Download PE2069 Auditory Neuropathy Spectrum Disorder (ANSD)

Patient and Family Education
Auditory Neuropathy
Spectrum Disorder (ANSD)
ANSD stands for
auditory neuropathy
spectrum disorder. It
is a type of hearing
loss where sound
enters the ear
normally but it isn’t
properly transmitted
What is ANSD?
ANSD is a type of hearing loss in which the inner ear (cochlea) receives sound
normally but there is a problem in how that sound is transmitted to the brain.
This may be due to a breakdown in how the auditory signals travel from the
inner ear to the brain via the hearing nerve, or there may be damage to the
hearing nerve itself.
How is ANSD identified?
ANSD is identified by a series of tests. These include:
Evoked Otoacoustic Emission Evaluation (called the “EOAE”)
This test measures how the outer hair cells in your child’s cochlea work. It is
done by recording an echo in response to sound signals using a small probe
inserted into the ear canal.
Brainstem Auditory Evoked Response (called the “BAER”)
This test measures how the cochlea and hearing nerve respond to sound while
your child is sleeping.
When a child has normal EOAE (echo) test results and absent or abnormal
BAER responses, this may mean they have ANSD. Note that over time, the
echo may disappear. We may also assess how your child’s ears respond to
loud acoustic stimuli or sounds using special tests such as acoustic reflexes
and cochlear microphonic.
How can ANSD affect hearing?
• Hearing loss may or may not be present
• Hearing loss can range from mild to profound and can change in degree
over time
• Hearing loss can be in one ear or both ears
• A child with ANSD may have difficulty understanding speech
• Often speech understanding may not match behavioral hearing test results
• A child’s hearing and ability to understand speech may fluctuate
• Some children with ANSD have trouble hearing in noisy conditions and
other children are not able to hear in any condition
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Auditory Neuropahty Spectrum Disorder (ANSD)
To Learn More
• Audiology
206-987-5173
• Ask your child’s
healthcare provider
• www.seattlechildrens.org
What places a child at increased risk for ANSD?
•
•
•
•
Premature birth
Jaundice in newborns
Treatment with medications that can affect hearing (ototoxic medications)
Genetic sydromes such as Charcot-Marie-tooth Disease, Friedreich’s
Ataxia and other genetic conditions that affect the hearing nerve
• Oxygen deprivation at birth
How is ANSD managed?
Free Interpreter
Services
• In the hospital, ask
your child’s nurse.
• From outside the
hospital, call the
toll-free Family
Interpreting Line
1-866-583-1527. Tell
the interpreter the
name or extension you
need.
The goal of treatment is to work closely with your child’s healthcare team to
manage your child’s ANSD. This may include:
• Closely monitoring your child’s speech and language development.
• Evaluating and monitoring your child’s listening skills by an aural
habilitation therapist.
• Regular behavioral hearing tests every 3 to 4 months starting when your
child is 7 to 9 months old.
• Referral to early intervention or school based services, depending on your
child’s age.
• Recommendations for hearing devices or other assistive listening devices
may be made based on your child’s behavioral test results.
• In some cases, a cochlear implant (CI) may be considered in children who
demonstrate significant degrees of hearing loss.
Seattle Children’s offers interpreter services for Deaf, hard of hearing or non-English speaking patients, family members and
legal representatives free of charge. Seattle Children’s will make this information available in alternate formats upon request.
Call the Family Resource Center at 206-987-2201.
This handout has been reviewed by clinical staff at Seattle Children’s. However, your child’s needs are unique. Before you act
or rely upon this information, please talk with your child’s healthcare provider.
© 2015 Seattle Children’s, Seattle, Washington. All rights reserved.
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PE2069
Audiology
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