This rare inner ear disease is principally characterized by progressive bilateral fluctuating hearing loss and occurs when the body's own immune system starts to attack the hearing mechanism of the ear... much like rheumatoid arthritis is when the body's immune system attacks the joints. Just as with any other auto-immune disorder, it is mainly treated with repeated courses of prednisone.
However, if the hearing gets poor enough, the patient may become essentially deaf for which even hearing aids are ineffective which is what happened to Mr. Limbaugh.
To restore his hearing, he underwent surgical placement of a cochlear implant at the House Ear Institute in Los Angeles. It is truly a "bionic" ear in that a computer hears the world around him and directly stimulates the hearing nerve bypassing the cochlea.
Here's a transcript of him describing what it's like.
Source:
Questions on the Host's Hearing. March 18, 2011
Rush Limbaugh's Cochlear Implant Surgery A Success. Audiology Online. 1/3/2002.
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