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Autoimmune Ear Disease

1. What is autoimmune inner ear disease?

When an invader, such as a virus, attacks the body, the immune system controls the response. This is a necessary and needed function for us to live safely. If the immune system is malfunctioning, the body can mistakingly attacking its own cells as if they were invading viruses or bacteria. 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. A variant of the disorder is bilateral attacks including hearing loss and tinnitus that resemble Meniere’s disease and includes abnormal blood tests for antibodies.

Frequently, individuals with AIED also have other autoimmune disorders such as ankylosing spondylitis, systemic lupus erthematosus (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.

2. What is the treatment for autoimmune inner ear disease?

Individuals who contract AIED may benefit from hearing aids, BAHA implantation, or in bilateral cases, cochlear implants. A specialized interdisciplinary team of professionals is necessary to treat all of the issues associated with AIED. This group of individuals should include an Otologist or Otolaryngologist, and an audiologist, and possibly a vestibular rehabilitation expert.

Medical treatment, frequently in conjunction with immune system medical specialists (called Rheumatologists) is useful to stop the disease at its source. Many new medicines and a better understanding of the immune system have helped in the medical treatment of these disorders in the recent past.

The California Ear Institute is a leading global center for otological, otolaryngological and audiological treatment. Dr. Joseph Roberson, a board certified neurotologista, has successfully treated thousands of patients with hearing and balance related disorders.

3. What are the consequences of avoiding treatment?


Patients must seek medical treatment for any change in hearing or balance regardless of whether or not AIED is suspected to be the source of the hearing loss. Evaluation for beneficial drug treatment or surgery cannot be established without a comprehensive medical examination and associated testing. Avoiding medical evaluation for AIED or any other hearing or balance impairment related condition is not recommended, and can lead to permanent hearing loss and/or balance disturbance.



Additional Notes

Patients searching the internet for information about AIED or any other medical issue should know when reading about individual case histories, that generally it is the patients with the most severe symptoms who either post their own experiences or are included in medical review journals. As a result, it is easy to become overly alarmed and assume the worst. Patients should keep this in mind as they explore the available resources, and look for sources that are certified by HON or other reputable health reviewing organizations. As with all medical conditions, prompt treatment by experienced medical personnel give the best chance for a positive outcome.


If you suspect that you have AIED, or you have noticed a change in your hearing, consult a CEI otologist. The outlook for treatment is excellent, once the diagnosis has been made and appropriate treatment is initiated.

Click here to make an appointment with the California Ear Institute to consult with one of our board certified otologists regarding your ear-related condition.

California Ear Institute