Autoimmune Inner Ear Disease

Autoimmune inner ear disease (AIED) is a rare type of hearing loss. It is a form of sensorineural hearing loss. This means it impacts the way sound is processed in the inner ear and carried to the brain. AIED is responsible for only 1 percent of all cases of sensorineural hearing loss.1

Other types of hearing loss include conductive hearing loss. This occurs when sound is physically blocked from getting into the ear. An example of this is earwax inside the ear or fluid from an ear infection.1

What causes AIED?

As its name implies, AIED is an autoimmune issue. Similar to how the body mistakenly attacks the joints in rheumatoid arthritis, AIED happens when the body attacks the inner ear. The inner ear structures and the nerves related to them are how we process and hear sounds. The immune system thinks parts of the inner ear are foreign invaders (like bacteria or viruses).1-3

The body starts making antibodies and releasing other inflammation-causing cells directed at the inner ear and causes damage. The exact targets of this reaction are not well understood. Some experts think the blood vessels, nerves, or proteins of the inner ear are specifically attacked. It is unclear why the autoimmune process starts in the first place. The damage caused in the inner ear leads to hearing loss.1-3

Signs and symptoms

AIED may start with symptoms in one ear only. However, in many cases, it will progress to involve both ears. The amount of hearing loss may vary in each ear. Primary AIED occurs on its own, with only hearing loss present. Secondary AIED occurs alongside another autoimmune condition like RA.1-3

Hearing loss from AIED develops relatively quickly. Although the extent of hearing loss can change over time, it most often progresses over 3 to 90 days. Specific symptoms of AIED may vary, but include:1-3

  • Ringing in the ears (tinnitus)
  • Hissing or roaring sound in the ears
  • Ear fullness
  • Dizziness or balance problems
  • Feeling that you are spinning (vertigo)

How is AIED diagnosed?

There are many things that can cause hearing loss. AIED is especially hard to diagnose because there are no specific tests for it. Doctors will perform hearing tests, ask more about symptoms, and look inside the ear to start the process. A person with AIED will also undergo hearing tests.1-3

Certain blood tests may be helpful to look for signs of other underlying immune system problems. The tests are especially helpful if a person has signs of a full-body autoimmune condition, like muscle aches, joint pain, fevers, or fatigue.

In some situations, brain imaging (like an MRI) may be helpful to look for other underlying causes of hearing loss related to the brain.1-3

Since AIED is hard to diagnose, it is often a diagnosis of exclusion. This means other easily identifiable causes are pursued first. If there is no obvious cause of hearing loss, a person may be diagnosed with AIED. Some cases of AIED respond to steroids. Because of this, steroids may be used early on to see if hearing improves. If a person’s hearing improves with steroid use, it is more likely they have AIED.1-3

Treatment

As mentioned, steroids may be helpful in AIED. The sooner a person with AIED is treated with steroids, the more likely they are to have some improvement in their hearing loss.

If a person does not respond to steroids, other immune system-suppressing medications may be used. These include drugs called methotrexate, rituximab, and cyclophosphamide, among others.

Outside of drugs, hearing aids or cochlear implants may improve symptoms.1-3

Since AIED is potentially reversible early on, seeing a doctor as soon as you notice signs of sudden hearing loss is always a good idea. Sharing all symptoms, both related to your hearing as well as the rest of your body, can help lead to the correct diagnosis. If you are concerned about developing AIED, talk with your doctor about your risk.1-3

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Written by: Casey Hribar | Last reviewed: March 2021