LANGUAGE CognitiveDevelopment

Failure to speak single words by 18 months and phrases by 30 months

(Speech and Language Impairment) 10-15% of toddlers 3% are at risk of persistent speech and language problems and learning disability 4 5 % beyond 3 years

Mental retardation Hearing impairment Autism Emotional and Behavioral Disorders Environmental deprivation Developmental language disorder

Most common cause of language delay (> 50 % of cases)

Speech delay is universal among retardates

MENTAL RETARDATIONDEFINITIONA significantly subaverage general intellectual functioning which manifests itself during the developmental period and is characterized by inadequacy in adaptive behavior.INCIDENCEAbout 3% of the population

ETIOLOGY OF MENTAL RETARDATIONCongenital syndromes, geneticChromosomal Single gene defect Major malformations Presumed genetic

B. Congenital syndromes, nongeneticIntrauterine infection Maternal systemic disease Maternal drug ingestion Maternal gestational disorders

DOWN SYNDROMETrisomy 21Incidence: 1:800-1,000 babiesClinical featuresMedical problems40-50% - congenital heart disease50% - visual/hearing impairment10% - intestinal malformations15-20% - Alzheimers disease/dementiaIncreased risk of thyroid problems/leukemiaAverage life span: 55 years

ETIOLOGY C. Perinatal and Postnatal FactorsPrematurity and its complications Birth asphyxiaHead traumaCNS infectionToxins Hypoxic eventsChronic severe systemic disease Nutitional deficiencies Socioeconomic deprivationD. Unknown

MOD-SEVDistribution of Classification of Mental Retardation

LEVEL OFMRIQ SCOREEDUC.EQUIVADAPTIVE BEHAVIOR -ADLLEVEL OF SUPPORTBorderline70-79EducableIndependent EmployableIntermittentMild50-55 to70EducableEmployable in simple jobsIntermittent prn basisModerate35-40 to50-55TrainableTrainableEmployed in sheltered env.Limited; more than prnSevere20-25 to35-40TrainableDependentTrainable in some basic ADLExtensive; support at least 1/day ProfoundBelow 20Custodial(life sup)Dependent in all ADLSPervasive

Mental Retardation Language problem: immaturity of overall language skills. Language as well as the other developmental streams, particularly the visual-motor stream and adaptive skills are delayed.

Global developmental delay

DIAGNOSISNeurodevelopmental Assessment:

Comprehensive history.Complete physical and neurological examination.Appropriate laboratory studies.Developmental screenings.Judicious referrals to supporting professionals.MULTIDISCIPLINARY APPROACH

MANAGEMENTRole of the physician is limited; management is generally psychoeducational.

most deviant degree of communicative disorder characterized by a triad of impairments

Impaired social relatednessImpaired communication and playStereotypic/ritualistic activities

PREVALENCE

Not a rare disorder 4-7 fold increase in the incidence for the last 7 years 1 in 500 Male more than female ( 4-5: 1 )

ETIOLOGY

AUTISM IS A NEURODEVELOPMENTAL DISORDER ( BRAIN GROWTH DISORDER )BRAIN ABNORMALITIES* structural* biochemicalGENETICS

Presenting Signs of Autism

Speech / language delayLack of interest in peer or adult interactionPoor or limited eye contactHyperactivity or hypoactivitySevere feeding and sleeping difficultiesFascination with parts of toys (eg. spinning wheels)Stereotypies (eg. hand flapping)Attachment to unusual objects

DIAGNOSIS

No medical testsUse of diagnostic criteria by skilled professionalsDetermination of communication, behavioral and developmental levelsTeam evaluation and discussion

Management

The most important interventionin autism is EARLY and INTENSIVE REMEDIAL EDUCATION that addresses bothbehavioral and communication disorders.

Autism: Prognosis

Short-termEarly predictors of better outcome:- communicative language andplay before 5 years- higher IQLong-termVocational training for higher functioning autistics.

PREVALENCE:5-6 per 1000 birthsCongenital SNHL: 1/1000 birthsAt age 5 years, 10-15% of children fail hearing screening

RISK FACTORS A family history of hereditary childhood sensorineural hearing loss Congenital infections known to be associated with hearing loss Cranifacial anomalies Birthweight less than 1500 gms Hyperbilirubinemia at a serum level requiring exchange transfusion.

RISK FACTORS 6. Ototoxic medicationsBacterial meningitis Apgar score of 0-4 at 1 minute or 0-6 at 5 minutes Mechanical ventilationfor 5 days or longer Stigmata of a syndrome known to include hearing loss

DEGREES OF HEARING IMPAIRMENTLevel of HL Description Etiology

16-25 dB Slight hearing lossSerous otitis perforation, SNHL, tympanosclerosis25-30 dBMild hearing lossSerous otitis perforation, tympanosclerosis, SNHL

DEGREES OF HEARING IMPAIRMENT Level of HL Description Etiology

30-50 dBModerate hearing lossChronic otitis, middle ear anomaly, SNHL50-70 dBSevere hearing lossSNHL or mixed loss from SN or middle ear disMore than 70 dBProfound hearing lossSensorineural or mixed loss

AVERAGE AGE OF IDENTIFICATIONMILD SNHL 3 to 4 years oldMODERATE TO PROFOUND SNHL 23 monthsUNILATERAL OR HIGH FREQUENCY LOSSES 5 to 6 years old

Most frequent complaints: Lack of response to speech/noise Poor speech development

Less frequent complaints: Behavior problems Balance problems/ear fingeringComprehension deficit leads to delay in the acquisition of speech and language forms

HEARING EVALUATIONMETHODS: Auditory brainstem evoked response(ABR, BAER, BERA) Behavioral play audiometry Otoacoustic emission (OAE) Tympanometry

MANAGEMENT MEDICAL ASSISTIVE DEVICES- hearing aids- cochlear implants EDUCATION

HYPERACTIVE; DISTURBED CHILDImpaired comprehension and production of linguistic forms in relationship to social communicative abilities

Delay in speech as a result of lack of stimulation and attention

Inadequate acquisition of language in the absence of a hearing loss, documented neurologic lesion, mental retardation, or primary emotional disorder.

Prevalence ( DSM-IV ) : 3-5 % of children

DEVELOPMENTAL LANGUAGE DISORDERPATHOGENESIS/ETIOLOGY:

unrelated to perinatal risk factors, early language deprivation, bilingualism

genetic contribution is the only factor that has been implicated to any substantial degree

62% of DLD children studied had an affected parent Tallal, et.al

LANGUAGEASSESSMENT DifficultReasons:

Most parents do not really focus on early language milestones.

Difficult to assess language directly in the well baby setting.

Sensitivity of 72%Specificity of 83 %Middle-class communitySCREENING TESTS PARENTAL CONCERNS Early language Milestone Scale (ELMS) Denver II Clinical Linguistic and Auditory Milestone Scale (CLAMS) Gessell Schedules of Infant Development

Audiological EvaluationThe first step is to rule out a hearing deficit. It is not enough to rely upon parents report or screening in pediatric office, because unilateral or mild hearing deficit, which can cause speech impediment is likely to be missed in the office.

Diagnostics

Neuropsychological Assessment

Speech/Language Evaluation

Individualized Multidisciplinary