What is it?

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.

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Auditory neuropathy.

Signs & symptoms

* mild to severe hearing loss.
* sounds fading in and out.
* difficulty understanding spoken words (speech perception)
* normal hearing but with poor speech perception.
* worsened speech perception in noisy environments.

Diagnosis

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.

Treatment

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).

☝️ This is not a substitute for professional medical advice. Please consult with your physician before making any medical decision.

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