Autoimmune inner ear disease (AIED) is a rare disorder that accounts for less than 1% of all hearing impairment or dizziness cases. AIED involves progressive, two-sided hearing loss and/or dizziness believed to be caused by one’s own antibodies or immune cells attacking the inner ear’s hearing and balance mechanisms. AIED hearing loss can be accompanied by tinnitus and imbalance.

Diagnosing AIED can be challenging but is typically based on history, physical examination, blood tests, audiometric testing, and perhaps, most importantly, the responsiveness of the hearing loss to immunosuppressive therapies, such as steroids or methotrexate. Another common diagnostic approach involves looking for evidence of other autoimmune conditions such as thyroid disease, rheumatoid arthritis, or systemic lupus erythematous, to name a few.

There are several protocols for treating AIED. In classic cases involving subacute, progressive, bilateral hearing loss, a four-week trial of steroids is initiated. With hearing improvement, the steroid use is slowly tapered. If hearing stabilizes, steroid treatment can be stopped altogether. If hearing deteriorates when treatment is stopped, continued therapy may involve low dose steroids or other immunosuppressant medications including Methotrexate, Cytoxan, Enbrel, Remicade, and Humira.

In some cases, the hearing loss does not respond to steroid treatment. The loss may also become so profound that hearing aids are no longer beneficial. In such cases, cochlear implants can be very effective in restoring hearing.

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