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Autoimmune Inner Ear Disease Discussion
when the immune system causes a hearing loss

Updated March 19, 2009

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I had an MRI done to eliminate a tumor. I recovered hearing for a week or two with the Prednisone. Then on Nov. 10, 1998, I collapsed in the airport parking lot on a business trip. I had severe vertigo (I never even knew what vertigo was before that!) and was taken to the hospital. In the few days prior, I was lost my hearing again in the right ear. That morning, the hearing in my left ear started to go as well, and the tinnitus was very loud in both ears. I was getting dizzy, losing my sense of balance, then BAM! In the weeks that followed, I never regained my sense of balance. In addition to becoming totally deaf in one ear and almost completely in the other, I was on a cane and couldn't drive for 3 months. I never regained hearing in my right ear to this day. Shortly afterward I was diagnosed with AIED (due to the positive response to steroids and the identical pattern in both ears 5 weeks apart. The Western Blot assay is sometimes used to confirm but we didn't in my case as they were pretty sure!) Since the same thing happened in my left ear five weeks later as happened in my right ear, it was assumed something (my immune system) was attacking that inner ear tissue. It's too long for a virus or something else. Most likely, they figure I caught a common virus, it went into my ear, and essentially my immune system over-reacted and started attacking the healthy tissue as well as the virus. After the virus was gone, my body to this day is still trying to destroy the inner ear tissue. By mid-December, I was diagnosed as having 'severe bilateral vestibulopathy' and 'oscillopsia'. All it means is the inner ear on both sides has been significantly destroyed, and that particular tissue does not repair itself. Oscillopsia is the condition where your eyes don't focus right - your vision is fine, but your eyes move when your head moves. It makes balance very difficult. The inner ear controls the sensations from hearing, sight, movement and combines them into the sense of balance. When it's shot and things don't get coordinated like they should, it's rough! But with therapy you can overcome - compensating in other ways. After a year, you probably wouldn't know I had a balance condition. I'm 39, fit, active... I was climbing a mountain a week before this! My wife and I compete on horses, I mountain bike and backpack... I've regained my ability to do all these since then.

Anyway, the battle has always been focused on salvaging the scrap of hearing I have left in my left ear since the right one is gone. I function OK with a digital programmable hearing aid in the one ear for now. I took a series of Decadron (also a steroid) shots (called perfusion therapy I think) through the ear drum into the inner ear - something like 18 times (I lost count after while). Successfully got some hearing back in my left ear each time too, for awhile anyway - a couple months. I have been on Prednisone almost non-stop over a year. We've tried to taper off (I've been on as much as 60mg/day) but whenever we dip below 15-20mg/day, I start losing the hearing again, so they bump it back up. I've been on chemo drugs as well, hoping they could be used to suppress the immune system in lieu of the Prednisone which has bad long-term side-effects. I was on Cytoxan (chemo) daily for a few months earlier this year but was not tolerating it well (fatigue, hair loss, the usual crap). I have been on a milder form - Methotrexate - for 5 months now (weekly dosage) and tolerate it very well. Unfortunately it still hasn't allowed me to get off Prednisone so either it's not working or I'm not yet on a high enough dose (I'm on 20 mg/week - 25 mg is about the high end a body can take.)

The specialists presume we're delaying the inevitable - total deafness. I am an experiment for them (by my choice) of sorts. I will get a cochlear implant in my bad ear after my "good" drops off completely. I'd guess this coming year I'll be totally deaf at the current rate of degradation. They keep boosting the power on the hearing aid, and I keep cranking it up to the max just to hear a little ... we can only play that game so long. And I want to get off the drugs too. Let nature take its course. I guess I want to know that I 'tried everything' first, though ..."
—NICKSTANGER
"I've had hearing problems for a long time...maybe longer than I realize. But it's been a minor annoyance. In the last 6 months, however, my hearing has gone downhill rapidly. I can no longer understand speech if I can't see the speaker's face, and then not if there's background noise. So, two visits to an ENT and audi later, the doctor's current hypothesis is Something Wrong in the Inner Ear. His theory is autoimmune disorder. Treatment is steroids or a chemotherapy drug that has the added benefit of helping with this. To me, the side effects of either drug (I'm hypersensitive to steroids)are worse than going totally deaf!"
—KATHI

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Autoimmune Inner Ear Disease Discussion when the immune system causes a hearing loss

Autoimmune Inner Ear Disease Discussion
when the immune system causes a hearing loss