Transcript The Effect of Universal Newborn Hearing Screening on Identifying

The Effect of UNHS on Early Cochlear
Implantation
Antonia Brancia Maxon, Ph.D.
Diane Brackett, Ph.D.
Jennifer Cox, M.A.
Alicia Ayles, M.A.
New England Center for Hearing
Rehabilitation
NECHEAR
Why is Early Identification of Hearing
Loss so Important?
• Congenital hearing loss interferes with the
most basic human need to communicate with
others
• Hearing loss is the most frequent birth defect
• Undetected hearing loss has serious negative
consequences
• There are dramatic benefits associated with
early identification of hearing loss
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UNHS lowers age of intervention
• Infants with hearing loss should be fit
with hearing aids by four months old
• Infants with hearing loss should be
enrolled in early intervention by four
months old
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UNHS lowers age of intervention
• When a six month hearing aid trial is
used
– Benefit from traditional amplification can
be determined by 10 months old
• Infants who do no benefit should not
have to wait for the implant.
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UNHS affects the age of cochlear
implant candidacy identification
• Until recently the recommended age of
implantation was 18 months old.
• Now 12 months is recommended lowest
age.
• Surgeons are implanting even younger
infants.
NECHEAR
Early Identification Ensures Some
Critical Criteria Are Met
• Objective measures are available to
make a diagnosis within the first month
of life.
– will know degree of hearing loss
– will know the configuration of hearing loss
– will know if there are middle ear problems
• Can begin process of candidacy
evaluation
NECHEAR
Early Identification Ensures Some
Critical Criteria Are Met
• Parents have accepted the hearing loss
and are ready to begin appropriate
intervention.
• Parents are aware of communication
modality options.
• Parents know the role of and need for
sensory devices.
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Candidacy
• Bilateral severe to profound
sensorineural hearing loss
• Infant/toddler cannot benefit from
traditional amplification
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Diagnosis to Decision - Parent
• Need to understand:
–
–
–
–
no device is a cure
therapy is long-term
language learning is an ongoing process
access to acoustic speech cues
• how access relates to child’s progress
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Diagnosis to Decision - Parent
• Need to:
– understand the difference between
reacting to cues received through hearing
and other senses
– identify auditory responses
– learn how to use the home, family,
environment in a positive way for auditory
and language learning
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Diagnosis to Decision - Parent
• Need to be comfortable with equipment
– know how to use it properly
– know how to modify clothing to
accommodate it
– accept that people will look, question,
comment on it
– able to troubleshoot equipment and child’s
auditory behavior
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Diagnosis to Decision - Parent
• Need to understand the audiogram
– know the degree and configuration
– know the basics of acoustic cues of speech
– know how speech perception is affected by
their child’s hearing loss
– know what their child actually receives
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Diagnosis to Decision - Child
• Child needs to be fit with best
amplification
– must get the most auditory stimulation
possible
• body-worn FM system
• snap-on transducers
• power transducers
• earmolds - good fit at all times
NECHEAR
Diagnosis to Decision - Child
• Child needs to be enrolled in an EI
program that stress auditory function
– learning
– learning
– learning
– learning
to use hearing
to respond to sound
to respond to speech
the need for communication
NECHEAR
Assessing Auditory Function
• From initial enrollment in EI always
assessing auditory function
• Should not make decision about hearing
aid or cochlear implant based on pure
tone audiogram
• Need to determine if child is able to
make maximal use of residual hearing
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Assessing Auditory Function
• Child needs to receive speech at an
appropriate level with amplification
– detection is not enough
– have to look across frequencies for access
to all speech sounds
– speech needs to be received at a useable
level
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Assessing Auditory Function
• Child must have good access to speech
of others and own speech
– at different distances from sound source
• Child should alert to a variety of speech
and non-speech sounds
– at different distances from sound source
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Assessing Auditory Function
• Child’s output
– vocalization
• precursor to speech production
– intentional vocalization
• precursor to language
– vocalization + gesture
• intention to communicate
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Indications the Child is a Candidate
• Child shows limited or no detection of
amplified speech
• Comparisons of audiological data and
functional use of hearing
• Knowledge of the child’s auditory
limitations
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Factors that Facilitate CI Success
• Parents know about hearing loss and
accept long-term problems
• Parents understand the implant is not a
cure
• Parents are committed to implant use
• Parents are committed to therapy
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Factors that Facilitate CI Success
• Family has access to therapy and
mapping facilities
• Family is motivated
• One parent at home - minimal day care
• The household is organized
• Child is vocalizing
• Child has normal cognitive level
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Absence of one or more of those
facilitating factors does not rule out
the child’s candidacy.
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Why Implant Early
• Critical period of communication
development
• Children implanted below 10 years old
– children implanted before age 3 showed a
greater period of auditory plasticity
– open-set speech recognition could not be
achieved after 6 years old
(Manrique, et al, 1999)
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Why implant early?
• Early is better for children with hearing
aids (Apuzzo & Yoshinaga-Itano, 1995)
• Early implantation
– allows for extended listening time before
social and school demands start
– decreases chance of a large gap between
chronological and communication age
– promotes acquisition of speaking and
language
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Why implant early?
• Children implanted early (2-3 years)
make greater growth than later
implanted (3-5 years) children
– expressive vocabulary
– receptive vocabulary
– dramatic improvements first year post
implant
(Brackett and Zara, 1998)
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Why implant early?
• Surgical risks are minimal
• Complications are infrequent
• Objective testing can be used to predict
appropriate programming
• Can use audiological behavioral
measures for programming
(Cohen, Drous, Shapiro & Waltzman, 2003)
NECHEAR
Information Needed by Parents
• Parents wanted most information prior
to surgery, but wanted continued
informational support post-implant
• Parents felt emotional support was most
lacking
• Majority of parents felt there needed to
be a professional liason between CI
center and educational program
(Most and Zaidman-Zait, 2003)
NECHEAR
Cochlear Implant Surgeon Selection
• Competent and experienced with
infants
• Familiar with surgical placement of
receiver/stimulator package that
reduces potential damage from head
impact
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Cochlear Implant Surgeon Selection
• Familiar with techniques that reduce
intracranial complications
• Willing to make accommodations for
child and family regarding
hospitalization, pre- and post-surgery
conditions
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Mapping Audiologist Selection
• Competent and experienced with
infants
• Familiar with VRA
• Availability of multiple testers
• Availability of appropriate equipment
and reinforcement
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Mapping Audiologist Selection
• Knows about speech acoustics
• Uses functional measures to fine tune
the map
• Accepts input from parents and
rehabilitation professional
• Maps the implant “on demand”
• Knows about aural rehabilitation
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Surgery to Stimulation
• Child
– becomes familiar with and gets used to
equipment
– uses hearing aid on non-implanted ear
• Aural rehabilitation
– communication focus continues
– conditioning sessions to facilitate mapping
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Initial Stimulation
• Short term goal is good access to
speech across frequencies
• Weekly sessions until have a good map
• Use input from parent(s) and
rehabilitation professional
• Use functional responses to modify
maps
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Rehabilitation Professional Selection
• Knowledgeable about:
– infant behavior
– infant development
– infant communication
– auditory development
– activities that facilitate auditory-vocal
behavior
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Rehabilitation Professional Selection
• Knowledgeable about:
– infant adaptation to equipment
– home therapy suggestions
– troubleshooting the equipment
– troubleshooting the child’s behaviors
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Rehabilitation Programming
• Keeping external components on head
• Keeping body processor comfortable
• Continued conditioning for mapping
when necessary
• Mapping as required
– parent request
– rehabilitation professional request
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Case Study - Stevie
• UNHS - failed bilaterally (before mandate in
CT)
• Bilateral profound SNHL diagnosed - 5 mos
• Hearing aids fit - 5 mos
• Enrolled EI (2x/week) - 6 mos
• FM fit - 10 months
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Case Study - Stevie
• Aided response levels - 50-60 dB HL
• Aided SAT - 45 dB HL
• CI evaluation - 10 mos
• CI surgery - 13 mos
• Initial stimulation - 14 months
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Case Study - Stevie
(evaluation at 32 months)
• Speech perception: 100% spondees, 83%
monosyllables
• Speech production: 75% consonants, 97%
vowels
• Vocabulary: Receptive - not basal, Expressive
- <1.9 years
• Utterances: 4-5 words, not many verbs, rising
inflection for questions
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Case Study - Stevie
(evaluation at 46 months)
• Speech perception: 100% consonants, 100%
vowels
• Speech production: 90% consonants, 100%
vowels
• Vocabulary: Receptive - 10 month delay,
Expressive - 8 month delay
• Sentences: 5-7 words, nouns and verbs some
adjectives, possessive, plural markers
emerging, some pronouns.
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Case Study - Anna
• UNHS - failed bilaterally (post mandate)
• Bilateral profound SNHL diagnosed - 3.5
weeks
• Hearing aids fit - 4 weeks
• Enrolled EI (2x/week) - 4 weeks
• Hearing aids and FM fit - 4 weeks
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Case Study - Anna
• Aided response levels - 40-50 dB HL
• Aided SAT - 35 dB HL
• CI evaluation - 10 mos
• CI surgery - 11 mos
• Initial stimulation - 12 months
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Case Study - Anna
(evaluation 28 months)
• Speech perception: excellent
• Speech production: errors - m/n, g/d, omits
ch, t, m, z
• Vocabulary: Receptive - 21 months,
Expressive - 21 months
• PLS: Auditory Comp - 31 months, Expressive
Comp - 28 months, Total Language - 30
months
NECHEAR
Universal Newborn Hearing
Screening
• Lowers the age of hearing loss
diagnosis
• Lowers the age amplification is provided
• Lowers the age of enrollment in early
intervention
• POTENTIALLY lowers the age of
cochlear implantation
NECHEAR
Benefits of Early Cochlear
Implantation
• Minimizes the effects of auditory
deprivation
• Electrical stimulation provides necessary
information to auditory system at
critical period for speech and language
acquisition.
• Provides child with a good chance to
develop normal speech and language
NECHEAR