U.S. DEPARTMENT OF HEALTH AND HUMAN SERVICES ∙ National Institutes of Health

NIDCD Fact Sheet | Hearing and Balance

Auditory Neuropathy

What is auditory neuropathy?

Auditory neuropathy is a hearing disorder in which 

the inner ear successfully detects sound, but has a 

problem with sending sound from the ear to the brain. 

It can affect people of all ages, from infancy through 

adulthood. The number of people affected by auditory 

neuropathy is not known, but current information 

suggests that auditory neuropathies play a substantial 

role in hearing impairments and deafness.

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 have greater impairment in speech 

perception than hearing health experts would predict 

based upon their degree of hearing loss on a hearing 

test. For example, a person with auditory neuropathy 

may be able to hear sounds, but would still have difficulty 

recognizing spoken words. Sounds may fade in and out 

or seem out of sync for these individuals.

What causes auditory neuropathy?

Researchers report several causes of auditory neuropathy. 

In some cases, the cause may involve damage to the 

inner hair cells—specialized sensory cells in the inner 

ear that transmit information about sounds through 

the nervous system to the brain. In other cases, the 

cause may involve damage to the auditory neurons 

that transmit sound information from the inner hair 

cells to the brain. Other possible causes may include 

inheriting genes with mutations or suffering damage to 

the auditory system, either of which may result in faulty 

connections between the inner hair cells and the auditory 

nerve (the nerve leading from the inner ear to the brain), 

or damage to the auditory nerve itself. A combination of 

these problems may occur in some cases. 

Auditory neuropathy is a 

hearing disorder in which 

the inner ear successfully 

detects sound, but has 

a problem with sending 

sound from the ear to 

the brain.

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What are the roles of the outer and 

inner hair cells?

Outer hair cells help amplify sound vibrations entering 

the inner ear from the middle ear. When hearing is 

working normally, the inner hair cells convert these 

vibrations into electrical signals that travel as nerve 

impulses to the brain, where the brain interprets the 

impulses as sound.

Although outer hair cells—hair cells next to and more 

numerous than inner hair cells—are generally more prone 

to damage than inner hair cells, outer hair cells seem to 

function normally in people with auditory neuropathy.

Are there risk factors for auditory 

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 

include inadequate oxygen supply during or prior to 

birth, premature birth, jaundice, low birth weight, and 

dietary thiamine deficiency. In addition, some drugs used 

to treat pregnant women or newborns may damage 

the baby’s inner hair cells, causing auditory neuropathy. 

Adults may also develop auditory neuropathy along with 

age-related hearing loss. 

Auditory neuropathy runs in some families, and in some 

cases, scientists have identified genes with mutations 

that compromise the ear’s ability to transmit sound 

information to the brain. Thus, inheritance of mutated 

genes is also a risk factor for auditory neuropathy. 

Some people with auditory neuropathy have neurological 

disorders that also cause problems outside of the hearing 

system. Examples of such disorders are Charcot-MarieTooth syndrome and Friedreich’s ataxia.

Health professionals—

including otolaryngologists 

(ear, nose, and throat 

doctors), pediatricians, 

and audiologists—use a 

combination of methods 

to diagnose auditory 

neuropathy.

How is auditory neuropathy 

diagnosed?

Health professionals—including otolaryngologists 

(ear, nose, and throat doctors), pediatricians, and 

audiologists—use a combination of methods to diagnose 

auditory neuropathy. These include tests of auditory 

brainstem response (ABR) and otoacoustic emissions 

(OAE). The hallmark of auditory neuropathy is an absent 

or very abnormal ABR reading together with a normal 

OAE reading. A normal OAE reading is a sign that the 

outer hair cells are working normally.

An ABR test uses electrodes placed on a person’s head 

and ears to monitor brain wave activity in response 

to sound. An OAE test uses a small, very sensitive 

microphone inserted into the ear canal to monitor the 

faint sounds produced by the outer hair cells in response 

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.
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Does auditory neuropathy ever get 

better or worse?

Some newborn babies who have been diagnosed with 

auditory neuropathy improve and start to hear and speak 

within a year or two. Other infants stay the same, while 

some get worse and show signs that the outer hair cells 

no longer function (abnormal otoacoustic emissions). In 

people with auditory neuropathy, hearing sensitivity can 

remain stable, get better or worse, or gradually worsen, 

depending on the underlying cause.

What treatments, devices, and other 

approaches can help people with 

auditory neuropathy to communicate?

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 

severely impaired. Adults with auditory neuropathy 

and older children who have already developed spoken 

language may benefit from learning how to speechread 

(also known as lip reading).

What research is being done on 

auditory neuropathy?

Scientists have identified genes involved in causing some 

cases of auditory neuropathy, and are working to identify 

what goes wrong in the auditory system when a person 

inherits a mutant gene. Researchers are also continuing 

to investigate the potential benefits of cochlear implants 

for children with auditory neuropathy, and are examining 

why cochlear implants may benefit some people with the 

condition but not others.
Where can I find additional 

information about auditory 

neuropathy? 

The NIDCD maintains a directory of organizations that 

provide information on the normal and disordered 

processes of hearing, balance, taste, smell, voice, 

speech, and language. Visit the NIDCD website 

at https://www.nidcd.nih.gov/directory to search 

the directory.

More NIDCD fact sheets on Hearing and Balance:

} American Sign Language

} Assistive Devices for People with Hearing, Voice, 

Speech, or Language Disorders

} Cochlear Implants

} 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.

For more information, contact us at:

NIDCD Information Clearinghouse

1 Communication Avenue 

Bethesda, MD 20892-3456 

Toll-free voice: (800) 241-1044 

Toll-free TTY: (800) 241-1055 

Email: nidcdinfo@nidcd.nih.gov

https://www.nidcd.nih.gov

 Follow the NIDCD on Twitter at @NIDCD

The NIDCD supports and conducts research and 

research training on the normal and disordered 

processes of hearing, balance, taste, smell, voice, 

speech, and language and provides health information, 

based upon scientific discovery, to the public.

Auditory Neuropathy

NIH Pub. No. 03-5343

September 2016

NIH...Turning Discovery Into Health®