What is Autoimmune Inner Ear Disease

Autoimmune Inner Ear Disease occurs when the immune system mistakes cells in the inner ear as a virus and attacks them. Patients usually begin experiencing hearing loss in one ear as the immune system kills the cells in your inner ear. If left untreated, it will eventually spread to the other ear and cause lasting damage.

It's a rare disease afflicting less than 1 percent of all Americans suffering from hearing loss, and therefore is often misdiagnosed by doctors.
autoimmune inner ear disease
Unfortunately, many people suffering from Autoimmune Inner Ear Disease also suffer from other types of autoimmune disorders. For example, your immune system may attack the eyes or entire body in addition to the ear.

Even if the body isn't directly attacking the inner ear, debris from the body attacking other areas of itself can be deposited in the inner ear and cause damage.

What Causes Autoimmune Inner Ear Disease

While there is no solid evidence to prove the cause of Autoimmune disease, there is a theory that drugs or viruses can cause the body to confuse helpful and harmful tissue. Genetics may also play a role in it as some people being more susceptible to the disease than others.

Autoimmune Inner Ear Disease Symptoms

Autoimmune Inner Ear Disease symptoms usually include:
  • hearing loss,
  • tinnitus, and
  • vertigo.

Medical professionals often misdiagnose it as an ear infection as most of the symptoms overlap. As this disease causes permanent damage, it's important to catch it early to prevent permanent hearing loss from spreading.

Treatment Options

Unfortunately, because the disease is rare, there isn't an effective test to diagnose it. Many doctors will perform several balancing tests to see how your body is connecting with your brain. They may also do bloodwork, but test results are often inconclusive.

More commonly, your doctor will prescribe medication and treat you as though you do have Autoimmune Inner Ear Disease. If your body responds positively to treatment, you will usually be diagnosed after the fact.
During the first few weeks, your doctor will probably recommend steroids. Unfortunately, while steroids are very effective, they often have strong side effects. One study showed that hyperglycemia was the most common side effect occurring in 17 percent of patients, while weight gain came in a close second.

Due to the side effects of steroids, your doctor will probably move you to a long-term medication after a few weeks. Most long-term medications include methotrexate, azathioprine, and cyclophosphamide.
Many patients also use hearing aids to compensate for any hearing they have lost in the process.


Despite the rarity of Autoimmune Inner Ear Disease, scientists are working on new treatments. Gene therapy is a solution that helps damaged ear cells function properly again and restore hearing. While it isn't perfect yet, scientists are hopeful it will be successful soon.

While Autoimmune Inner Ear Disease is a serious disease, catching it early and getting on a treatment plan will allow you to retain some hearing and function in daily activities.