ment of language structure are more likely to adopt 'milieu' markers of heritable SU. The first is phonological short-term methods, in which the intervention is interwoven into natural memory, typically assessed by asking the child to repeat non- episodes of communication, and the therapist builds on the sense words of increasing length, such as 'hampent' or 'blonter- child's utterances, rather than dictating what will be talked staping' (Gathercoleetal.1994). Poor performance on this task about. In addition, there has been a move away from a focus characterizes many children with SU, even those who had early solely on grammar and phonology toward interventions that difficulties that appear to have resolved. Furthermore, deficient develop children's social use of language, often working in performance on non-word repetition showed very high heri- small groups that may include normally developing as well as .tability in a twin study (Bishop et al. 1996). The second area in l~nguage-impaired peers (Gallagher 1996; Hayden & Pukonen which many children with DLD have disproportionate difficul- 1996). ties concerns certain aspects of grammar. Children with DLD Another way in which modern approaches to remediation dif- can have major problems in adding appropriate verb endings, fer from the past is that parents are more likely to be directly in- such as past tense '-ed', when given an eliciting sentence frame volved, particularly with preschool-aged children (Girolametto (e.g. Q: 'Here the boy is raking the leaves. What has he just et al. 1996). Methods such as the Hanen approach" involve done?' A: 'Raked the leaves') (Rice2000). Rice & Wexler (1996) videoing interactions between parent and child and then using have postulated an underlying impairl11ent of an innate system these when working with groups of parents in a constructive that has evolved to handle specific types of grammatical rule. way to help them facilitate communication. With the exception of non-word repetition, the measures used to A radically different approach has been developed by Tallal assess such hypotheses are not available as standardized tests, et al. (1996), who have devised a computer-based intervention, making them 1nsuitable for clinical use at present. Nevertheless, FastForword, that involves prolonged and intensive ttaining on the work is promising in suggesting that we may be able to specific components of language and auditory processing. The develop more selective language measures that will identify theory underlying this approach maintains that language diffi- homogeneous groups of children with a common aetiology. culties are caused by a failure to make fine-grained auditory That would be a considerable improvement on the current discriminations in the temporal dimension, and the training position, where the same child mayor may not receive a diag- materials are designed to sharpen perceptual acuity, in much the nosis of DLD depending on which tests are used to assess verbal ability. The fact that there is sttong genetic influence on DLD "See website at http://hanen.velocet.ca/programs_parentoshtml 671
  • 10. CHAPTER39 sameway as has been demonstratedin animal experiments. By phonological teststhat require no speechfrom the child. Some embeddingtraining in attractive computerized games,children children with phonological problems have difficulties in dis- can be persuadedto participate in thousands of training trials, criminating between similar speechsounds, such as 'pat' vs. in a way that would simply not be possible with a standard 'cat', when asked,for instance,to selecta picture to match what therapist-basedinteraction. they haveheard. However, the most common difficulty is not so For all thesetypes of intervention, there are few adequately much in telling soundsapart, asin recognizingthat different ex- contl;olled trials that allow one to assessclinical efficacy.In gen- emplars of the same sound are indeed the same sound. So if eral, one does not seemiracle cures, but this is not to say that asked to say whether 'bag' or 'boat' rhymes with 'rag', or to gains are negligible (Bryne-Saricks 1987; Law et at. 1998). A judge whether 'soup' or 'coat' begins with the same sound as clinical trial assessing FastForword approach showed sig- the 'Sam', the child with phonological problems may perform at nificant gainsrelative to a control group (Merzenich et at. 1996), chancelevels (Bird et at. 1995). Suchobservations suggestthat but questionsremain about the persistence and generalizability the difficulty is one of categorization of speech,rather than poor of theseeffects.Sincethe initial controlled trial, the authors have acuity for differencesbetweenspeech sounds. gathered a large amount of data on pre- and post-intervention The prognosis of pure phonological disorder is much better language test scoresof children enrolled in FastForword, but than that of languagedisorder (Bishop & Adams 1990;Johnson this is difficult to evaluate without controls for practice and et at. 1999), especiallyif the phonological difficulties resolveby placebo effects (TallaI2000). Furthermore, it is not possible to the time the child starts school (Bird et at. 1995). It is difficult know which specific components of this complex intervention to estimate the prevalenceof phonological problems, because are most effective,or whether the whole gamut of different exer- studies typically do not discriminate betWeen different types of cisesis essentialto achievetherapeutic benefits. speech problem; lisping and other deviations,specificphonolog- ical impairments, and speech problems accompanyinglanguage D I t I h I . I d. d impairment all tend to be included together. Furthermore, eve opmen a p ono oglca Isor er . prevalenceappearsstrongly age-dependent, with speechprob- It is customary to draw a distinction betWeen speech, physi- the lems declining sharply betWeen3 and 6 years of age (Morley cal act of articulating speechsounds, and language,the whole 1972). After excluding children with additional handicaps, complex system of combining elements of sound at different Johnsonetat. (1999) obtained a prevalence estimateof6.1 % for levelsof complexity to expressmeaning. It is possibleto have a specific speech-onlyimpairments at 5 years of age.This figure languageimpairment with normal speech(e.g.in cases DLD of excludes the children from this sample who had comorbid where the child speaksclearly but doesnot comprehendor pro- speechand languageimpairment. Shriberg et at. (1999) report- ducecomplex syntactic constructions). The conversesituation is ed a prevalenceof speechdelay in US 6-year-oldsof 3.8%, with also seen,when the child has somedifficulty in producing clear comorbid languageimpairment in around 12% of thesecases. speechbut the underlying languageskills are intact, e.g. in cases Little is known about risk factors and aetiology of phonological of dysarthria (seebelow). The child who persistsin using imma- disorders, although, as with other communication disorders, ture or deviant sound patterns but who has no physical b~sis boys are at greater risk than girls (Shriberg et at. 1999). Inter- for this disorder does not fit so neatly into this dichotomous vention is carried out by speech-languagetherapists, and view. Speechis undoubtedly the prese~ting problem, but the typically involves games and exercisesto develop the child's underlying impairment appearsto be linguistic rather than one awareness phonemic contrasts (Deanet at. 1995). of of motor control: a failure to learn which speech soundsare dis- tinctiv.ein the am.bientlanguage.Often th~ speech errors involve Developmental verbal dyspraxia a persistenceof Immature patterns. For Instance, sounds pro- ducedin the back of the mouth, suchas 'k' and 'g' are not distin- Developmental verbal dyspraxia is a controversial diagnostic guishedin the child's output from those produced in the front of category that is defined differently by different experts,and not the mouth, suchas't' and 'd', so that 'cat' may be pronounced as usedat all by someauthorities (fora review seeCrary 1993). The 'tat' and dog as'dod'. The terms 'phonological disorder' (DSM- central characteristicin most definitions is that there aredifficul- IV) and 'phonological impairment' have superseded such labels ties in speechproduction that suggestan impairment of motor as 'functional articulation disorder' to refer to such problems. programming, because is the length and complexity of what is it The term 'phonological' implies that the child's difficulties are uttered, rather than the specific speechsounds used,that is the linguistic rather than motoric, perhapsakin to thoseof an adult main factor determining accuracy.In children with this diagno- mastering a foreign language. Most of us have difficulty in sis, one is likely to seespeecherrors that are inconsistent from learning to use a new set of speechsounds, not becauseour one occasion to the next, that are particularly evident in poly- articulators are in any way defective, but because have not we syllabic words, and that involve transpositions of speech internalized the sound distinctions that are critical in the soundsrather than simple substitution of one soundfor another. language. For instance, Bradford & Dodd (1996) reported a dyspraxic Evidencethat a phonological disorder is not just a problem in child whoserenderingsof 'elephant' on three separateoccasions articulating soundsaccuratelycan be obtained using specialized were 'ewint', 'wuwit' and 'uwit'. 672
  • 11. r" SPEECHAND lANGUAGE DIFFICULTIES Debate continues over the question of whether problems in Drought syndrome.However, Clark et at. (2000.),in a recentre- sequencingnon-verbal movementsshould be part of the diag- view of 47 cases,noted that most children with this condition nostic criteria: someauthorities maintain that to be regardedas have additional complex impairments, including mild pyrami- dyspraxic, the child should be impaired in imitating sequences dal tetraplegia, learning difficulties, behaviour problems and of non-speech movementsof the tongue and mouth. Not all chil- epilepsy.Crary (1993) noted that remarkably little clinical or re- dren who make inconsistentphonological errors haveextensive searchattention has beenpaid to developmental dysarthria, al- difficulties producing non-speech movements,raising the ques- though it was well describedby Morley et at. (1954) more than tion of where they should beclassified(Bradford & Dodd 1996). 40 yearsago. It appearsto be a strongly familial condition. In addition, there is the question of whether dyspraxia should be Dysarthria and anarthria involve difficulties affecting speech diagnosedin a child who hasbroader difficulties with expressive rather than language,and so one would expect to find normal language, or only in those with a relatively pure problem in language comprehension and normal literacy skills in pure speech output. In practice, many children who receivethis diag- cases.However, quite often the aetiological factors that cause nosis do have associatedproblems affecting language, literacy articulation difficulties also lead to problems in other areas,in- and phonological awareness (Stackhouse1992). cluding hearing and language. The lack of agreeddiagnostic criteria make it impossible to ma~e generalizations about risk factors, prevale?ce or .prog- Selective mutism nosls.The causeof developmentalverbal dyspraxia remains an enigma, but it appearsto be strongly familial (Morley 1972). It Selectivemutism is diagnosedwhen a child is able to speakbut is also worth noting that although their grammatical difficulties fails to do so except in very restricted situations, such as with have beenemphasizedin published accounts, the phenotype in close family. This disorder was previously known as 'elective the three-generationalfamily mentioned in the section on DLD mutism', but the terminology was modified in DSM-IV to avoid also involved severely dyspraxic speech (Hurst et at. 1990). the connotation of volitional behaviour.The diagnosisis strong- Crary (1993) provides'an overview of approachesto interven- . lysuggestedwhen one finds mutism in a child whohasnoneuro- tion for developmentalverbal dyspraxia. logical or structural abnormalities of the articulators, and who has normal languagecomprehension,as well as a normal early A th . dd th . historyof usinglanguage. However, crucialpoint that needs the nar ria an ysar ria b bl h d . h h hold d o k. .. to eesta IS e 1St att ec I oesspea In somesituations. Anarthria or dysarthria is diagnosed when speech problems As Dummit et at. (1997) noted, this condition is more pro- arise becauseof structural or neurological abnormalities of perly regardedasa form of anxiety disorder rather than a speech articulatory control. Anarthria is the term used when there is and languagedisorder. Rates of comorbid anxiety and phobic no ability to produce speech,whereas dysarthria refers to dis- disorders are high, both in affected children and in their first- ordered articulation caused by weakness, incoordination or degreerelatives. However, differential diagnosiscan be compli- structural abnormalities of the articulators. It is important to cated by the fact that some children with selectivemutism do distinguish these articulation problems, where speech is im- have developmentallanguagedisorders (Kristensen2000), sug- paired because problems producing articulatory movements, of gesting that self-consciousness about inadequate communica- from developmentalphonological disorders, where the child is tion skills may play a.part in maintaining mutism. neurologically normal and capable of producing articulatory Persistent selectivemutism affects less than 1 in 1000 chil- movements(seeabove). Neurological conditions that can cause dren, although the frequency of transient mutism in children anarthria or dysarthria include cerebral palsy and Mobius starting school is much higher.Girls are two to three times more syndrome,in which there is agenesis cranial nerve nuclei and of likely to be affectedthan boys.The causes selectivemutism re- of associated facial immobility. Structural abnormalities of articu- main unknown. Although conventional wisdom maintains that lators that can lead to dysarthric speech include cleft palate and physical or sexual abuseor other kinds of trauma may precipic TreacherCollins syndrome.There are a number of other genetic tate selectivemutism, there is little evidenceof this (Black & syndromesthat are associatedwith unusual proportions of the Uhde 1995), and the strong familial component to the disorder articulators and/or hypotonicity which affects tongue control, suggests that Dummit et at. (1997) may be correct in regarding e.g.Down syndrome. this disorder as the extreme end of a biologically basedcon- Where no specificsyndromeis detected,one should bealert to tinuum of temperamentand social behaviour. the possibility of dysarthria when there is poor co-ordination or Behaviour modification methods have been shown to be ef- weaknessof facial muscles,as evidencedby drooling, feeding fective in re-establishing speech(Sluckin et at. 1991), but the problems, or difficulties imitating simple oral movementssuch long-term prognosis of selectivemutism is neverthelesspoor. as moving the tongue from side to side or pursing the lips. There is a high rate of personality disorder and psychiatric prob- Worster-Drought (1974) stressedthat anarthria can occur in lems associatedwith a history of selectivemutism (Kolvin & children in the absenceof any other neurological impairment, Fundudis 1981). Dummit et at. (1997) argued that therapeutic and he gave detailed descriptions of this condition, which is interventions should focus on alleviating anxiety, but there has known as both congenital suprabulbar paresis and Worster- beenno systematicresearchon the efficacyof this approach. 673