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Considerations in Pediatric Audiological Assessment of Children With Multiple Disabilities: An Overview Faye P. McCollister, EdD University of Alabama,

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Presentation on theme: "Considerations in Pediatric Audiological Assessment of Children With Multiple Disabilities: An Overview Faye P. McCollister, EdD University of Alabama,"— Presentation transcript:

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.

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