Autoimmune Inner Ear Disease

One rare cause of hearing loss is autoimmune inner ear disease, or AIED. In AIED, the patient’s own antibodies or immune response attacks the inner ear, which results in progressive hearing loss and dizziness or vertigo.

Generally, the patient experiences hearing loss along with tinnitus (whistling, ringing, or hissing sounds) in both ears, and attacks of vertigo or dizziness. This takes place over a period of several months, and looks a lot like Meniere’s disease, another cause of hearing loss. One thing that helps distinguish AIED from Meniere’s disease is an abnormal blood test for self-antibodies. In addition AIED may (or may not) be associated with other immune disorders. Most patients who are diagnosed with AIED—65 percent—are women.

Doctors say that only about 1 percent of patients with hearing loss and dizziness have AIED, but the actual number is uncertain. According to some experts, about 16 percent of patients with Meniere’s disease in both ears, and 6 percent of patients with Meniere’s in one ear also may have AIED. In fact, some researchers are starting to suspect that Meniere’s disease may be an autoimmune disorder.

Causes of AIED

Doctors believe that AIED is related to either immune cells or antibodies that attack and damage the inner ear, but the actual cause of the condition is uncertain. There are a number of theories, some of which are related to other auto-immune conditions such as lupus, scleroderma and rheumatoid arthritis. The theory that experts consider the most likely is that antibodies cause accidental damage to the hair cells in the ear in the process of fighting off another disease such as a virus or bacterial infection. Other theories involve allergens or genetic factors.

Diagnosing AIED

Diagnosing AIED can be difficult. Your doctor will take a medical history, do a physical exam, and run blood tests, hearing tests, and vestibular tests. He or she also may order an MRI, and look at your body’s response to immunosuppressive medications.

One other diagnostic tool—and probably the most telling—is the use of steroids. If your doctor gives you steroids and your hearing gradually improves over a period of several weeks, chances are pretty good he’s going to come back with a diagnosis of AIED. The bottom line here, though, is that there is no specific test for AIED, so doctors rely on other evidence to establish that you do have an autoimmune disorder.

Treating AIED

There are several ways of treating AIED. If it’s progressing rapidly, your doctor may try several weeks of steroids. If you respond to that, your doctor probably will switch you to a chemotherapy type of medication, since long term steroid use can result in serious side effects. Other treatments may include plasmapheresis, which is similar to dialysis in that in removes unwanted substances from the blood; however, this means you must be hooked up to the equivalent of a dialysis machine periodically. Some doctors are using anti-TNF (tumor necrosis factor) medications with some success.

If you don’t respond to steroids or other medications, you may need hearing aids or—at the most extreme—cochlear implants.