From Mary Kugler, R.N.,
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There are many things known to cause hearing loss, among them certain toxic drugs, neurological diseases, cancer, Lyme disease, and infectious diseases. But sometimes the cause is unknown, as in idiopathic progressive bilateral sensorineural hearing loss (IPBSNHL).

• idiopathic = cause unknown
• progressive = gradually gets worse
• bilateral = affects both ears
• sensorineural = damage to the sensory cells and/or nerve fibers of the inner ear
• hearing loss = loss of at least 30 decibels at any frequency

Some researchers believe that some kinds of IPBSNHL are due to an autoimmune disorder, in which the body's own immune system attacks the inner ear. This is called autoimmune inner ear disease. Other researchers believe that there are additional, as yet unknown, factors at work in IPBSNHL.

Treatment
Physicians have found that treating people with autoimmune inner ear disease can stop, and even reverse, some of the hearing loss, especially if treatment is started early. Corticosteroid medications such as prednisone (Deltasone) are often effective. The medicine can be put right into the inner ear under local anesthesia, or through the use of tiny ear tubes put in place surgically. If the steroid doesn't help, cyclophosphamide (Cytoxan) or methotrexate (Rheumatrex) may be given.

There has been a national clinical trial conducted at nine sites to study the effectiveness of these medications in treating autoimmune inner ear disease. (For information, see the ClinicalTrials.gov page.)

Cochlear implants
If medication is not able to save the hearing of a person with autoimmune inner ear disease, an electronic device called a cochlear implant can be used. The device is partially implanted into the hearing organ (cochlea) of the inner ear. A microphone, processor, and transmitter are worn outside the ear. The person can then hear voices and sounds, although the quality is not like natural hearing, and the person requires some training in order to be able to carry on a conversation.

Rush Limbaugh, the talk-show host, lost his hearing to IPBSNHL in October 2001. On December 19 of that year he had cochlear implant surgery, after medications failed to help him.

Cochlear implants do not work for all cases of hearing loss. However, someone like Mr. Limbaugh, who had verbal language skills and only a short period of deafness, usually benefits from an implant.

Information for this article was taken from:
- Broughton, S., & Meyerhoff, W. L. (2001). Inner ear, autoimmune disease. eMedicine, accessed at http://www.emedicine.com/ent/topic496.htm
- Dembner, Alice. "Limbaugh has rare hearing disorder." The Boston Globe, 10/11/01.
- House Ear Institute. "Rush Limbaugh's Cochlear Implant Surgery A Success." Press release, 12/20/01.
- Self Help for Hard of Hearing People (SHHH). Autoimmune Inner Ear Disease.