Autoimmune inner ear disease (AIED) is a rare condition in which the immune system attacks and damages the inner ear. It causes a variety of irritating symptoms, including dizziness, tinnitus and hearing loss. Let’s take a look at the causes of AIED, the symptoms, how it’s diagnosed and how it’s treated.

What causes autoimmune ear disease?

AIED is caused by the body’s immune system incorrectly responding to cells in the inner ear as if they were a virus or bacteria. Antibodies attack the inner ear and this leads to inflammation of the blood vessels in the area. When the tissue is inflamed the ear cannot function as normal and this is why hearing loss takes place.

It isn’t clear why AIED occurs, but some people with the condition also have other autoimmune diseases such as rheumatoid arthritis, lupus and ulcerative colitis. These conditions are all ‘systemic’ which means they affect the entire body. Middle-aged women experience AIED more frequently than other demographics, but it isn’t clear why.

What are the symptoms of autoimmune inner ear disease?

AIED causes gradual hearing loss that usually starts in one ear and progresses to the other. It takes weeks or months for the condition to develop to affect both ears. Hearing ability often fluctuates.

Many people also experience balance problems and dizziness. Vertigo is another common symptom and this causes a sensation of spinning that can impact balance.

It’s common to feel a fullness or feeling of pressure in the ear with AIED. Many people report tinnitus too, which is ringing in the ear, although it can also present as a roaring or hissing noise. If a systemic autoimmune disease is present, there could be additional symptoms affecting the rest of the body such as aching muscles, fatigue and fever.

How is autoimmune inner ear disease diagnosed?

Since AIED is relatively rare while its key symptoms are very common, it is difficult to diagnose. Often it is initially diagnosed as an ear infection until symptoms persist and spread to the other ear. When patients have already been diagnosed with a systemic autoimmune disease, diagnosing AIED as a secondary condition is easier.

Diagnosis is usually made by assessing the patient’s medical history and performing a physical exam of the ear. Hearing tests can determine the severity of hearing loss. Blood tests can often help to identify inflammatory processes taking place in the body which could point towards an AIED diagnosis.

How is autoimmune ear disease treated?

Sometimes AIED is treated with corticosteroids which work to reduce inflammation. The problem with corticosteroids is that they can cause a wide variety of side effects, and most people can’t take them for more than a few weeks. Sometimes this is enough to get the condition under control. If not, other immunosuppressant medications can be prescribed to reduce the body’s immune response and alleviate the condition.

When AIED is identified and treated early, sometimes it’s possible to reverse hearing loss. However, if the condition has persisted for a long time, hearing loss may be permanent. Where necessary, a hearing aid can be incredibly helpful in improving your hearing. Sometimes a cochlear implant is useful. This is a very small device that is fitted surgically and stimulates nerves in the inner ear to send signals to the brain which interprets them as sound.