3. Definition
•IDEA (Individuals with Disabilities Education
Act) 2007 defines hearing loss:
• Deaf child is one who cannot understand
spoken communication even with a hearing
aid.
• A hard of hearing child is one who can hear
spoken communication, but this does not
necessarily mean that he understands it.
4. Definition
•Permanent childhood hearing impairment (PCHI):
confirmed permanent B/L hearing impairment
exceeding 40 d.BHL (average of pure tone
thresholds at 0.5, 1,2 & 4 kHz in better hearing ear).
•Congenital or acquired
•Prelingual deafness Vs post-lingual deafness -for
the child's education and management
5. Degree of hearing loss
•0-15 dB Normal
•15-25 Minimal Hearing Loss
•26-40 Mild Hearing Loss
•41-55 Moderate Hearing Loss
•56-70 Moderately Severe
Loss
•71-90 Severe Hearing Loss
•>90 dB Profound Hearing Loss
6. Common Misconceptions Held by Public and
Clinical Facts
Misconception
• Parents will know if their child
has a hearing loss by the time
their child is 2-3 months of age
• Parents can identify a hearing
loss by clapping their hands
behind the child’s head
Clical Fact
• Priror to the universal screening the
average age at which children were
found to have a hearing loss is 2-3
years. Children with mild to moderate
hearing loss were often not identified
until 4 years of age
• Children can compensate for a hearning
loss. They use visual cues such as
shadows or parental expressions and
reactions, or they may feel the breeze
caused by the motion of hands.
7. Misconception
• The HRR is all that is needed to
identify children with hearing
loss
• Hearing loss does not occur
often enough to justify the use of
universal screening programs
• Tests are not reliable and cause
too many infants to be referred
to specialists
• There is no rush to identify a
hearing loss. The loss does not
need to identified until a child is
aged 2-3 years
• Children younger than 12 month
cannot be fitted with hearing
aids.
Clical Fact
• The HRR misses approx. 50% of all
children with hearing loss
• Hearing loss affects approx. 2-4 per
1000 live birth and it has estimated to
be one of the most common congenital
anomalies
• Referral rate are as low as 5-7%
• Children identified when they are older
than 6 months can have speech and
language delays. Children identified
when they are younger than 6 months
do not have these delays and are equal
to their hearing peers in terms of
speech and language
• Children as young as 1 month of age
can be fit with and benefit from hearing
aids
8. Epidemiology
• One child in 1000 born - bilateral permanent childhood hearing
impairment (PCHI)
• About 60% of these children= mod, Hearing loss(41-60 dBHL)
• Remainder have severe (61-80 dBHL) or profound (>8ldBHL)
(www.who.int/pbd/dcafncss/hcaringjmpa irmcncgradcs/ en/index)
• The prevalence of PCHJ increases with age-s further 1 in
l 000 develop acquired or progressive hearing impairment.
• PCHI- one of the most common abnormality present at birth
• The prevalence of bearing Loss- greater than that of most other diseases
and syndromes (eg, phenylketonuria, sickle cell disease) screened at
birth.
9. The risk factors for newborn:
•(Joint Committee on Infant 1-Iearing (JCIH) 2000
Position Statement)
• »Farnily h/o permanent childhood sensorineural hearing
loss
•»In utero infection - cytornegalovirus, rubella,
toxoplasmosis or herpes
• »Craniofacial anomalies- morphological abnormalities of
the pinna & EAC
•»Neonaral indicators - hyperbilirubinernia,
persistent pulmonary
• hypertension of the newborn (PPI-fN), mechanical
ventilation
• Postnatal infections - bacterial meningitis
10. Universal newborn hearing
screening (UNHS)
• National Institute for deafness and other communicative
disorders (NIDCD)
• - consensus conference -on early identification of hearing loss-
1993
• •Recon11nended -UNHS
• Screening I•• three months of life
• Taken up by various agencies -
• American academy ofotolaryngology- I-INS
• American academy ofspeech and language
• American academy ofaudiology
• :'Pass" or "Refer,"
• ."Refer" -
• follow up testing
• confirmed 7 referral to otolaryngologist
11. Efficacy of Early Identification and
Intervention
•Initially-
•- In 1988 -hearing loss identified in children in the
USA- 2.5 yrs
•- Severe to profound hearing loss or with multiple
disabilities identified at or
•before age 2.5 yrs
•- Mild-to-moderate: not identified until school
•Universal newborn hearing screening program 1997-
2001
•Mean age of diagnosis - 3.9 months
•Mean age of intervention - 6.1 months
12. • }>Definition ofearly identification and intervention - evolved over the
years
• }>Early identification -defined as intervention before the age of J 8
months
• }>The implementation of universal screening programs "?definition of
early identification and intervention re-examined
•>'- Early identification - diagnosis as early as age 3 months
• }>Early intervention - by age 6 months
• }>Better language scores in severe- profound deaf children- hearing loss
identified at an average age of l l .9 months, compared to l9.5 months
(White SJ and White RE, l 987)
• }>Children identified and wore bearing aids by the age of 6 months"?
acquired vocal communicative and linguistic skills better than children
identified at a later age
(Robinshaw HM. 1995)
• }>Critical period ofearly identification & intervention- younger than 6
months
(Yoshinago et al., 1998)
13. •}>Better language scores in severe- profound deaf
children- hearing loss identified at an average age of l
l .9 months, compared to l 9.5 months
(White SJ and White RE, l
987)
•}>Children identified and wore bearing aids by the
age of 6 months"? acquired vocal communicative
and linguistic skills better than children identified at
a later age
(Robinshaw HM. 1995)
•}>Critical period of early identification &
intervention- younger than 6 months
(Yoshinago et al.,
14. Delayed Diagnosis
• Permanently impaired speech and
language skills
•Reduced intellectual ability
• Lowered adult earnings
• More limited social skill
development
15. (Joint Committee on Infant Hearing (JCIH) 2007
Position Statement)
1. Definition of targeted hearing loss expanded to include neural hearing loss (eg.
auditory neuropathy/dyssynchrony)
2. Separate protocols for neonatal intensive care units (NTCUs) and well-baby
nurseries. ABR screenings for all N1CU babies, as well as babies admitted for >5
days-sueural bearing loss will not be missed.
3. Referrals for all infants who do not pass ABR screening in the NlCU.
4. Rescreening of all infants should include re-evaluation of both ears, even if the
infant only failed one ear in the initial screening.
5. Audiologists with expertise in evaluating newborns should conduct diagnostic
evaluations.
6. Children identified with hearing loss 7 fit amplification within l month of
diagnosis.
7. .A genetics consultation to families ofinfants with hearing loss.
8. Children with bearing loss should "?evaluation by an otolaryngologist &
ophthalmologist.
9. Children with any degree of bilateral or unilateral hearing loss "?Consider early
intervention services
10.. Families "?all communication options and available hearing technologies
11.J. Early intervention services 7 by professionals with expertise in bearing loss
16. Methods of Screening Otoacoustic
emission (OAE)
•Principle:-Response of outer hair cells to acoustic
stimuli
•- Assess cochlear integrity
•- Fast test for normal preneural cochlear function
•Set up:
•Probe assembly in EAC
•Tonal I click stimuli - delivered
•OAE - generated by cochlea
•Measured -microphone
17. If middle ear normal-s assesses cochlear function
in
frequency range 0.5-6kHz
-Fast, efficient, frequency specific
Limitations:
- Efficacy reduced by contamination of low
frequency
noise in busy nursery, wax/debris in EAC
- Middle ear pathology
-Auditory neuropathy-OAE 111ay be normal-s
ABR
- Stay in NICU >5 days-s ABR
18. •Automated ABR (AABR):
- Auditory function from 8th nerve 7 auditory
brainstem
Set up-
-Electrodes - forehead, mastoid, nape of neck
-Click stimuli - in the canal earphone - 35 dB
-Compare infant's waveform - normative ABR
infant data
-Response- pass I fail
-Screening tool - < 6 months
19. Advantages:
-Can be done -with background noise
-No interpretation required
Solely-screening technique 7identify
who require follow up testing
Disadvantages:
-Lacks frequency specific information
Can't differentiate type/degree of hearing
Loss
Requires increased preparation time prior
to test
20. Diagnostic auditory brainstem
reflex:
-Not used in UNI-IS because of length of
procedure, cost, audiologist
-Unlike AABR-35 dB, Intensity of stimuli
varied -manual ABR
-Allows to know - severity of hearing loss
-Type of hearing loss
-Not used for screening but for follow up
21. Follow -up testing
..Infants who do not "pass" 7F/U at l month
interval
..F/U allows7multiple testing sessions, medical
intervention, parental counseling, appropriate
amplification before age of 6 months
,.If flu delayed - need for sedation increases
If infant fail 2nd screening session -diagnostic
ABR/OAE
;..Auditory neuroparhy/dyssynchrony- Nonna)
OAE
but abnormalABR
22.
23.
24. Hearing loss
Syndromic hearing Loss
1/3 syndromic
Dominant
Waardenburg
BOR
Sticker’s
NF2
Teacher Collins
Recessive
Usher
Pendred
Jarvell/Lange-Nielsen
X-linked
Alport
Mitochondrial
Chromosomal
Down Syndrome
2/3 syndromic
75% Recessive
23% Dominant
23% X-linked
1% Mitochondrial
Non syndromic hearing
loss
25. Disabilities that occur with deafness (%)
Dearness with No Other Disabilities 60.1
Learning Disability 10.7
Intellectual Disability 9.8
Attention Deficit Disorder (ADD/ADHD 6.6
Blindness and Low Vision 3.9
Cerebral Palsy 3.4
Emotional Disturbance 1.7
Other Condition 12.1
(Gallaudet Research Institute, Jan 2003
26. Syndromic hearing Loss
• Pendred
• Usher
• Branchio-oto-renal
• Waardenburg
• Jervell and Lange-Nielsen
• Alport
• CHARGE association
27. Non syndromic hearing Loss
• Isolated genetic HL without any
other recognized abnormalities
• Two thirds of all congenital SNHL
• Over 50 genes; new ones
discovered every year
• Testing available for only a small
few
28. Connexin 26 (GJB2)
> Most common hereditary SNHL
>Causes 50o/o of non-syndromic SNHL in the
US and Europe.
> Recessive inheritance
> 35delG -extremely common (2.5%
carrier rate)
>SNHL variable (severity, symmetry,
progression).
>Genetic testing widely available
29. Inner ear malformations
30- 35% of patients with SNHL – have
malformations
Even higher% in unilateral SNHL
Malfon11ations can occur in isolation, or as
part of a syndrome
Why is diagnosis important?
Risk of meningitis
Risk of progression ofHL Complicates
cochlear implantation
30. Inner ear malformations
7Michel aplasia: most severe, complete absence of
bony & membranous labyrinth
7Mondini's aplasia: only basal coil present
7Sheibe's (cochleosaccular)dysplasia: most
common, dysplasia in cochlea & saccule
7Bing-Siebenn1an dysplasia: complete absence of
membranous labyrinth but bony labyrinth
present
7Alexander's dysplasia: Limited cochlear duct
differentiation at the level of the basal coil
7Enlarged vestibular aqueduct: early onset SNHL,
progressive
7Se111icircular canal malformations
31. Evaluation
History:
Presentation depends on:
• Degree of hearing loss Patient's age
• When bearing loss began ?
• Threshold of suspicion by parents
- Presence of identifiable risk factors
- 2/3 rd cases - parent’s I suspicion
IO % cases - Paediatrician
15 % cases - health caregivers
Meantime b/w Is, suspicion and diagnosis -9 n1onths
32. Evaluation of hearing loss:
Concern over child's hearing
Behaviow·al problems/personality defects
Congenital/postnatal - profound deafness - loss
of cooing by age of 6-9 months
Minor speech impediments, school failure
Mental retardation, autism, attention deficits,
adjustment disorders
34. Important Elements
,.
Perinatal history
,.Family history
Neonatal history
•Prematuri ty
•NICU
-Mechanical ventilation
-Infections
•Hyperbilirubinemia (transfused ?)
•TORCHS/maternal infections
35. Usually normal
Detect syndromic features
Pigment anomalies (Waardenburg)
Ear pits, branchial anomalies (BOR)
Abnormal external ear (CHARGE,
Craniofacial abnormalities
Pinna
- Microtia
- Canal atresia
Physical Examination
38. Aim of aetiological investigations
Aim:
• Try answer parents - "why is my chi Id
deaf?”
• Identify and treat medical conditions
• Assist early decision making
Appropriate communication modes
Educational placement & counselling
• To inform genetic counselling
- Epidemiological research
39. Laboratory Studies
•Depending - patient's history & physical findings-
s biochemical
evidence
•Diagnosis of SNHL-
-Tcsting thyroid function, measuring BUN and creatinine levels and
urinalysis.
•ECG - diagnosing- arrhythmia- Jervell Lange-Nielsen syndrome
• BIL hearing loss - (eg, ESR)
-Autoimmune inner ear disease
-Generic screen
40. •Serology (suspected viral, autoimmune, syphilitic)
·CBC
Risk ofthalassemia or sickle cell disease
Macro thrombocytopenia & leukocyte inclusions (a/wAlport syndrome)
-Biochemistry
BUN & electrolyte abnormalities with renal dysfunction (e.g., Alport
syndrome)
Lipid profile
Glucose
-Thyroid function tests
Congenital or acquired hypothyroidism
Pendred syndrome
-Autoimrnune work-up
ESR Imrnunoglobulin
Complement
41. • Non-contrast CT of temporal bone
Gold standard
Quick but may require sedation
Involves ionizing radiation Excellent
for almost all causes
• MRI
Detect abnormality of CNS Certainly
indicated in some unusual cases
43. Assessmentof hearing
Arousal test:
Sound stimuli 7light sleep"? arousal
Auditory response cradle(ARC):
Baby in cradle-behaviour in response to
sound
stimulus-strunk & limb movement, head
jerk and
respiration
44. Electrophysiological testing:
- Key developmental age: 0 -6 months
- Mainly employed -Newborn screening
- Preschool screening /surveillance
- Diagnostic testing - l" 6 months
(Sininger et al.. 2003)
Also used -BOA fails to give reliable results
- Confirm hearing threshold -prior fitting bearing
aid /cochlear implant
Audiometric Evaluation: when & what to do?
45. Behavioural observation audiometry (BOA):
Key developmental age: 0 -6 months
• Sound stimuli-s response 7cbange in
behaviour e.g., alerting,
widening of eyes or facial grimacing, arousal
from sleep
•Auropalpebral reflex
•Moro reflex:
•Cessation reflex: cessation of an activity
-Use < 6 months -superseded by -OAE,ABR
46. Infant distraction test (IDT):
Key developmental age: 6-18 months
Normal response: Sound 7head turn to locate the
source of sound
Visual Reinforcement audiometry (VRA):
-Key development age -6 to 36 months
-Conditioning technique
-Child trained to look for an auditory
stimulus by turning head-reinforced by flashing
light or toy
47. Performance testing:
-Key developmental age: 2-5 yrs
-Used till cooperation with PTA achieved
-Child conditioned to wait for a sound � respond �
play activity
-Detennine -minimal threshold response
Pure tone audiometry(PTA):
-Key dcvelopmentaI age: > 3yrs
-lnsrructed to raise the corresponding hand - sound is heard
-CHL and SNHL can be differentiated
-Quick and easy screening test - effective tool in schools
-Disadvantages: formal evaluation takes tune/equipment
fully performed only -older, cooperative patients
49. Aetiology investigations in children with congenital bilateral sensorineural hearing loss
Core investigations (Level 1) - Modified
1. General History
a) Maternal health during pregnancy
b) Birth, perinatal, postnatal history
c) Exposure to nose, ototoxic, drugs, head injury, ear diseases , meningitis, viral illness, immunization
status
d) Developmental milestones – speech and languages, motor milestone
2. Family history
a) History of hearing loss or risk factors associated with hearing loss in atleast 3 generation to include
siblings parents and grandparents, uncles, aunts and first cousins wherever possible.
3. Clinical examination
a) Full physical examination including the head and neck, particularly eyes and ears, limbs including
hands and feet skin and nails, chest, abdomen, spine and cardiovascular system for any dysmorphic
features or abnormalities.
b) Age appropriate developmental assessment {child guidance clinic, psychiatry OPD)
4. Audiology
a) Age appropriate hearing assessment (BOA/play audiometry / PTA) and tympanometry
b) ABR and DAE ( once middle ear infection is ruled out)
c) Age appropriate hearing assessment of first degree relatives i.e siblings and parents
5. Ophthalmic assessment
a) Rule out non specific problems of squint and refractive errors
b) Clarify a diagnosis such as CHARGE association, Usher syndrome or congenital CMV or Rubella
6. Imaging
a) MRI of the inner ears and internal auditory meatus (IAMs) – left to cochlear implant team if CT
planned
7. Urine for microscopic hematuria – r/o Alport's syndrome
50. 8. Referral to clinical geneticist – test for connexin 26 & 30 and genetic counselling
Additional investigation (Level 2)
1. Hematology – Complete blood count – ESR
2. Biochemistry
1. BUN,S creatinine, electrolytes-suspected Alport or Alstrom syndromes
3. Serology
a) Congenital CMY infection
a) Babies <1yr – maternal 1gG
b) Babies > CMV 1gG
b) Congenital Rubella Syndrome
a) Babies up to 6 months-rubella specific 1gM
c) Congenital Toxoplasma Infection
a) Babies up to 6 months 1gM
d) Syphilis-can be carried out any time
4. Rental Ultrasound:
a) Suspected brachio-oto-renal syndrome
b) Family history of renal problems
5. HRCT scan of temporal bone-left to the cochlear implant team if CT planned
6. ECG – suspected jervell lange Nielson Syndrome
7. Immunology test
8. Metabolic screen blood and urinalysis
9. Chromosomal analysis
51. Algorithm
Known or suspected aetiology (e.g. meningitis,
trauma): �
a. At diagnosis:
•Oph tha I mology
•Additional tests and/ or treatments only as
clinically indicated by aetiology or clinical
course
b. Serial Audiograms
52. 2. Unilateral SNHL:
a. At diagnosis:
-Imaging study
-Ophthalmology
b. If imaging normal
COnsider Genetics referral
c. Serial Audiograms
53. Bilateral SNHL (unknown aetiology)
a. At diagnosis:
•Imaging of temporal bone
•Ophthalmology
-Genetics referral (ideally, specialist in I-
IL)
•EKG
•UA
-Labs if clinically indicated (rarely)
b. Serial Audiograms, other referrals
55. Management of associated disorders
-
Cardiac (prolonged QT) -awareness, medication
Meningitis vaccinations:
SN}IL population, particularly those with
malformations, at higher risk than general
population
• Thyroid disease
• Meningitis- steroids reduce the incidence of
SNHL and
improve survival
• Sudden SNHL- high dose steroids+/-
antivirals may
improve recovery of hearing if begun promptly
(controversy)
56. Management
Deafness-s heavy social & economic burden on individual,
family, community & country
Multidisciplinary approach:
Parents' reaction to the diagnosis:
-Shock, denial, anger, acceptance
-Dealt sympathetically
Subsequent management of the hearing impaired child:
I .Appropriate hearing aid selection
2.Proinotion of the development of language & communication
skills and if possible, speech development
57. a
Management
Medical:
CHL:
- Otitis media or its sequelae
- Otitis media with persistent effusion >3 months
- Obstruction of EAC
- If hearing loss continues 7 amplification
• bearing aid
• speech therapy
SNHL:
• Can't be medically treated
• Arnplificatiou with bearing aids
• Speech therapy may be beneficial
58. Surgical management:
-Some causes of CHL
-Persistent chronic or recurrent otitis media
-Cholesteatoma
-Bone-anchored hearing aid (BAHA):
- Microtia
- Anotia - auricular reconstruction
- Persistent otorrhea
SNHL can not be treated with surgical means
other than cochlear implantation
59. Hearing aids
• SNHLcan't be corrected to normal by any form of medical or
surgical Rx
• Some CHL- congenital abnormalities of the EAC & middle ear not
suitable for surgical Rx
• Birth of totally deafchild-rare-shigh powered hearing aids for
residual hearing
• Types ofhearing aids:
I. Personal hearing aids: body worn aids, behind tbe ear aids,
BAHA
II. Aids not entirely worn by tbe listener: speech trainer, group
hearing
aids, Radio (FM) hearing aids, infrared hearing aid system, loop
system
60. Cochlear Implantation
-Candidacy coordinated by audiologist
-Requires specific expertise
•Rapidly evolving field
-Much success in very young(< 18
months)
children
-Ongoing technological advances
and opportunities
61. Communication methods in the education of deaf children
1.Auralism: use only speech and lipreading as a means of
communication
-Signing- strongly discouraged or prevented
-Oralsits-ability to develop speech inhibited by allowing
signing
2.Finger spelling:
3.Cued speech:
M,P,B or K,D,L- not distinguished by lipreading alone
Different hand shapes in different positions close to the
speakers mouth to enable the child to discriminate the lip
movement
62.
63. 4.Signing system (manualism):
British sign language, American sign language
S.lotal communication:
Uses any and all modes of communication
(combination of speech,
gestures, signing, finger spelling, speech
reading/lip reading, reading
and writing)
Controversy:
-Sensory inputs (auditory & visualj-senhances language
development
•Total communication-s impair speech developement
64. The Deaf Community
Supportive, strong community
Does not view hearing loss as a
disability
Rich history and language
(e.g., American Sign Language)
> Children with cochlear implants may
have limited access to the deaf
community
66. Assistive Devices
):>Obtain devices - doorbells, timers, alarm
clocks and fire alarms
), Alerting devices
hearing dog
alarm clock with flashing light
devices producing strong vibration
>Telecommunication devices
Telephone amplifier
Telephone coupler attached to hearing aid
Telecommunication devices for deaf (TDD)
):>Teletypewriters (TTYs) machines - enable deaf people
to use the phone
67. Follow-up
>"' Deaf child-s Follow up
)Audiologist:
- Monitor progression
- Hearing loss
- Refit hearing aids – match changing losses/
growth of ears
)i> Pediatricians:
Monitor linguistic/ social development
);>Children who are deaf or hard of heating are at
particular risk
for abuse
