Auditory neuropathy is a hearing disorder in which sound enters the inner ear normally, but the transmission of signals from the inner ear to the brain is impaired. People with auditory neuropathy may have normal hearing, or a hearing loss ranging from mild to severe. Treatments may include hearing aids, cochlear implants, and other technologies.
Auditory neuropathy is a hearing disorder in which sound enters the inner ear normally, but the transmission of signals from the inner ear to the brain is impaired. People with auditory neuropathy may have normal hearing, or a hearing loss ranging from mild to severe. They usually have trouble understanding speech clearly. 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. It can affect people of all ages, from infancy through adulthood.
Although auditory neuropathy is not yet fully understood, scientists believe the condition probably has more than one cause. In some cases, it may involve damage to the specialized sensory cells in the inner ear that transmit information about sounds through the nervous system to the brain. Other causes may include faulty connections between the inner hair cells and the nerve leading from the inner ear to the brain, or damage to the nerve itself. A combination of these problems may occur in some cases.
Auditory Neuropathy was first identified in the 1980s when advanced testing procedures became available to measure the action of the cells in the cochlea.
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. In adults with auditory neuropathy, hearing can remain stable, fluctuate up and down, or progressively worsen, depending on the underlying cause.
Researchers are still seeking effective treatments for people with auditory neuropathy. They may include 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. However, no tests are currently available to determine whether an individual with auditory neuropathy might benefit from a hearing aid or cochlear implant.
To read how the condition affected one child, click here.