Download CONGENITAL, PERINATAL, AND NEONATAL INFECTIONS

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
Factors to Consider
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Subject Variables
Environmental Variables
Test Variables
Multiple Disabilities
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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
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Age
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Corrected age
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Chronological age
Auditory age
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Gestational period
Type of response
Level of response
Developmental age
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Cognitive level
Language level
Subject Variables
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Additional Disabilities
Cognitive level
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Determines appropriate behavioral
technique
Determines level of response, type of
response
Determines appropriate reinforcer
Motor disorders/cerebral palsy
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Head turn responses compromised
Play activity may be limited
Fatigue
Subject Variables
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Additional Disabilities (cont.)
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Vision
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Can not see visual reinforcers
Can not process visual instructions
Needs glasses for assessment, if prescribed
Seizure disorder
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Flicker stimulation with lighted reinforcer
Absence, petit mal, and grand mal seizures
Additional Disabilities
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Other problems
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Failure to thrive
Cystic fibrosis
Chromosomal abnormalities
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Fragile x syndrome
Drug exposed baby
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Fetal alcohol syndrome
Subject Variables
Support equipment
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Ventilator
Apnea monitor
Head support
Wheel chair
Communication
board
Head pointer
Restraints
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Access to booth
Need more space
Creates noise
Prevents response
observation
Subject Variables
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Family
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Priority of hearing in multidisciplinary
diagnostic process
Resources, social interaction skills
Health literacy
Native language, cultural diversity
Preferred method for communication
Cultural Diversity
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Issues
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Prevalence
Treatment
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funding and legality
Cultural Diversity
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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
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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
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Alberg and Kerr (2004) developed a list
of considerations for service providers
working with multicultural populations.
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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.
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There may be different dialects among people
from the same country.
Cultural Diversity
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Racial, cultural and socioeconomic differences
may exist among individuals from the same
country.
Interpreters may have difficulty explaining
medical and technical information
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May be difficult for the family to understand.
Families sometimes enter the U.S. illegally.
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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
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Medications
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Seizure
Cardiac
Psychotropic
ADHD
Subject Variables
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Behavior
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Calm, non-vocal
Agitated, vocal, crying
Age appropriate attention span
Clinging, will not separate
Environmental Variables
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Size of test booth
Location of speakers
Location of observation window,
lighted
Commercially available reinforcers
Handheld reinforcers
Environmental Variables
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Movement Restricting Furniture
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High chair
Table chair
Infant carrier
Papoose board
Blanket for swaddling
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Use blankets/pillows for support
Use belt for stability
Environmental Variables
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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
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Commercially available reinforcement units,
Variety of puppets, lighted obs window
Test Protocol Considerations
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The Audiologist
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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
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Limited amount of time
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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
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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
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Characteristics of auditory responses
Developmental characteristics
Parent-child interaction
Anatomical variations
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Pigmentation variations
Facial or limb abnormalities
Hirsutism (Hairiness)
Test Battery Approach
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Air and bone conduction
OAEs
ABR/ASSR
Acoustic Immittance
Air conduction
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Allow longer response times
Speech stimuli (simple commands)
and other broad band stimuli
Insert earphones, preferred
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placement
Sound field
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To assess type of response to sounds
Bone Conduction
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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
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VRA
TROCA/VROCA
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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
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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
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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
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Amplification
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FMs or other ALDs
EI
Case Reports
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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
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Incurving fifth
finger
Simian Crease
Flat faces
Frontal bossing
Frequent hearing
problems,
conductive and/or
sensory neural
Down Syndrome
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Behavioral testing-best after 10 months
of age
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Success of behavioral testing is often
dependent on cognitive abilities as well as
the presence of other disabilities
Psychomotor Damage
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Psychomotor
Involvement
Spasticity
 Hypotonicity
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Cleft Lip and Palate
Newborn hearing screening often
compromised by MEE
ABR often needed
Goldenhar Syndrome
Goldenhar Syndrome
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Oculoauriculovertebral Dysplasia
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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
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Examples: Hunter and Hurler Syndrome
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Hunter: x-linked recessive, typically less
severe
Hurler: autonomic recessive
Mucopolysaccharidoses
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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
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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
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EI
Medical personnel e.g. neurology,
ophthalmology etc.