Auditory Neuropathy

U.S.
DEPARTMENT OF HEALTH AND HUMAN SERVICES ∙ National Institutes of Health
NIDCD Fact Sheet | Hearing and Balance
Auditory Neuropathy
What is auditory neuropathy? the nervous system to the brain. In other cases, the
cause may involve damage to the auditory neurons
Auditory neuropathy is a hearing disorder in which that transmit sound information from the inner hair
the inner ear successfully detects sound, but has a cells to the brain. Other possible causes may include
problem with sending sound from the ear to the brain. inheriting genes with mutations or suffering damage to
It can affect people of all ages, from infancy through the auditory system, either of which may result in faulty
adulthood. The number of people affected by auditory connections between the inner hair cells and the auditory
neuropathy is not known, but current information nerve (the nerve leading from the inner ear to the brain),
suggests that auditory neuropathies play a substantial or damage to the auditory nerve itself. A combination of
role in hearing impairments and deafness. these problems may occur in some cases.
When their hearing sensitivity is tested, people with
auditory neuropathy may have normal hearing or hearing
loss ranging from mild to severe. They always have
poor speech-perception abilities, meaning that they
have trouble understanding speech clearly. People with Auditory neuropathy is a
auditory neuropathy have greater impairment in speech
perception than hearing health experts would predict hearing disorder in which
based upon their degree of hearing loss on a hearing
test. For example, a person with auditory neuropathy the inner ear successfully
may be able to hear sounds, but would still have difficulty
recognizing spoken words. Sounds may fade in and out detects sound, but has
or seem out of sync for these individuals.
a problem with sending
What causes auditory neuropathy?
Researchers report several causes of auditory neuropathy.
sound from the ear to
In some cases, the cause may involve damage to the
inner hair cells—specialized sensory cells in the inner
the brain.
ear that transmit information about sounds through
NIDCD...Improving the lives of people with communication disorders

What are the roles of the outer and
inner hair cells? Health professionals—
Outer hair cells help amplify sound vibrations entering
the inner ear from the middle ear. When hearing is
including otolaryngologists
working normally, the inner hair cells convert these
vibrations into electrical signals that travel as nerve (ear, nose, and throat
impulses to the brain, where the brain interprets the
impulses as sound. doctors), pediatricians,
Although outer hair cells—hair cells next to and more
numerous than inner hair cells—are generally more prone
and audiologists—use a
to damage than inner hair cells, outer hair cells seem to
function normally in people with auditory neuropathy.
combination of methods
Are there risk factors for auditory
to diagnose auditory
neuropathy? neuropathy.
There are several ways that children may acquire auditory
neuropathy. Some children diagnosed with auditory
neuropathy experienced particular health problems
before or during birth or as newborns. These problems How is auditory neuropathy
include inadequate oxygen supply during or prior to
birth, premature birth, jaundice, low birth weight, and
diagnosed?
dietary thiamine deficiency. In addition, some drugs used Health professionals—including otolaryngologists
to treat pregnant women or newborns may damage (ear, nose, and throat doctors), pediatricians, and
the baby’s inner hair cells, causing auditory neuropathy. audiologists—use a combination of methods to diagnose
Adults may also develop auditory neuropathy along with auditory neuropathy. These include tests of auditory
age-related hearing loss. brainstem response (ABR) and otoacoustic emissions
(OAE). The hallmark of auditory neuropathy is an absent
Auditory neuropathy runs in some families, and in some
or very abnormal ABR reading together with a normal
cases, scientists have identified genes with mutations
OAE reading. A normal OAE reading is a sign that the
that compromise the ear’s ability to transmit sound
outer hair cells are working normally.
information to the brain. Thus, inheritance of mutated
genes is also a risk factor for auditory neuropathy. An ABR test uses electrodes placed on a person’s head
and ears to monitor brain wave activity in response
Some people with auditory neuropathy have neurological
to sound. An OAE test uses a small, very sensitive
disorders that also cause problems outside of the hearing
microphone inserted into the ear canal to monitor the
system. Examples of such disorders are Charcot-Marie-
faint sounds produced by the outer hair cells in response
Tooth syndrome and Friedreich’s ataxia.
to auditory stimulation. ABR and OAE testing are painless
and can be used for newborn babies and infants as well
as older children and adults. Other tests may also be used
as part of a comprehensive evaluation of an individual’s
hearing and speech-perception abilities.
2
Does auditory neuropathy ever get severely impaired. Adults with auditory neuropathy
better or worse? and older children who have already developed spoken
language may benefit from learning how to speechread
Some newborn babies who have been diagnosed with (also known as lip reading).
auditory neuropathy improve and start to hear and speak
within a year or two. Other infants stay the same, while What research is being done on
some get worse and show signs that the outer hair cells
auditory neuropathy?
no longer function (abnormal otoacoustic emissions). In
people with auditory neuropathy, hearing sensitivity can Scientists have identified genes involved in causing some
remain stable, get better or worse, or gradually worsen, cases of auditory neuropathy, and are working to identify
depending on the underlying cause. what goes wrong in the auditory system when a person
inherits a mutant gene. Researchers are also continuing
What treatments, devices, and other to investigate the potential benefits of cochlear implants
approaches can help people with for children with auditory neuropathy, and are examining
why cochlear implants may benefit some people with the
auditory neuropathy to communicate? condition but not others.
Researchers are still seeking effective treatments
for people with auditory neuropathy. Meanwhile,
professionals in the hearing field differ in their opinions
about the potential benefits of hearing aids, cochlear
implants, and other technologies for people with auditory
neuropathy. Some professionals report that hearing
aids and personal listening devices such as frequency
modulation (FM) systems are helpful for some children
and adults with auditory neuropathy. Cochlear implants
(electronic devices that compensate for damaged or
nonworking parts of the inner ear) may also help some
people with auditory neuropathy. No tests are currently
available, however, to determine whether an individual
with auditory neuropathy might benefit from a hearing
aid or cochlear implant.
Debate also continues about the best ways to educate

and improve communication skills in infants and
children who have hearing impairments such as auditory
neuropathy. One approach favors sign language as the
child’s first language. A second approach encourages
the use of listening skills—together with technologies
such as hearing aids and cochlear implants—and spoken
language. A combination of these two approaches may
also be used. Some health professionals believe it may be
especially difficult for children with auditory neuropathy
to learn to communicate only through spoken language
because their ability to understand speech is often
3
Where can I find additional For more information, contact us at:
information about auditory

NIDCD Information Clearinghouse
neuropathy? 1 Communication Avenue
Bethesda, MD 20892-3456
The NIDCD maintains a directory of organizations that
Toll-free voice: (800) 241-1044
provide information on the normal and disordered
Toll-free TTY: (800) 241-1055
processes of hearing, balance, taste, smell, voice, Email: nidcdinfo@nidcd.nih.gov
speech, and language. Visit the NIDCD website
at https://www.nidcd.nih.gov/directory to search https://www.nidcd.nih.gov
the directory. Follow the NIDCD on Twitter at @NIDCD
More NIDCD fact sheets on Hearing and Balance:

} American Sign Language
The NIDCD supports and conducts research and
} Assistive Devices for People with Hearing, Voice, research training on the normal and disordered
Speech, or Language Disorders processes of hearing, balance, taste, smell, voice,
} Cochlear Implants speech, and language and provides health information,
based upon scientific discovery, to the public.
} Telecommunication Relay Services
} Usher Syndrome
} Your Baby’s Hearing Screening
Visit the NIDCD website at https://www.nidcd.nih.gov to

read, print, or download fact sheets.
Auditory Neuropathy
NIH Pub. No. 03-5343
September 2016
NIH...Turning Discovery Into Health®

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U.S.

DEPARTMENT OF HEALTH AND HUMAN SERVICES ∙ National Institutes of Health

NIDCD Fact Sheet | Hearing and Balance

NIDCD...Improving the lives of people with communication disorders

Debate also continues about the best ways to educate

information about auditory

More NIDCD fact sheets on Hearing and Balance:

Visit the NIDCD website at https://www.nidcd.nih.gov to

NIH...Turning Discovery Into Health®

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