AIED (Autoimmune Inner Ear Disease) has made national news due to the famous radio talk show host Rush Limbaugh’s diagnosis and treatment of the disease.
AIED was first described in 1979. The most common sign and symptom is a significant sensorineural hearing loss that occurs over a period of weeks to months. It usually affects both ears. Some patients also have dizziness or true vertigo.
The diagnosis of AIED is made by the clinical signs and symptoms of a rapidly progressive sensorineural hearing loss. In any person with presumed AIED, it is necessary to rule out other possible causes of progressive sensorineural hearing loss, such as an acoustic neuroma, neurosyphilis, and Meniere’s disease. About 20% of the patients have other autoimmune diseases, such as Lupus or rheumatoid arthritis.
The mainstay of treatment is a high dose, long-term steroids that may go on for 4 to 5 months. About 6% of patients with AIED progress to the point of profound hearing loss. At that point, the only option for hearing is the cochlear implant.