Autoimmune Inner Ear Disease (AIED)

When a virus attacks, the immune system defends the body. When the immune system malfunctions, its defense capabilities sometimes mistake the body's own cells for invading viruses or germs and attack them, which is referred to as autoimmunity. The immune system can attack the whole body or just certain systems, 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 cause harm. When the ear is itself attacked, this is known as autoimmune inner ear disease (AIED). The progression of damage and functional loss caused by AIED can be rapid.

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

The most prominent symptom of AIED is bilateral asymmetric progressive or fluctuating sensorineural hearing loss, meaning hearing loss in both ears of different severity that occurs unpredictably or worsens over time. The hearing loss typically occurs over several months but can progress over several years and is often accompanied by tinnitus. The presence of vertigo and other symptoms typically related to vestibular loss depends on the degree of the loss and whether 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; Meniere's disease is sometimes present.

Early diagnosis is important because treatment can be effective in stopping the progression or reversing the damage, but identifying 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.

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 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.