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Development in the Hearing      Impaired Child    Dr Varsha Atul Shah    Associate Consultant    Department of Neonatal & ...
Colorado Newborn Screening            Program                 Per 100 000 birthsCongenital HL    260 (200 bilateral)Hypoth...
Classification of Severity of    Hearing ImpairmentSeverity                dBMild                  27 - 40Moderate        ...
Hearing Impairment In InfancyEnvironmental          Genetic Causes  Causes (50%)           (HHI) (50%)                 Syn...
Environmental Causes   Perinatal infections       CMV, Rubella, other IUI   Meningitis       bacterial   Prematurity ...
Hereditary Hearing        Impairment (HHI) Congenital HHI          Delayed onset HHI Present at birth        Develop later...
Syndromic HHI   Chromosomal disorders       Down Syndrome       Trisomies   Non Chromosomal    disorders       Noonan...
Non-Syndromic HHI: the               genetics   Gene mutation   Genetic heterogeneity   “DeaFNess” gene loci       AR ...
Undetected Hearing Loss                 Hearing loss is a  Screening                 hidden disability                 whi...
The Importance of Hearing            Hearing is important for                 Language development                     ...
Unilateral or Mild Bilateral HL    Relatively minor disability    Speech recognition & understanding under     adverse c...
Children with minimal sensorineural           hearing loss:   Poorer comprehension & communication   Impairment in funct...
Moderate-profound HL   Impairment of speech/language    development   Reduction of academic achievement   Disturbance i...
Problems faced by HI Children               Hearing              Impaired              Children Language   Non – Language ...
Language Related Issues1.   When adults speak with the HI child        They tend to give less verbal praise and become   ...
Language Related Issues1.   When HI children seek clarification,        They tend to repeat rather than revise3.   HI chi...
Language Related Issues1.   HI children have poor sentence structure    Children with language difficulties     face diff...
Non-Language Issues    More physically–tired (more effort required)    Emotional outcomes- Poor self-esteem,     motivat...
Non-Language Issues1.   Behaviour problems        Aggression        Impulsivity        Immaturity        Inattention, ...
Assessment of Language   Proper diagnostic hearing assessment   Proper treatment i.e. aids / CI / AVT / sign   Hearing ...
Concomitant DisabilitiesSeveral disabilities occur more commonly in HIchildren  Intellectual / cognitive disability (8%)  ...
Concomitant DisabilitiesLearning disability (9%)      Diagnosis based on discrepancy between       intellectual potential...
Hearing Impairment & Learning   Previous studies showed that HI children have      Linguistic / language problems      ...
Early intervention is essential    Neural circuits of auditory system     rewired by external stimulations very     early...
ScreeningIdentification                 Breakdown at                   any stage  Follow up       jeopardizes             ...
The UNHS                              programme was                              piloted at the                           ...
Echocheck version 2.13.3ILO, Otodynamics(July 2000 - )
Echoport ILO 288, Otodynamics(Nov 1998 - )
Do not only hear mebut also listen to me…
Development in hearing impaired.rev
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Development in hearing impaired.rev

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Development in hearing impaired.rev

  1. 1. Development in the Hearing Impaired Child Dr Varsha Atul Shah Associate Consultant Department of Neonatal & Developmental Medicine, SGH Visiting Consultant K K Hospital,Child Development Unit
  2. 2. Colorado Newborn Screening Program Per 100 000 birthsCongenital HL 260 (200 bilateral)Hypothyroidism 25PKU 7CF 50 Pediatrics Jan 1998
  3. 3. Classification of Severity of Hearing ImpairmentSeverity dBMild 27 - 40Moderate 41 - 55Moderately-severe 56 - 70Severe 71 - 90Profound ≥ 91 American National Standards Institute 1969
  4. 4. Hearing Impairment In InfancyEnvironmental Genetic Causes Causes (50%) (HHI) (50%) Syndromic Non Syndromic (30%) (70%)
  5. 5. Environmental Causes Perinatal infections  CMV, Rubella, other IUI Meningitis  bacterial Prematurity  multiple risk factors Hyperbilirubinaemia Ototoxicity  aminoglycosides, loop diuretics
  6. 6. Hereditary Hearing Impairment (HHI) Congenital HHI Delayed onset HHI Present at birth Develop later in lifeSyndromic: Non Syndromic:both conductive & SNHL most often SNHL
  7. 7. Syndromic HHI Chromosomal disorders  Down Syndrome  Trisomies Non Chromosomal disorders  Noonan’s syndrome  Craniosynostosis  Pendred Syndrome  Osteogenesis Imperfecta  Charcot-Marie Tooth  Waardenburg Syndrome
  8. 8. Non-Syndromic HHI: the genetics Gene mutation Genetic heterogeneity “DeaFNess” gene loci  AR (77%) DFNB loci (1-33)  AD (22%) DFNA loci (1-41)  X-linked (1%) DFN loci (1-8)  Mitochondrial (< 1%) maternal inheritance with susceptibility to aminoglycosides
  9. 9. Undetected Hearing Loss Hearing loss is a Screening hidden disability which has long termIdentification consequences on Follow up development if theIntervention appropriate intervention is not Outcome instituted early
  10. 10. The Importance of Hearing  Hearing is important for  Language development  especially in the first 5 years of life  Speech development  Speech perception  Speech production (including articulation, timing and prosody)
  11. 11. Unilateral or Mild Bilateral HL  Relatively minor disability  Speech recognition & understanding under adverse conditions (e.g. classroom noise) → Attention Behaviour Overall educational achievement  Psychosocial well being
  12. 12. Children with minimal sensorineural hearing loss: Poorer comprehension & communication Impairment in functional status 37% failed at least 1 grade Bess FH, Ear Hear Oct 1998
  13. 13. Moderate-profound HL Impairment of speech/language development Reduction of academic achievement Disturbance in social & emotional development
  14. 14. Problems faced by HI Children Hearing Impaired Children Language Non – Language Concomitant Issues Issues Disabilities
  15. 15. Language Related Issues1. When adults speak with the HI child  They tend to give less verbal praise and become less stimulating2. HI children have reciprocal communication issues  They use attention-seeking statements or methods to enter a conversation  They have difficulty deciding when to enter a conversation
  16. 16. Language Related Issues1. When HI children seek clarification,  They tend to repeat rather than revise3. HI children have limited vocabulary:  Vocabulary of the HI is 0 - 9 words at 18 months  Vocabulary of normally-hearing children = 20 – 50 words
  17. 17. Language Related Issues1. HI children have poor sentence structure Children with language difficulties face difficulty in  Learning new skills & concepts  Making sense of events around  Solving problems  Literacy – reading & writing skills
  18. 18. Non-Language Issues More physically–tired (more effort required) Emotional outcomes- Poor self-esteem, motivation3. Socialization skills  Difficulty in making friends & being accepted socially  Often feel rejected & isolated  Weaker play skills (especially those associated with language and social skills)
  19. 19. Non-Language Issues1. Behaviour problems  Aggression  Impulsivity  Immaturity  Inattention, frustration2. Family dynamics  Challenges in family adjustment (emotional, financial and time factors) will impact on the child and his/her family - child relationship
  20. 20. Assessment of Language Proper diagnostic hearing assessment Proper treatment i.e. aids / CI / AVT / sign Hearing Age important Appropriate non verbal and verbal language assessments:  Receptive language skills  Expressive language skills
  21. 21. Concomitant DisabilitiesSeveral disabilities occur more commonly in HIchildren Intellectual / cognitive disability (8%)  Based on generalized developmental delay with poor adaptive or functional skills Emotional / behavioural problems (4%)  Inappropriate, disruptive, aggressive behaviour
  22. 22. Concomitant DisabilitiesLearning disability (9%)  Diagnosis based on discrepancy between intellectual potential and achievement in reading and writing skills  Average / above average IQ with specific deficits in reading/writing (dyslexia)Neurological problemsVisual impairment
  23. 23. Hearing Impairment & Learning Previous studies showed that HI children have  Linguistic / language problems  Cognitive / thinking / learning problems Hearing impairment alone does NOT equate to low intellectual ability or inability to learn With early intervention, children can develop good language skills
  24. 24. Early intervention is essential  Neural circuits of auditory system rewired by external stimulations very early in life  If such stimulations not received at that time, circuits will not develop fully Pediatric Clinics of North America Feb 1999
  25. 25. ScreeningIdentification Breakdown at any stage Follow up jeopardizes entire effortIntervention Outcome
  26. 26. The UNHS programme was piloted at the Singapore General Hospital in 1998In 2002 the project become theNational UNHS programmeinvolving all public hospitals,polyclinics and some privatehospitals
  27. 27. Echocheck version 2.13.3ILO, Otodynamics(July 2000 - )
  28. 28. Echoport ILO 288, Otodynamics(Nov 1998 - )
  29. 29. Do not only hear mebut also listen to me…

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