Autoimmune Inner Ear Disease

Autoimmune inner ear disease (AIED) is a syndrome of progressive hearing loss, tinnitus, balance problems, dizziness and vertigo that is caused by antibodies or immune system cells attacking the inner ear.   Frequently, individuals with AIED also have other autoimmune disorders such as ankylosing spondylitis, systemic lupus erythematosus (SLE), Sjogren’s syndrome, Cogan’s disease, ulcerative colitis, Wegener’s granulomatosis, rheumatoid arthritis, scleroderma, or Behcet’s syndrome.

AIED is a rare disease, which accounts for less than 1% of all cases of hearing impairment or dizziness. Approximately 16% of individuals have bilateral Meniere’s disease and 6% of individuals have Meniere’s disease of any variety that may be due to immune dysfunction.

Treatment

Medical treatment, frequently in conjunction with immune system medical specialists (called Rheumatologists) is useful to stop the disease at its source.   A variety of medical therapies are used to improve and stabilize hearing and balance symptoms. Individuals who contract AIED may benefit from hearing aids, BAHA implantation, or in bilateral cases, cochlear implants.