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

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


1
Considerations in Pediatric Audiological
Assessment of Children With Multiple
DisabilitiesAn Overview
  • Faye P. McCollister, EdD
  • University of Alabama, Emeritus
  • Diane L. Sabo, PhD
  • Childrens 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
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

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 reinforcerneeds 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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