Considerations in Pediatric Audiological Assessment of

Children With Multiple Disabilities:An Overview

Faye P. McCollister, EdDUniversity of Alabama, Emeritus

Diane L. Sabo, PhDChildren’s Hospital of Pittsburgh

University of Pittsburgh

Consulting AudiologistsNational Center for Hearing Assessment and

Management

Factors to Consider

Subject Variables Environmental Variables Test Variables

Multiple Disabilities

Approximately 40 % of Children with Hearing Loss Will Have Multiple Disabilities(CADS, Gallaudet)

Will Require Interdisciplinary Team Management

Will Require Modifications of Diagnostic Protocols

Subject Variables

Age Corrected age

Gestational period Chronological age Auditory age

Type of response Level of response

Developmental age Cognitive level Language level

Subject Variables Additional Disabilities Cognitive level

Determines appropriate behavioral technique

Determines level of response, type of response

Determines appropriate reinforcer Motor disorders/cerebral palsy

Head turn responses compromised Play activity may be limited Fatigue

Subject Variables Additional Disabilities (cont.)

Vision Can not see visual reinforcers Can not process visual instructions Needs glasses for assessment, if prescribed

Seizure disorder Flicker stimulation with lighted reinforcer Absence, petit mal, and grand mal seizures

Additional Disabilities

Other problems Failure to thrive Cystic fibrosis Chromosomal abnormalities

Fragile x syndrome Drug exposed baby

Fetal alcohol syndrome

Subject Variables

Support equipment Ventilator Apnea monitor Head support Wheel chair Communication

board Head pointer Restraints

Access to booth Need more space Creates noise Prevents

response observation

Subject Variables

Family Priority of hearing in multidisciplinary

diagnostic process Resources, social interaction skills Health literacy Native language, cultural diversity Preferred method for communication

Cultural Diversity

Issues Prevalence Treatment

funding and legality

Cultural Diversity A growing number or

children with hearing loss in the United States are from families that are non-native English speaking

The 2000 U.S. Census shows that nearly one out of five Americans speak a language other than English at home.

Cultural Diversity Informational materials should

be provided in native languages for parents and at understandable reading levels.

Communication options chosen by families for their child should be respected and supported.

Cultural Diversity Alberg and Kerr (2004) developed a

list of considerations for service providers working with multicultural populations. Families are more comfortable with service

providers who speak their language and understand their culture.

Printed material should be available in the language of the client base.

There may be different dialects among people from the same country.

Cultural Diversity Racial, cultural and socioeconomic

differences may exist among individuals from the same country.

Interpreters may have difficulty explaining medical and technical information

May be difficult for the family to understand. Families sometimes enter the U.S. illegally.

will not qualify for public assistance medical and technical services (e.g., hearing aids)

finding financial assistance for these families is challenging, at best

Subject Variables Medications

Seizure Cardiac Psychotropic ADHD

Subject Variables Behavior

Calm, non-vocal Agitated, vocal, crying Age appropriate attention span Clinging, will not separate

Environmental Variables Size of test booth Location of speakers Location of observation

window, lighted Commercially available

reinforcers Handheld reinforcers

Environmental Variables Movement Restricting Furniture

High chair Table chair Infant carrier Papoose board Blanket for swaddling

Use blankets/pillows for support Use belt for stability

Environmental Variables Control room/test room

communication Accessible toys for distraction to

maintain controlled boredom Ear protection for test assistants Variety of reinforcers to maintain high

level of responding Commercially available reinforcement

units, Variety of puppets, lighted obs window

Test Protocol Considerations The Audiologist

Should be experienced in evaluating young children

Should adhere to published guidelines Proper facilities Knowledgeable about etiology of

hearing loss and comprehensive case management

Test Protocol Considerations Limited amount of time

Condition with speech, child more likely to respond

Use stair case approach, decrease intensity across frequencies selected rather than up and down at single frequency

Use limited number of frequencies (500, 4000, 1000, fill in if possible)

Test Protocol Considerations

Need Audiological Test Battery

Issue is not always getting equipment on and keeping it on but also the behavioral responses may not be observable or may have interference Behavioral with cognitive age

appropriate technique Physiologic tests

Observations Characteristics of auditory

responses Developmental characteristics Parent-child interaction Anatomical variations

Pigmentation variations Facial or limb abnormalities Hirsutism (Hairiness)

Test Battery Approach Air and bone conduction OAEs ABR/ASSR Acoustic Immittance

Air conduction Allow longer response times Speech stimuli (simple

commands) and other broad band stimuli

Insert earphones, preferred placement

Sound field To assess type of response to sounds

Bone Conduction Allow longer response times Issues of keeping vibrator in

place especially with cranial malformations; need to ensure adequate pressure

Introduction of masking simultaneously with stimuli

Methods VRA TROCA/VROCA

Tangible reinforcement often is useful for children with developmental disabilities

Selection of appropriate reinforcer—needs to be meaningful to the patient

Play audiometry Conventional Audiometry

ABR/ASSR Air and bone conduction,

frequency specific stimuli Issues of noise from child i.e.

myogenic noise often high Issues of noise from supportive

equipment

Acoustic Immittance Tympanometry--high frequency

probe tones as needed Acoustic reflex testing--often

compromised by noise Common problems: excessive

cerumen, malformed ear canals, involuntary movements (e.g. teeth grinding)

Management of Hearing Loss Amplification

FMs or other ALDs EI

Case Reports Normal pregnancy, delayed

developmental milestones, short attention span

Hypotonicity Cardiac problem Vision problem Diagnosed with Down syndrome Suspected hearing loss Frequent otitis media, managed

by pediatrician

Down Syndrome Incurving fifth

finger Simian Crease Flat faces Frontal bossing Frequent hearing

problems, conductive and/or sensory neural

Down Syndrome Behavioral testing-best after 10

months of age Success of behavioral testing is often

dependent on cognitive abilities as well as the presence of other disabilities

Psychomotor Damage

Psychomotor Involvement Spasticity Hypotonici

ty

Cleft Lip and Palate

Newborn hearing screening often compromised by MEE

ABR often needed

Goldenhar Syndrome

Goldenhar Syndrome Oculoauriculovertebral Dysplasia

Unilateral malformation of craniofacial structures (eye, oral and musculoskeletal anomalies)

Hearing loss can be sensorineural and/or conductive in one or both ears

Sensorineural component may not identified because of the assumption of conductive due to malformation

Mucopolysacharidosis

Examples: Hunter and Hurler Syndrome Hunter: x-linked recessive, typically less

severe Hurler: autonomic recessive

Mucopolysaccharidoses Heterogeneous group Excessive mucopoly saccharides

storage Variability in expression May have mental retardation Conductive, sensorineural, or mixed

HL; maybe progressive Frequent otitis media Severe forms may result in death in

second decade of life

Conclusion The key to good audiologic

assessment of children with multiple disabilities is EARLY diagnosis and frequent follow up.

Progressive hearing loss is often associated with multiple disabilities (in association with syndromes)

Case coordination is essential for optimizing diagnosis and treatment EI Medical personnel e.g. neurology,

ophthalmology etc.