Development in the Development in the Hearing Impaired ChildHearing Impaired Child

Dr Varsha Atul ShahDr Varsha Atul Shah

Associate ConsultantAssociate Consultant

Department of Neonatal & Department of Neonatal & Developmental Medicine, SGHDevelopmental Medicine, SGH

Visiting ConsultantVisiting Consultant

K K Hospital,Child Development K K Hospital,Child Development UnitUnit

Colorado Newborn Screening Colorado Newborn Screening ProgramProgram

Per 100 000 birthsPer 100 000 births

Congenital HLCongenital HL 260 (200 bilateral)260 (200 bilateral)

HypothyroidismHypothyroidism 2525

PKUPKU 77

CFCF 5050

Pediatrics Jan 1998Pediatrics Jan 1998

SeveritySeverity dBdB

MildMild 27 - 4027 - 40

ModerateModerate 41 - 5541 - 55

Moderately-severeModerately-severe 56 - 7056 - 70

SevereSevere 71 - 9071 - 90

ProfoundProfound 91 91American National Standards Institute American National Standards Institute 19691969

Classification of Classification of Severity of Hearing Severity of Hearing

ImpairmentImpairment

Hearing Impairment In InfancyHearing Impairment In Infancy

Environmental Environmental CausesCauses (50%)(50%)

Genetic Causes Genetic Causes (HHI)(HHI) (50%)(50%)

Syndromic (30%)

Non Syndromic (70%)

Environmental CausesEnvironmental Causes

Perinatal infectionsPerinatal infections CMV, Rubella, other IUICMV, Rubella, other IUI

MeningitisMeningitis bacterialbacterial

PrematurityPrematurity multiple risk factorsmultiple risk factors

HyperbilirubinaemiaHyperbilirubinaemia OtotoxicityOtotoxicity

aminoglycosides, loop diureticsaminoglycosides, loop diuretics

Hereditary Hearing Hereditary Hearing Impairment (HHI)Impairment (HHI)

CongenitalCongenital HHI HHIPresent at birthPresent at birth

Delayed onsetDelayed onset HHI HHIDevelop later in lifeDevelop later in life

Syndromic:

both conductive & SNHL

Non Syndromic:

most often SNHL

Syndromic HHISyndromic HHI

Chromosomal disordersChromosomal disorders Down SyndromeDown Syndrome TrisomiesTrisomies

Non Chromosomal Non Chromosomal disordersdisorders Noonan’s syndrome Noonan’s syndrome CraniosynostosisCraniosynostosis Pendred SyndromePendred Syndrome Osteogenesis Osteogenesis

ImperfectaImperfecta Charcot-Marie ToothCharcot-Marie Tooth Waardenburg SyndromeWaardenburg Syndrome

Non-Syndromic HHI: the Non-Syndromic HHI: the geneticsgenetics

Gene mutation Gene mutation

Genetic heterogeneityGenetic heterogeneity

““DDeaeaFNFNess” gene lociess” gene loci AR (77%)AR (77%) DFNB loci (1-33)DFNB loci (1-33)

AD (22%)AD (22%) DFNA loci (1-41)DFNA loci (1-41)

X-linked (1%)X-linked (1%) DFN loci (1-8)DFN loci (1-8)

Mitochondrial (< 1%)Mitochondrial (< 1%) maternal maternal inheritance inheritance with with susceptibility to susceptibility to aminoglycosidesaminoglycosides

Undetected Hearing LossUndetected Hearing Loss

Hearing loss is a Hearing loss is a

hidden disability hidden disability

which has long which has long

term consequences term consequences

on development if on development if

the appropriate the appropriate

intervention is not intervention is not

instituted earlyinstituted early

Screening

Identification

Follow up

Intervention

Outcome

The Importance of Hearing The Importance of Hearing

Hearing is important forHearing is important for

1.1. LanguageLanguage development development especially in the first especially in the first

5 years of life5 years of life

2.2. SpeechSpeech development development Speech perceptionSpeech perception Speech production Speech production

(including (including

articulation, timing articulation, timing

and prosody)and prosody)

Unilateral or Mild Bilateral HLUnilateral or Mild Bilateral HL Relatively minor disabilityRelatively minor disability Speech recognition & understanding under Speech recognition & understanding under

adverse conditions (e.g. classroom noise)adverse conditions (e.g. classroom noise)

AttentionAttention

BehaviourBehaviour

Overall educational Overall educational achievementachievement

Psychosocial well beingPsychosocial well being

Children with minimal sensorineuralChildren with minimal sensorineural

hearing loss:hearing loss:

Bess FH, Ear Hear Oct 1998Bess FH, Ear Hear Oct 1998

Poorer comprehension & communicationPoorer comprehension & communication

Impairment in functional statusImpairment in functional status

37% failed at least 1 grade37% failed at least 1 grade

Moderate-profound HLModerate-profound HL

Impairment of speech/language Impairment of speech/language developmentdevelopment

Reduction of academic achievementReduction of academic achievement

Disturbance in social & emotional Disturbance in social & emotional development development

Problems faced by HI Problems faced by HI ChildrenChildren

HearingImpairedChildren

LanguageIssues

Non – LanguageIssues

ConcomitantDisabilities

Language Related Language Related IssuesIssues

1.1. When adults speak with the HI childWhen adults speak with the HI child They tend to give less verbal praise and They tend to give less verbal praise and

become less stimulatingbecome less stimulating

2.2. HI children have reciprocal HI children have reciprocal communication issuescommunication issues They use attention-seeking statements or They use attention-seeking statements or

methods to enter a conversation methods to enter a conversation They have difficulty deciding when to enter a They have difficulty deciding when to enter a

conversationconversation

Language Related Language Related IssuesIssues

3.3. When HI children seek clarification,When HI children seek clarification, They tend to repeat rather than reviseThey tend to repeat rather than revise

4.4. HI children have limited vocabulary:HI children have limited vocabulary: Vocabulary of the HI is 0 - 9Vocabulary of the HI is 0 - 9

words at 18 monthswords at 18 months Vocabulary of normally-hearingVocabulary of normally-hearing

children = 20 – 50 words children = 20 – 50 words

Language Related Language Related IssuesIssues

5.5. HI children have poor sentence structureHI children have poor sentence structure

6.6. Children with language difficulties Children with language difficulties

face difficulty in face difficulty in

Learning new skills & conceptsLearning new skills & concepts

Making sense of events aroundMaking sense of events around

Solving problemsSolving problems

Literacy – reading & writing skillsLiteracy – reading & writing skills

Non-Language Non-Language IssuesIssues

1.1. More More physically–tiredphysically–tired (more effort (more effort required)required)

2.2. Emotional outcomes-Emotional outcomes- Poor self-esteem, Poor self-esteem, motivationmotivation

3.3. Socialization skillsSocialization skills Difficulty in making friends & being accepted Difficulty in making friends & being accepted

sociallysocially Often feel rejected & isolatedOften feel rejected & isolated Weaker play skills (especially those associated Weaker play skills (especially those associated

with language and social skills)with language and social skills)

4.4. Behaviour problemsBehaviour problems AggressionAggression ImpulsivityImpulsivity ImmaturityImmaturity Inattention, frustrationInattention, frustration

5.5. Family dynamicsFamily dynamics Challenges in family adjustment (emotional, Challenges in family adjustment (emotional,

financial and time factors) will impact on the financial and time factors) will impact on the child and his/her family - child relationshipchild and his/her family - child relationship

Non-Language IssuesNon-Language Issues

Assessment of Language Assessment of Language

Proper diagnostic hearing assessmentProper diagnostic hearing assessment Proper treatment i.e. aids / CI / AVT / Proper treatment i.e. aids / CI / AVT /

signsign Hearing Age importantHearing Age important Appropriate non verbal and verbal Appropriate non verbal and verbal

language assessments:language assessments: Receptive language skillsReceptive language skills Expressive language skillsExpressive language skills

Concomitant DisabilitiesConcomitant Disabilities

Several disabilities occur more commonly Several disabilities occur more commonly in HI childrenin HI children

Intellectual / cognitive disability (8%)Intellectual / cognitive disability (8%) Based on generalized developmental Based on generalized developmental

delay with poor adaptive or functional delay with poor adaptive or functional skillsskills

Emotional / behavioural problems (4%)Emotional / behavioural problems (4%) Inappropriate, disruptive, aggressive behaviourInappropriate, disruptive, aggressive behaviour

Concomitant DisabilitiesConcomitant Disabilities

Learning disability (9%)Learning disability (9%) Diagnosis based on Diagnosis based on discrepancydiscrepancy

between intellectual potential and between intellectual potential and achievement in reading and writing skillsachievement in reading and writing skills

Average / above average IQ with specific Average / above average IQ with specific deficits in reading/writing (dyslexia)deficits in reading/writing (dyslexia)

Neurological problemsNeurological problems

Visual impairmentVisual impairment

Hearing Impairment & Hearing Impairment & LearningLearning

Previous studies showed that HI children havePrevious studies showed that HI children have Linguistic / language problemsLinguistic / language problems Cognitive / thinking / learning problemsCognitive / thinking / learning problems

Hearing impairment alone Hearing impairment alone does NOT does NOT equate to low intellectual ability or inability to equate to low intellectual ability or inability to learnlearn

With early intervention, children can With early intervention, children can develop good language skillsdevelop good language skills

Early intervention is Early intervention is essentialessential

Neural circuits of auditory system Neural circuits of auditory system rewired by external stimulations rewired by external stimulations very early in lifevery early in life

Pediatric Clinics of North America Feb Pediatric Clinics of North America Feb 19991999

If such stimulations not received at that If such stimulations not received at that time, circuits will not develop fullytime, circuits will not develop fully

Screening

Identification

Follow up

Intervention

Breakdown at any stage

jeopardizes entire effort

Breakdown at any stage

jeopardizes entire effort

Outcome

The UNHS The UNHS programme was programme was piloted at the piloted at the Singapore General Singapore General Hospital in 1998Hospital in 1998

In 2002 the project become In 2002 the project become the National UNHS the National UNHS programme involving all programme involving all public hospitals, polyclinics public hospitals, polyclinics and some private hospitalsand some private hospitals

Echocheck version 2.13.3 ILO, Otodynamics

(July 2000 - )

Echoport ILO 288, Otodynamics

(Nov 1998 - )

Do not only hear me Do not only hear me but also listen to me…but also listen to me…