Download Infant Hearing Testing - Alexander Graham Bell Association

Infant Hearing
Screening and Testing
Meredith A. Holcomb, Au.D., CCC-A
Coordinator, MUSC Cochlear Implant Program
Instructor of Audiology
AG Bell / MUSC Pediatric Conference
October 18, 2013
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Hearing Loss Statistics
3-4/1000 live births have HL >30dB
1/1000 profound HL
10% with Auditory Neuropathy
In SC –
60,000 – 70,000 live births per year
120 - 140 children with HL per year
60 - 70 profound HL per year
12 – 14 children with AN per year
– 1-2 unilateral AN
– 10-12 bilateral AN
Background – Infant Hearing Screening
1988:
– Maternal Child Health Bureau funded newborn screening pilots in 3 states
1993:
– National Institutes of Health issued consensus statements stating all NICU
babies should be screened before hospital discharge and universal screening
should occur for all infants within first 3 months of life
1994:
– Joint Committee on Infant Hearing (JCIH) position statement endorsed goal of
universal detection of infants with HL
1999:
– American Academy of Pediatrics released statement recommending newborn
hearing screening and intervention
2000:
– JCIH position statement
2007:
– JCIH position statement (changes from 2000)
2013:
– Supplement to 2007 JCIH position statement
Joint Committee on Infant Hearing
– Member Organizations
American Academy of Audiology
American Speech-Language-Hearing Association
American Academy of Pediatrics
American Academy of Otolaryngology – HNS
Alexander Graham Bell Association
Council on Education of the Deaf
Directors of Speech and Hearing Programs in State Agencies
– Supporting Organizations
Centers for Disease Control and Prevention
US Maternal and Child Health Bureau
Boys Town National Research Hospital
JCIH Principles
1) All infants should have access to hearing screen by
1 mo of age
2) All infants who do not pass the initial screen and
subsequent re-screen should have audiological
confirmation of hearing loss by 3 mo of age
3) All infants with confirmed hearing loss should receive
intervention services by 6 mo of age
4) EHDI system should be family centered
Birth
Hospital
Other
Providers
Family
Educators
Pediatrician
Child
SpeechLanguage
Pathologist
Audiologist
ENT
Early
Interventionist
JCIH Principles
5) Child and family should have immediate access to highquality technology (HA, CI, assistive devices)
6) All infants and children should be monitored for HL in the
medical home
7) Interdisciplinary intervention programs should be
provided by knowledgeable professionals for HI children
and families
8) Information services should be used to measure
outcomes and report effectiveness of EHDI services
Communication is Key
Birth hospital should convey hearing screen results to
parents and medical home
Parents should receive appropriate f/u and resource
information
Information should be communicated in culturally
sensitive and understandable format
Results of hearing screens and evals should be promptly
sent to medical home and state
Families should be made aware of ALL communication
options and available hearing technologies
Early Intervention
Any degree of bilateral or unilateral permanent HL,
including ANSD
Central referral points of entry for specialty services for
infants with HL
Services provided by knowledgeable professionals with
expertise in HL
Families should be offered home-based and centerbased intervention options
Connect with other families with children with hearing
loss
**EI services should begin no later
than 6 mo of age!
JCIH 2007: What changes
were needed?
Definition of Targeted Hearing Loss
Expanded from congenital bilateral and
unilateral sensory or permanent
conductive hearing loss to include neural
hearing loss
– Auditory Neuropathy Spectum Disorder
–
–
–
–
–
Normal Cochlear Function (OAE testing)
Abnormal ABR with Cochlear Microphonic
Hearing loss
Speech/language delays
Poorer than expected benefit with hearing aids
Initial Hearing Screen/Rescreen
Protocol Changes
Recommends separate protocols for NICU and wellbaby nurseries:
– NICU
10-15% of newborns spend time in NICU at birth
This population is at risk for neural hearing loss
ABR is the only appropriate screening technique for NICU
If do not pass ABR initial screen, referral should be made directly to
audiologist for rescreen via ABR
– Well-Baby Nursery
OAE or ABR screen can be used, but only ABR will detect neural
HL
If do not pass initial ABR, should not be rescreened using OAE
Outpatient Rescreening
Should occur within 1 month of hospital discharge
Both ears should be rescreened always!
If infant is readmitted to hospital within first month of life
when there are conditions associated with potential
hearing loss, repeat hearing screen is necessary
JCIH states okay to rescreen with OAE as long as one
ear passed ABR at birth
MUSC Infant Screening
Recommendations
All babies should receive ABR initial screen
All babies should be rescreened with ABR
OAE should NOT be used alone as hearing
screen – will likely miss ANSD diagnosis
High frequency tympanogams (1000 Hz probe
tone)
Both ears should be retested
Diagnostic Audiology Evaluation
Otoscopy
High-frequency (1000 Hz) probe tone
tympanogram
OAE – if middle ear status is clear
ABR testing
ASSR testing??
Diagnostic Audiology Evaluation
For children younger than age 3 years, one ABR test is
recommended as part of the completed audiology
diagnostic evaluation for confirmation of permanent
hearing loss
Re-evaluations for infants with risk factors for late onset
HL should be customized and individualized
Infants who pass initial screen but have risk factors
should have at least one diagnostic audiology evaluation
by 24-30 mo of age
For families who elect hearing aids, fitting should occur
within one month of diagnosis of HL
Otoacoustic Emission (OAE)
Testing
– Measurements obtained
from ear canal with probe
– Records cochlear
responses to acoustic
stimuli
– Reflects status of
peripheral auditory
system extending to the
cochlear outer hair cells
– Will NOT identify
Auditory Neuropathy
OAEs – Pros & Cons
Pros of OAEs
– Frequency specific
– Present at birth
– Infant can be awake for testing
Cons of OAEs
–
–
–
–
Only provides info about OHC status
Requires normal middle ear function
Response altered by ambient noise
Does not indicate degree of hearing loss
Auditory Brainstem Response
(ABR) Testing
– Measurements obtained from
surface electrodes
– Records neural activity in
cochlea, auditory nerve, and
brainstem in response to
auditory stimuli
– Reflects status of peripheral
auditory system, 8th nerve,
and brainstem auditory
pathway
– Will identify Auditory
Neuropathy
ABR – Pros & Cons
Pros of ABR
– Indicates degree of hearing loss
– Assesses greater area of the auditory
pathway
Cons of ABR
– Restrictive frequency specificity due to abrupt
stimulus
– Assesses only synchronous neural function
– Infant must be asleep for testing
ASSR (Auditory Steady State Response)
Pros & Cons
Pros of ASSR
– Uses amplitude modulated tone – more
frequency specific
– Can stimulate at higher levels than ABR
Cons of ASSR
– Inaccurate estimates of threshold in cases of
normal hearing to mild hearing loss
Diagnostic ABR Testing
Insert Earphones
Natural Sleep or Sedation
Two Runs Per Test Scenario
Waves Must Be Repeatable
Diagnostic ABR Testing
ToneBurst Stimuli
4000 Hz, 1000 Hz, 500 Hz, 2000 Hz
Single Polarity Click Stimulus (high intensity)
If no response on 4000 Hz toneburst
Looking for Cochlear Microphonic (CM) to
diagnose Auditory Neuropathy
– Must reverse polarity when stimulus phase is inverted
– No sound run – to differentiate between stimulus artifact
and CM
Bone conduction testing
Medical Evaluation
Otolaryngologist evaluation
– With knowledge of pediatric hearing loss
Genetics consultation
Ophthalmologist evaluation
Speech evaluation
Current Challenges
Lost to follow-up
Shortage of qualified professionals
Lack of timely referrals
Lack of timely intervention
Lack of funding
Reimbursement issues
Interesting Case
Case Study
Full-term infant
ABR newborn screen at birth
– Pass R, refer L
OAE rescreen (child would not sleep for
ABR screen)
– Pass R, some OAE responses L
– Normal tympanograms
Scheduled for diagnostic ABR
Case StudyToneBurst
Testing
Case Study – ABR (clicks)
Case Study
Sent to ENT for medical work-up
MRI ordered to evaluate cochlear nerves
Child found to have cochlear nerve
deficiency
Key Points To Remember
1 month – hearing screen
3 months – diagnose hearing loss
6 months – early intervention services
Child/family is the focus
ABR screening is optimal
WHEN IN DOUBT, REFER!!!
Thank you!