1. Considerations in Pediatric Audiological Assessment of Children With Multiple Disabilities: An Overview
Faye P. McCollister, EdD
University of Alabama, Emeritus
Diane L. Sabo, PhD
Children’s Hospital of Pittsburgh
University of Pittsburgh
Consulting Audiologists
National Center for Hearing Assessment and Management

2. Factors to Consider Subject Variables Environmental Variables
Test Variables

3. 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

4. Subject Variables Age Corrected age Chronological age Auditory age
Gestational period
Chronological age
Auditory age
Type of response
Level of response
Developmental age
Cognitive level
Language level

5. 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

6. 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

7. Additional Disabilities
Other problems
Failure to thrive
Cystic fibrosis
Chromosomal abnormalities
Fragile x syndrome
Drug exposed baby
Fetal alcohol syndrome

8. Subject Variables Support equipment Access to booth Need more space
Ventilator
Apnea monitor
Head support
Wheel chair
Communication board
Head pointer
Restraints
Access to booth
Need more space
Creates noise
Prevents response observation

9. Subject Variables Family
Priority of hearing in multidisciplinary diagnostic process
Resources, social interaction skills
Health literacy
Native language, cultural diversity
Preferred method for communication

10. Cultural Diversity
Issues
Prevalence
Treatment
funding and legality

11. 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.

12. 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.

13. 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.

14. 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

15. Subject Variables
Medications
Seizure
Cardiac
Psychotropic
ADHD

16. Subject Variables Behavior Calm, non-vocal Agitated, vocal, crying
Age appropriate attention span
Clinging, will not separate

17. Environmental Variables
Size of test booth
Location of speakers
Location of observation window, lighted
Commercially available reinforcers
Handheld reinforcers

18. 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

19. 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

20. 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

21. 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)

22. 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

23. Observations Characteristics of auditory responses
Developmental characteristics
Parent-child interaction
Anatomical variations
Pigmentation variations
Facial or limb abnormalities
Hirsutism (Hairiness)

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

25. 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

26. 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

27. Methods VRA TROCA/VROCA Play audiometry Conventional Audiometry
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

28. 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

29. 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)

30. Management of Hearing Loss
Amplification
FMs or other ALDs EI

31. 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

32. Down Syndrome Incurving fifth finger Simian Crease Flat faces
Frontal bossing
Frequent hearing problems, conductive and/or sensory neural

33. 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

34. Psychomotor Damage
Psychomotor Involvement
Spasticity
Hypotonicity

35. Cleft Lip and Palate
Newborn hearing screening often compromised by MEE
ABR often needed

36. Goldenhar Syndrome

37. 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

38. Mucopolysacharidosis
Examples: Hunter and Hurler Syndrome
Hunter: x-linked recessive, typically less severe
Hurler: autonomic recessive

39. 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

40. 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.