Auditory Neuropathy

Causes

The outer ear sends vibrations to the inner ear. There, hair cells break them into electrical signals. These are sent to the brain. It filters them as sound.

AN may be due to 1 or more of these causes:

• Problems with the hair cells in the inner ear
• Bad links between the hair cells and the nerve to the brain
• A damaged nerve
• Nerve problems

Risk Factors

Things that may raise the risk of AN are:

• Other family members who have had hearing loss
• Lack of oxygen at birth or having a very low birth weight
• Jaundice after birth
• Gilbert syndrome—a genetic disorder
• Infections, such as mumps
• Problems with the immune system
• Being around chemicals or medicines that cause hearing loss, such as some chemotherapies
• Tumors of the nerve or those that press on the nerve
• Neurofibromatosis type 2—a genetic problem that causes tumors in the nerves
• Trauma

Symptoms

A person with AN may hear:

• Sounds that are not clear enough to hear
• Sounds that tune in and out
• Words and sounds that seem out of sync
• Ringing in the ears

Diagnosis

The doctor will ask about symptoms and health history. A physical exam will be done.

These tests may be done:

• Auditory brainstem response (ABR) to measure brainwaves
• Otoacoustic emissions (OAE) to record how the cells in the ear react to clicking sounds

Treatment

The goals of treatment are to:

• Save the person's current level of hearing
• Restore lost hearing
• Learn new ways to communicate

Treatment options are:

• Devices to help with hearing, such as hearing aids, listening devices, and cochlear implants
• Speech-language therapy, such as sign language and speech reading

Prevention

There are no known guidelines to prevent AN.