Autoimmune Inner Ear Disease

What is autoimmunity?
When a virus attacks, the immune system defends the body. The defense capabilities of a malfunctioning immune system can harm the body by mistaking and attacking the body's own cells as if they were invading viruses or germs. This is called autoimmunity.

In general, autoimmune disorders occur more frequently in women than men and less frequently in children and the elderly.

What is autoimmune inner ear disease?
The immune system can attack the whole body or just certain systems within the body, including the ear. Even if the ear is not being directly attacked, debris created by an autoimmune reaction in one part of the body can be transported to the ear by circulation, and hence cause problems to the ear. When the ear is attacked, the progression of damage and functional loss can be rapid.

Some autoimmune disorders that can affect the ear include Cogan's syndrome, relapsing polychondritis, polyarteritis nodosa, Wegener's granulomatosis, systemic lupus erythematosus, ulcerative colitis, Sjogren's syndrome, and rheumatoid arthritis.

Symptoms
The most prominent symptom of autoimmune inner ear disease is bilateral asymmetric progressive/fluctuating sensorineural hearing loss, typically occuring over several months. Sometimes the progression can be over several years. The presence of vertigo and other symptoms typically related to vestibular loss depends on the degree of the loss and whether the the damage has triggered a problem with fluctuating function (for example, if endolymphatic hydrops developed from the autoimmune reaction). The symptoms of autoimmune problems can be similar, even indistinguishable, from other vestibular disorders; a Meniere's syndrome is sometimes present.

Diagnosis
Diagnosing an autoimmune disorder as the cause of inner ear symptoms can be difficult. Aside from a history of other systemic autoimmune disorders and supportive blood tests, sometimes the diagnosis is made only if hearing improvement occurs in response to drug treatment.

Treatment
An early diagnosis is important because treatment can be effective in stopping the progression or reversing the damage.

The standard treatment for autoimmune reactions is immunosuppressive medication, sometimes delivered as long-term therapy. Such medications have body-wide effects and include steroids, chemotherapy agents, antitransplant rejection drugs, and the newer antitumor, necrosis-factor drugs. Because a rheumatologist has extensive training and experience in these treatments, they are often called on to manage the drug treatment in collaboration with the otolaryngologist or neurotologist.

From VEDA publication F-21, Autoimmunity and the Inner Ear.