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Auditory Neuropathy : Overview

What is auditory neuropathy?

Auditory neuropathy may be a 
hearing impairment during which the internal ear successfully detects sound, but features a problem with sending sound from the ear to the brain. It can affect people of all ages, from infancy through adulthood. The amount of individuals suffering from auditory neuropathy isn't known, but current information suggests that auditory neuropathies play a considerable role in hearing impairments and deafness.
When their hearing sensitivity is tested, people with auditory neuropathy may have normal hearing or deafness starting from mild to severe. They always have poor speech-perception abilities, meaning that they need trouble understanding speech clearly. People with auditory neuropathy have greater impairment in auditory perception than hearing health experts would predict based upon their degree of deafness on a hearing test. for instance , an individual with auditory neuropathy could also be ready 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 within the 
internal ear that transmit information about sounds through the systema nervosum 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 sensory system , either of which can end in faulty connections between the inner hair cells and therefore the acoustic nerve (the nerve leading from the internal ear to the brain), or damage to the acoustic nerve itself. a mixture of those problems may occur in some cases.

What are the roles of the outer and inner hair cells?

Outer hair cells help amplify sound vibrations entering the internal ear 
from the center ear. When hearing is functioning 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 susceptible 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 in which 
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 before birth, premature birth, jaundice, low birth weight, and dietary thiamine deficiency. additionally, some drugs wont to treat pregnant women or newborns may damage the baby’s inner hair cells, causing auditory neuropathy. Adults can also develop auditory neuropathy along side age-related deafness.


How is auditory neuropathy diagnosed?

Health professionals—including otolaryngologists (ear, nose, and throat doctors), pediatricians, and audiologists—use a mixture 
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 along side a traditional OAE reading. a traditional OAE reading may be a sign that the outer hair cells are working normally.

An ABR test uses electrodes placed on a person’s head and ears to watch 
brain wave activity in response to sound. An OAE test uses alittle , very sensitive microphone inserted into the auditory meatus to watch the faint sounds produced by the outer hair cells in response to auditory stimulation. ABR and OAE testing are painless and may be used for newborn babies and infants also as older children and adults. Other tests can also be used as a part of a comprehensive evaluation of an individual’s hearing and speech-perception abilities.


Does auditory neuropathy ever recover 
or worse?

Some newborn babies who are 
diagnosed with auditory neuropathy improve and begin to listen to and speak within a year or two. Other infants stay an equivalent , while some worsen and show signs that the outer hair cells not function (abnormal otoacoustic emissions). In people with auditory neuropathy, hearing sensitivity can remain stable, recover or worse, or gradually worsen, counting 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 within 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 private listening devices like FM (FM) systems are helpful for a few children and adults with auditory neuropathy. Cochlear implants (electronic devices that catch up on damaged or nonworking parts of the inner ear) can also help some people with auditory neuropathy. No tests are currently available, however, to work out whether a private with auditory neuropathy might enjoy a hearing aid or cochlear implant.

Debate also continues about the simplest ways to teach and improve communication skills in infants and youngsters who have hearing impairments like auditory neuropathy. One approach favors signing because the child’s mother tongue.


A second approach encourages the utilization of listening skills—together with technologies like hearing aids and cochlear implants—and speech . a mixture of those two approaches can also be used. Some health professionals believe it's going to be especially difficult for youngsters with auditory neuropathy to find out to speak only through speech because their ability to know speech is usually severely impaired. Adults with auditory neuropathy and older children who have already developed speech may enjoy learning the way to speechread (also referred to as lip reading).



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