What Is Auditory Neuropathy?

Auditory neuropathy is a rare form of hearing loss seen in some patients that occurs because of a break in the connection between the ear and the brain. These patients have healthy ears with no physical problems that might contribute to their hearing loss, but something has gone wrong along the auditory nerves, making it difficult to hear. Patients can have mild to severe auditory neuropathy, and there are a variety of treatment approaches available, rooted in different philosophies about hearing loss.

This condition can be diagnosed with a series of tests that are painless and minimally invasive. A doctor may recommend screening for auditory neuropathy in an infant or child who does not appear to respond normally to auditory stimuli, and in some regions routine screening is recommended for all children to catch issues early. An audiologist can perform the tests and determine if hearing loss is present, why it is occurring, and how severe it is. This information can be important for treatment.

The causes of auditory neuropathy are not well understood. It could be the result of physical damage, and it may have a genetic component as well. Some patients spontaneously recover while others do not. All patients have difficulty with speech comprehension, as the ability to understand speech can require a finely tuned sense of hearing. This can contribute to learning disabilities and may also make speech acquisition difficult, as infants and young children develop speaking skills by listening to the adults around them.

One option for management of auditory neuropathy is the use of hearing aids and implants to improve the patient's hearing. These can help the patient comprehend speech and hear a range of sounds that would not otherwise be perceptible. The patient may also benefit from speech therapy and accommodations like sitting up front in a classroom to be able to clearly hear the instructor. The doctor may recommend periodic reassessments to see how well the patient responds to treatment.

Other patients may be approached in a different way. Instead of receiving treatment to address the hearing loss, the patient might learn sign language, lipreading, and other tools for communication that do not require hearing. These treatment options are more common in regions with a lively Deaf culture, where people with hearing loss do not consider it a problem that needs to fixed. Patients who learn sign language may be presented with the option for hearing aids as they age and are able to make an informed decision about what kind of treatment they want.