Autoimmune Inner Ear Disease: Symptoms, Causes, and Treatments

Reviewed by Dr. Porter on March 10, 2021

Autoimmune inner ear disease is a rare condition that causes loss of hearing over just a few weeks or months, often with tinnitus or balance disorders. Scientists believe it occurs when the immune system attacks structures in the inner ear as if they were an invading microorganism or a foreign body.

Autoimmune inner ear disease is an uncommon problem, causing less than 1% of all cases of hearing loss. It is usually a devastating side effect of surviving an infection or of having some other autoimmune disease. But if you have this condition, it can place severe limitations on your life. Let’s take a closer look at what this rare disabling condition really is.

What is Autoimmune Inner Ear Disease?

The patient’s that audiologists see who have autoimmune inner ear disease typically have loss of hearing over a period of up to 90 days with ringing in the ears (tinnitus) or, sometimes, vertigo and other balance disorders. The technical definition of autoimmune inner ear disease is a (1) condition causing hearing loss in both ears (2) caused by an uncontrolled immune response.

This condition can occur during recovery from a bacterial infection, or as a complication of lupus, ulcerative colitis, or rheumatoid arthritis. It also occurs in people who have less common autoimmune disorders such as Sjogren’s syndrome, scleroderma, or dry eye syndrome.

In autoimmune inner ear disease, the immune system usually attacks one ear and then the other. Loss of hearing may be worse in one than the other.

If you develop this condition, you need to start treatment right away. The reason you need treatment for this condition quickly is that it can take as long as 90 days or as little as three days to cause permanent hearing loss. After that time, it’s impossible to stop hearing loss. It’s only possible to treat it.

Because this condition is relatively rare, causing less than 1% of all cases of nerve-related hearing loss, most commonly adult women who have not reached menopause, it’s often mistaken for an infection. That’s why it is important to be aware of the symptoms.

inner ear disease

Symptoms of Autoimmune Inner Ear Disease

The telltale sign of this condition your audiologist will be looking for is the speed at which your symptoms have developed. This doesn’t develop as quickly as other problems due to trauma. If your hearing is affected by a blast or by certain kinds of medication, it will be almost immediate.

On the other hand, this condition doesn’t come on slowly like age-related hearing loss, or presbycusis. If you have this condition your symptoms take at least a few days to develop, and probably take several weeks or even several months to become a problem for you.

Another signal that you have autoimmune inner ear disease is the presence of another autoimmune condition such as lupus, rheumatoid arthritis, ankylosing spondylitis, Sjogren’s syndrome, psoriasis, inflammatory bowel syndrome, polyarteritis nodosa, Guillain-Barre syndrome, or type 1 diabetes. Having one of these conditions isn’t proof that you have an autoimmune hearing and/or balance disorder, but all of these conditions are strong indications that further investigation is necessary.

There is one other important factor that your audiologist considers in diagnosing this condition: If you have autoimmune inner ear disease, it will respond to steroids.
This means that your audiologist and a medical specialist prescribing steroids will be working together to get you swift treatment and accurate diagnosis. Steroids block the activity of your immune system. If your hearing loss slows down once you are put on steroids, then it is likely that an overactive immune system is causing your hearing loss.

Those are the symptoms of this condition that your audiologist will be looking for. But what symptoms would you be experiencing?

Your audiologist will gather enough information about your medical history so that the audiologist and your medical doctor can rule out other conditions. They will want to make sure that the real problem isn’t something on this list.

  • If you start looking for information on the Internet, you will find references to autoimmune conditions in the ears caused by syphilis. Your audiologist won’t assume you have syphilis, and your doctor probably won’t test for it. It can cause these symptoms, but it is almost never the problem.
  • Your audiologist will consider the possibility of “sympathetic inner ear disease.” Sometimes if you have surgery in one, you can develop deafness in the other. Again, this is an even rarer condition.
  • Allergies, particularly food allergies, can affect the inner ear.
  • Ankylosing spondylitis is a progressive bone disease that takes away flexibility across the body. It could affect the tiny bones in your ears.
  • And sometimes symptoms of this condition overlap those of a tumor called an acoustic neuroma.

There are about a dozen other conditions that present the same symptoms as this condition, but they are very, very rare. Your audiologist and your doctors will work together to determine the precise cause of your condition.

Now let’s consider why people develop autoimmune inner ear disease.

inner ear inflammation

Causes of Autoimmune Inner Ear Disease

The truth is, audiologists and medical researchers don’t know what causes this condition, but there are several theories that make sense of the way patients experience it.

One theory of the cause of this condition is bystander damage. The immune system is known to release compounds called cytokines to fight infection. These compounds get into general circulation and some of the attack cells in the inner ear. Because the immune system releases cytokines in pulses, this would explain why symptoms of this condition are sometimes better and sometimes worse.

Another theory of the cause of this condition is intolerance. Most of the time the immune system isn’t really aware that the inner ear is even there. It is busy patrolling other parts of the body for infection and leaves the inner ear alone. When there is a severe infection, however, it can attack the unfamiliar cells of the inner ear as if they were an infection.

There are some indications that this condition is caused by cross-reactions to infection. Sometimes a protein on the surface of a cell in the inner ear can have the same contours as the protein on the surface of a bacterium. The immune system learns how to recognize the bacterium by a contour that happens to be the same as the contour on the cell in the inner ear.

There may be genetic factors that cause this condition, but scientists don’t know whether the genetic factors found in small populations of test subjects cause the disease in other groups. And there is always an influence of noise, exposure to loud sounds throughout life, toxic exposure, medications, and general heath.

Treatments for Autoimmune Inner Ear Disease

The most important thing you need to know about treatments for autoimmune inner ear disease is this:

It’s only possible to stop this condition if treatment is started just days after the onset of symptoms.

There is a brief window for treatments of the underlying autoimmune disease that is causing the hearing loss with medications that reduce the activity of the immune system. Usually these would be steroid drugs prescribed by a doctor specializing in this condition, although there are a few alternative medications that don’t have the side effects that steroids do.

Treatment needs to start before your doctor can be sure that your condition is really autoimmune inner ear disease. Your response to treatment will confirm the potential diagnosis.

Once the damage of the autoimmune process is done, then you need your audiologist to help you with hearing aids or a referral for a cochlear implant. That’s why it is always best to seek treatment right away. Harbor Audiology can help.

Harbor Audiology serves Sequim, Silverdale, Tacoma, Gig Harbor, Port Angeles, and Federal Way. We will help you get the most from insurance and VA benefits. Harbor Audiology has the experts you need to determine the precise cause of your ear pain so you can get the relief you need. Request your appointment online today!

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