Definition

Auditory neuropathy (AN) is when the nerve system of the inner ear doesn’t process sounds coming from the outer ear.

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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 one 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
• Damaged nerve
• Nerve problems

Risk Factors

You may have a higher risk if you also have:

• People in your family who have had hearing loss
• Lack of oxygen at birth
• Very low birth weight
• Jaundice after birth
• Gilbert syndrome —a genetic disorder
• Infections, such as mumps
• Problems with your 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 —genetic problem that causes tumors in the nerves
• Trauma

Symptoms

AN may cause:

• White noise—the sound is heard, but the word is not clear
• Sounds to tune in and out
• Words and sounds that seem out of sync
• Ringing in the ears— tinnitus

The level of hearing loss can vary.

Diagnosis

Your doctor will ask about your symptoms and health history.

You may have:

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

Treatment

Treating AN involves:

• Saving the hearing you have right now
• Restoring lost hearing
• Finding new ways of communicating

Talk with your doctor about the best plan for you. You may:

• Work with a team, such as::
  • Otolaryngologist (ENT)—doctor specializing in problems of the ear, nose, and throat
  • Audiologist—hearing specialist
  • Speech-language pathologist—communication problems specialist
• Using technology, such as:
  • Cochlear implants —surgically implanted devices that excite the nerve to send information to the brain
  • Hearing aids
  • Listening devices such as frequency modulation (FM) systems
• Having speech-language therapy, such as:
  • Sign language
  • Speech-reading—also known as lip-reading
  • Drills that join listening skills with technology

Prevention

In many cases, the cause of AN is unknown.

These steps may help:

• If you are pregnant, ask your doctor what you can do to try not to get an infection.
• Talk to your doctor if you have any health problems that are linked to AN.