Auditory Neuropathy

What is auditory neuropathy?

Auditory neuropathy is a hearing disorder in which sound enters the inner ear normally but the transmission of signals and the processing of those signals in the brain from the inner ear may be abnormal. It can affect people of all ages, from infancy through adulthood. The number of people affected by auditory neuropathy is not known, but the condition may affect a relatively large percentage of children who are deaf or hearing-impaired.

People with auditory neuropathy may have normal hearing, fluctuating hearing or hearing loss ranging from mild to severe; they always have poor speech-perception abilities, meaning they have trouble understanding speech clearly. Often, speech perception is worse than would be predicted by the degree of hearing loss. 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 for these individuals and seem out of sync.

What causes auditory neuropathy?

While the disease process is not completely understood, (and may be complex) there are a number of risk factors that seem to be associated with the development of difficulties in transmitting and recognizing the spoken word in these children. A genetic or family history may be present, or there may be a congenital abnormality of the brain or damage may occur after birth from lack of oxygen, or associated with severe jaundice or being born very premature and small. Drugs may damage the hearing pathway. Many children, however may not have any risk factors.

How is auditory neuropathy diagnosed?

Health professionals, including otolaryngologists (ear, nose, and throat doctors), pediatricians, and audiologists, use a combination of history and audiological tests to diagnose auditory neuropathy. These include tests of auditory brainstem response (ABR) and otoacoustic emissions (OAE). The hallmark of auditory neuropathy is an absent/negligible 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 monitors brain wave activity in response to sound using electrodes that are placed on the person’s head and ears. 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 stimulation by a series of clicks. 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 more comprehensive evaluation of an individual’s hearing and speech-perception abilities.

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 (otoacoustic emissions). In adults with auditory neuropathy, hearing can remain stable, fluctuate up and down, or progressively worsen, depending on the underlying cause.

What treatments, devices, and other approaches can help people with auditory neuropathy to communicate?

As symptoms vary with each individual child, management varies too. There are potential benefits with the use of hearing aids, cochlear implants, and other technologies like personal listening devices such as frequency modulation (FM) systems which may be helpful for some children.

Debate continues about the best ways to educate and provide communication skills for children who have hearing impairments such as auditory neuropathy. However, most hearing health experts agree that parents should work with a team of professionals who considers the situation and options for each child as well as the child’s family members and caregivers. Most also agree that parents and caregivers should interact often with infants who have auditory neuropathy by holding, facing, smiling at, and responding to the child.

Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: November 06, 2023 03:23 PM

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