Limbaugh Case Spotlights Rare Deafness Condition
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
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
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.