What Is Autoimmune Inner Ear Disease?

Medically Reviewed by Carol DerSarkissian, MD on December 08, 2022
3 min read

Autoimmune inner ear disease (AIED), is a rare disease that happens when your body's immune system mistakenly attacks your inner ear. It can cause dizziness, ringing in your ears, and hearing loss.

Less than 1% of the 28 million Americans who have hearing loss have it because of AIED. It's slightly more common in middle-aged women.

If you have AIED, you'll have hearing loss that starts in one ear and then spreads to the other. This may take weeks, or it could happen over a few months.

Other symptoms can include:

  • Dizziness or problems with your balance
  • Fullness in your ear
  • Tinnitus (ringing, roaring, or hissing in your ear)
  • Vertigo (a sense that you're spinning)

Your immune cells are always on the lookout for germs trying to invade your body. If they mistake cells in your inner ear for a virus or bacteria, they attack them. This is called an autoimmune reaction.

Your immune cells may harm other organs as well. Just under 30% of people who have AIED have another autoimmune disease that affects their whole body, such as rheumatoid arthritis, lupus, scleroderma, ulcerative colitis, or Sjoegren's syndrome (dry eye syndrome).

Because the symptoms of AIED are so common, it can be hard to diagnose. Many times, it's mistaken for an ear infection until hearing loss has spread to the second ear.

To diagnose AIED, your doctor will ask questions about your health and medical history, do a physical exam, and give you a hearing test. They'll also test your balance, which can show how well your inner ear is "talking" to your brain. You might also have blood work done.

There's no test that can tell for sure that you have AIED, but the results may show that you're having an autoimmune reaction. If they do, it's a good idea to see an otolaryngologist (ear doctor) who's also trained in autoimmune disorders.

Since you may not get a clear answer, your doctor may start you on treatment without a sure diagnosis to prevent damage to your hearing that can't be fixed. Many people aren't diagnosed with AIED until they start treatment and their symptoms get better.

Your doctor probably will give you a drug that helps with inflammation. High doses of steroids have been shown to work well for AIED, but they have many side effects. So you probably won't take them for more than a few weeks.

After you take the steroids, your doctor may prescribe a medication that can calm down your immune system. Other drugs like azathioprine (Imuran), cyclophosphamide (Cytoxan), and methotrexate are sometimes used for this.

A hearing aid can help you adjust to hearing loss, but in severe cases, your doctor might suggest a cochlear implant. This is a small device that affects the nerves in your inner ear that send signals to your brain. There, the brain turns them into sound. Part of the cochlear implant sits behind your ear. The other part is put under your skin during surgery.

As doctors learn more about AIED, more treatment options may be possible. These include drugs that work better with fewer side effects as well as gene therapy. New genes may be used to help damaged ear cells start working again.