Anyone out there with Autoimmune inner ear disease (AIED)?

@tinae Hello and welcome to Mayo Clinic Connect! I’m sure we can answer your question, but we’ll need more information. When I looked up AIED, the only thing I found was autoimmune ear disease. Is that it and is it your primary diagnosis?

Hi - yes it is auto immune inner ear disease. Basically I am losing my hearing due to this disease. Originally it was thought to be something else, but once it went bilateral it wax diagnosed. Mostly middle aged women, very rare.

@tinae Yes, it is very rare, maybe 15 out of 100,000! You must have seen all the right doctors in order to get a diagnosis.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6213300/
I’m also giving you the link to our hearing loss group because there are currently no discussions about AIED in this group. You can look through the discussions and find an appropriate one for you. You can also re-ask your question there:
https://connect.mayoclinic.org/group/hearing-loss/
Have the doctors made any suggestions for treatment?

Hi - I was diagnosed with AIED just curious if anyone else is been diagnosed with it and if you’ve had any luck with any of the meds or any functional medicine that might be working for you. This is extremely rare maybe 1 to 15 people and 100,000 mostly middle aged women. Thanks.

How completely remarkable! I joined this forum just moments ago.
I was diagnosed with AIED about 30 years ago. My experience with meds might no longer be relevant, research has since suggested better options (or at least better tested options. Still, here goes: I started taking cortisone and methotrexate. Sorry, I don't remember dosages, but I can remember that my face swelled up to the classic "moon-face" of high-dose cortisone. My life was pretty crummy with these meds. I was susceptible to an amazing array of opportunistic infections, crazy mood swings, sleep disturbance, etc. All in all, I decided that I'd rather be deaf than constantly sick. I have not taken either cortisone or methotrexate for many years now, and have no residual side effects.
I feel a little bad for not offering a more positive report. Your experience might be quite different, if only because the protocols have changed. Also, reactions to medications are highly individual.
Hope this was helpful
Becky

Hi Becky - wow 30 years ago I didn’t even know it was around then. So I am in the same situation as you prednisone for the last nine months moon face can’t sleep the whole thing methotrexate I’m on injections they don’t think it’s working. So the question is without the meds and yes it’s individual. Are you completely deaf do you have cochlear inplants, do you just wear hearing aids, can function, do you have vertigo? Are you dizzy? I like you I’m ready to handle the hearing loss. I just need to make sure I can stay upright. Thanks, Tina

I now have 2 CIs. Got the first one 15 years ago, the second just last year. Hearing aids before that. I guess that I’m now completely deaf without all the gear.
I never had vertigo, but I sure had/have crummy balance. Physical therapy helps enormously. Apparently your body can get balance info from eyes and muscles, not just from the hair cells that AIED wipes out in your ear. I’ll never be a gymnast on a balance beam, but I can muddle along in a mostly upright position. My only gripe about PT is that my insurance thinks it’s a luxury I don’t need.

Thanks for sharing Becky may I ask how old you are or how old you were when you were diagnosed? If I can go 15 years without the first guy I’ll probably be dead, so not really up 57 today but I am more worried about like working in the balance thing I mean like you I probably will decide just to go deaf, but at this point, I’m still on the methotrexate trying to see if I can find a functional medical doctor that might be able to prescribe more supplements and things I keep reading up on it I was hoping research would be better. I didn’t realize it’s been around that long so it’s terrifying to think after 30 years they still have no answers. Thank you for sharing. I really appreciate it.

Hi, Tinae!
I tested positive for 68 kD (kiloDalton)/HSP 70 (Heat Shock Protein) after sudden hearing loss in my Left ear in December. AIED was suspected, and I was put on high-dose Prednisone 60 mg/day, in the morning, and referred to a Neuro-Otologist at Anschutz in Aurora, Colorado. He did an extensive exam, and determined I have Idiopathic Sudden SensoriNeural Hearing Loss (ISSNHL). I’m going to have a new baseline Hearing Test when I get titered off Prednisone, and have a consult/fitting for hearing aids. Everyone is booked WAY out. Can’t get in until end of June! I try not to worry TOO much about going deaf, but it is definitely a big concern. Do I learn sign language, lip reading, or just wait to see if it can be managed somehow? There doesn’t seem to be much guidance available for proactive people. It might be kinda fun to learn new skills, tho’. I’ve read that over-the-counter hearing aids are not appropriate for my type of hearing loss. I need low-range and tinnitus features in hearing aids. Have you learned anything more about your treatment options or prognosis? I’d love to "hear" about it!

Hi @vault and @tinae, I have added this discussion to the Hearing Loss support group as well.

Because autoimmune inner ear disease (AIED) is defined as a condition of bilateral sensorineural hearing loss (SNHL) caused by an ‘uncontrolled’ immune system response, you may also be interested in these related discussions:
- Consultation for sensorineural hearing loss: https://connect.mayoclinic.org/discussion/consultation-for-sensorineural-hearing-loss/
- Can sensorineural hearing loss be prevented?: https://connect.mayoclinic.org/discussion/can-sensorineural-hearing-loss-be-prevented/
- Help: Sudden Sensorineural Hearing Loss (SSHL) - very scary: https://connect.mayoclinic.org/discussion/help-sshl-very-scary/

@vault, as you prepare to get hearing aids, you might find member advice helpful in the Hearing Loss support group https://connect.mayoclinic.org/group/hearing-loss/ Just put "hearing aids" into the search field and you'll find a wealth of discussions.