Autoimmune Inner Ear Disease - Symptoms and Treatment

Illustration of ear
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Autoimmune inner ear disease (AIED) occurs when the body's immune system attacks the structures in the inner ear causing rapid hearing loss and other symptoms. The disease is thought to be very rare but is likely under diagnosed since it has only been recognized as a distinct disorder since 1979 and because it is often misdiagnosed as Meniere's disease.

Why Autoimmune Inner Ear Disease Occurs

It's not always clear why the immune system sometimes goes awry and attacks organs and tissues it shouldn't.

In AIED the immune system may mistake the structures of the inner ear for a virus or other pathogens or it might occur as the result of an allergic response.

The immune system may attack only the inner ear or other parts of the body as well. Autoimmune diseases that can result in damage to the inner ear by the immune system include:

  • Cogan's syndrome
  • relapsing polychondritis
  • polyarteritis nodosa
  • Wegener's granulomatosis
  • systemic lupus erythematosus
  • ulcerative colitis
  • Sjorgen's syndrome
  • rheumatoid arthritis
  • psoriatic arthritis
  • ankylosing spondylitis

According to some medical professionals, if damage to the inner ear occurs as the result of a systemic autoimmune disease,  (such as those listed above), it is not truly considered AIED, which is the term used to describe the condition in which the inner ear seems to be the only part of the body the immune system is attacking. Regardless, the result of the immune system attacking the inner ear is very much the same.

Autoimmune inner ear disease seems to occur more frequently in women than men and most often in individuals between the ages of 20-50 years. However, AIED can occur in anyone.


The hallmark symptom of AIED is rapid hearing loss. It may begin in one ear but eventually involves both ears. Hearing loss occurs over a time period of weeks to months.

Other symptoms include:

  • vertigo
  • disequilibrium
  • tinnitus
  • a feeling that there is pressure in the ear or that the ears are very full

If damage to the inner ear is due to the systemic autoimmune disorder symptoms of AIED may be accompanied by symptoms of that particular illness.

Diagnosing Autoimmune Inner Ear Disease

Diagnosing AIED can be challenging since the symptoms overlap with many other conditions including Meniere's disease. Sometimes these two disorders can be differentiated, however, since bilateral hearing loss rarely occurs in Meniere's disease. Some of the following tests may be helpful in diagnosing AIED:

  • audiometry
  • rotary chair test
  • electrocochleography
  • blood tests including Western Blot analysis for antibodies to inner ear antigen

Other tests may be used to rule out disorders that cause similar symptoms. Sometimes the diagnosis of autoimmune inner ear disease is only confirmed following a positive response to treatment.

Treating Autoimmune Inner Ear Disease

The first line of treatment for autoimmune inner ear disease is corticosteroid medication to suppress the immune system. The medication is usually given orally but can also be given topically (directly into the ear). Putting the medication directly into the inner ear results in fewer side effects than oral medication but usually requires a minor surgical procedure.

Usually, a ventilation tube and possibly a wick is surgically placed in the auditory tube to deliver the medication directly into the inner ear.

Most patients respond well to treatment with corticosteroids but other treatments exist for those who cannot tolerate the side effects of corticosteroids or who do not have a positive response to the treatment. These include the administration of ototoxic drugs such as cyclophosphamide or methotrexate. Other treatments such as plasmapheresis or cochlear implants are reserved for individuals who do not respond to medications.


American Academy of Otolaryngology - Head and Neck Surgery. Fact Sheet: Autoimmune Inner Ear Disease. Accessed: April 26, 2014 from

American Hearing Research Foundation. Autoimmune Inner Ear Disease. Accessed: April 26, 2014 from

Medscape. Autoimmune Disease of the Inner Ear. Accessed: April 26, 2014 from

Vestibular Disorders Association. Autoimmune Inner Ear Disease. Accessed: April 26, 2014 from