Considerations in Pediatric Audiological Assessment of Children With Multiple Disabilities: An Overview

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 • 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. 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

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 • 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.