Limbaugh Case Spotlights Rare Deafness Condition

Medically Reviewed by Charlotte E. Grayson Mathis, MD
From the WebMD Archives

Doctors treating Rush Limbaugh recently revealed that the famed conservative talk-show host is suffering from rapid-onset hearing loss as a result of a rare condition called autoimmune inner ear disease (AIED). His case is focusing attention on an extremely rare disease that, at best, can be held at bay by current medications but can't be cured.

Limbaugh announced earlier in October that for all practical purposes, he is deaf.

Doctors at The House Ear Clinic/House Ear Institute in Los Angeles, where Limbaugh is being treated, said that they based their diagnosis on his medical history and hearing tests. While he doesn't have other symptoms of AIED (such as fluctuating degrees of hearing, dizziness, ear fullness, or sudden hearing loss), he has suffered rapidly progressive hearing loss since May.

Autoimmune Inner Ear Disease (AIED) causes hearing loss or dizziness. The hearing loss usually affects both ears, often accompanied by a ringing, hissing, or roaring sound in the ear. AIED is rare, occurring in less than 1% of all cases involving hearing loss and dizziness. Doctors believe it is caused by the body's immune system attacking the inner ear and damaging the hearing nerve. The immune system mistakenly identifies normal tissue as a harmful virus or a bacterium and attacks by releasing inflammatory chemicals. These chemicals damage the tissue, resulting in the onset of deafness. While researchers do not know why this process occurs, it has been noted that many patients have other autoimmune diseases such as rheumatoid arthritis, lupus, or scleroderma.

Prednisone, a steroid drug, is the best current treatment of AIED. The drug suppresses the immune system, relieving symptoms in many patients. However, it must be taken in high doses for long periods of time for it to be effective. Possible side effects of prednisone include weight gain, mood changes, facial hair, acne, premature osteoporosis, and cataracts.

A multi-institutional, clinical study is under way to measure the benefits and risks of treating AIED with a combination of prednisone and methotrexate, another immunosuppressive drug. If methotrexate works, it would provide a less-toxic treatment than prednisone, with fewer side effects.

Limbaugh is being treated with this experimental combination, along with an anti-inflammatory drug called Enbrel. If medications do not halt or reverse the AIED damage, he may need surgery and a device, such as a cochlear implant, to help him to hear.