LANGUAGE
Cognitive
Development
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 RETARDATION
DEFINITION
A significantly subaverage general
intellectual functioning which manifests
itself during the developmental period and
is characterized by inadequacy in adaptive
behavior.
INCIDENCE
About 3% of the population
ETIOLOGY OF MENTAL RETARDATION
A. Congenital syndromes, genetic
Chromosomal
Single gene defect
Major malformations
Presumed genetic
B. Congenital syndromes, nongenetic
Intrauterine infection
Maternal systemic disease
Maternal drug ingestion
Maternal gestational disorders
DOWN SYNDROME
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


Trisomy 21
Incidence: 1:800-1,000 babies
Clinical features
Medical problems






40-50% - congenital heart disease
50% - visual/hearing impairment
10% - intestinal malformations
15-20% - Alzheimer’s disease/dementia
Increased risk of thyroid problems/leukemia
Average life span: 55 years
ETIOLOGY …
C. Perinatal and Postnatal Factors
Prematurity and its complications
Birth asphyxia
Head trauma
CNS infection
Toxins
Hypoxic events
Chronic severe systemic disease
Nutitional deficiencies
Socioeconomic deprivation
D. Unknown
Distribution of Classification of
Mental Retardation
2.50%
2.00%
1.50%
1.00%
0.50%
0.00%
MILD MOD-SEV PROFOUND
LEVEL
OF
MR
Borderline
IQ
SCORE
EDUC.
EQUIV
ADAPTIVE
BEHAVIOR ADL
LEVEL OF
SUPPORT
70-79
Educable
Independent
Employable
Intermittent
Mild
50-55 to
70
Educable
Employable in
simple jobs
Intermittent –
prn basis
Moderate
35-40 to
50-55
Trainable
Trainable
Employed in
sheltered env.
Limited;
more than
prn
Severe
20-25 to
35-40
Trainable
Dependent
Trainable in some
basic ADL
Extensive;
support at
least 1/day
Profound
Below 20 Custodial
(life sup)
Dependent in all
ADLS
Pervasive
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
DIAGNOSIS
Neurodevelopmental Assessment:
1. Comprehensive history.
2. Complete physical and neurological
examination.
3. Appropriate laboratory studies.
4. Developmental screenings.
5. Judicious referrals to supporting
professionals.
MULTIDISCIPLINARY APPROACH
MANAGEMENT
Role of the physician is
limited; management is
generally psychoeducational.
• most deviant degree of communicative disorder
• characterized by a triad of impairments
Impaired social relatedness
Impaired communication and play
Stereotypic/ritualistic activities
PREVALENCE:
5-6 per 1000 births
Congenital SNHL: 1/1000 births
At age 5 years, 10-15% of children
fail hearing screening
RISK FACTORS
1. A family history of hereditary
childhood sensorineural hearing loss
2. Congenital infections known to be
associated with hearing loss
3. Cranifacial anomalies
4. Birthweight less than 1500 gms
5. Hyperbilirubinemia at a serum level
requiring exchange transfusion.
RISK FACTORS …
6. Ototoxic medications
7. Bacterial meningitis
8. Apgar score of 0-4 at 1 minute or 0-6 at
5 minutes
9. Mechanical ventilationfor 5 days or
longer
10. Stigmata of a syndrome known to
include hearing loss
DEGREES OF HEARING IMPAIRMENT
Level of HL Description
16-25 dB
25-30 dB
Slight
hearing
loss
Etiology
Serous otitis
perforation,
SNHL,
tympanosclerosis
Mild
Serous otitis
hearing loss
perforation,
tympanosclerosis,
SNHL
DEGREES OF HEARING IMPAIRMENT
Level of HL
Description
Etiology
30-50 dB
Moderate
hearing loss
Chronic otitis,
middle ear
anomaly, SNHL
50-70 dB
Severe hearing
loss
SNHL or mixed
loss from SN or
middle ear dis
More than 70 dB
Profound
hearing loss
Sensorineural or
mixed loss
AVERAGE AGE OF IDENTIFICATION
MILD SNHL – 3 to 4 years old
MODERATE TO PROFOUND SNHL
– 23 months
UNILATERAL OR HIGH
FREQUENCY LOSSES – 5 to 6
years old
Comprehension deficit leads to delay in the
acquisition of speech and language forms
Most frequent complaints:
1. Lack of response to speech/noise
2. Poor speech development
Less frequent complaints:
1. Behavior problems
2. Balance problems/ear fingering
HEARING EVALUATION
METHODS:
• 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” CHILD
Impaired 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 DISORDER
PATHOGENESIS/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
LANGUAGE
ASSESSMENT
Difficult
Reasons:
1. Most parents do not really focus on
early language milestones.
2. Difficult to assess language directly in
the well baby setting.
• PARENTAL CONCERNS
Sensitivity of 72%
Specificity of 83 %
Middle-class community
• SCREENING TESTS
1. Early language Milestone Scale (ELMS)
2.
Denver II
3. Clinical Linguistic and Auditory Milestone Scale
(CLAMS)
4. Gessell Schedules of Infant Development
• Audiological
Evaluation
The 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