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Anyone out there with Autoimmune inner ear disease (AIED)?
Hi - I have been on an emotional roller coaster for the last year since my diagnosis of AIED. Just looking to see if anybody else out there has been diagnosed and how they’re dealing with it. Thank you.
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I do have AIED, and as your doc predicted for you, I am deaf in both ears. Before you have an anxiety attack, this has been going on for about 30 years. Mostly I want to respond to your comment about hearing aids when you no longer have any residual hearing.
Your situation might be a candidate for cochlear implant(s). I am now deaf in both ears, no residual hearing. However I have cochlear implants for both ears. CIs do not depend on any hearing from your ear. In effect, they bypass the various parts within your ear that create natural hearing.
It's my understanding that insurance companies are much friendlier about CIs than hearing aids (bizarre, I know), perhaps because implantation is a surgical procedure. It's bizarre because CIs can cost in the neighborhood of $100,000, while even very expensive hearing aids are more like $7000. Mine were covered at almost 100%, all I had to pay out-of-pocket was a $350 "facility fee". Find a cochlear implant practice, and ask.
Most CIs can be set up to sync with a hearing aid. CIs have a learning curve, you have to ut in some time on self-directed rehab to get the most out of them. That said, It is nice to be able to have conversations, talk on the phone, etc.
Hi Amber - 1st of all I am shocked that you were on methotrexate for 17 years went off it at your doctor suggestion and lost your hearing. That seems insane to me and that when you went back on it you’re here instead gone I am so sorry. So I have been on prednisone for about nine months. I’m down to about 20 mg right now. I can tell the hearing has fluctuated a little bit but it’s tolerable. I don’t need my hearing aids in right now still get the muffled sound in loud areas like restaurants and you know sound sensitivity Did the methotrexate pills didn’t think it was working went to the injectables that company went out of business went back to the pills. My doctor Musta wrote a very convincing letter because the insurance company is currently approving Humira, which is a biologic every two weeks I take a shot I have one more month before we see if it’s gonna work if not more than likely, as with some of the other people had stated on here they tell you that you could lose your hearing . I wanted to learn Spanish, but I may have to learn sign language instead 😂
I am 57 years old. This started last July woke up in the morning with the fullness in there like you’re feeling I fortunately did luck out and have an amazing audiologist named Annie who was the one that actually diagnosed me with AIED off the record before the ENT did. You don’t live Wisconsin do you? Just thought I’d ask.
So curious, do you have tinnitus in the left ear, the ringing or lightheadedness, or vertigo at all?
I have the Tinnitus both ears mostly the right that’s the worst year that’s an adjustment. I also get a little off kilter so to speak if I get up too fast or I don’t even know why.
Funny story I haven’t been drinking since it started and I’m not a big drinker but I went out with the girls last weekend went ass over tea, kettle skin, both knees hit my cheek on the concrete, cut my hand. It wasn’t pretty I guess it could be worse, right.
And you saw the post regarding cochlear implants. She’s had it for 30 years. I didn’t know it was around 30 years ago but that’s at least something that we can look up to and knowing that people can have a successful, happy life with them if we get to that point.
Let me know how that hearing test went!!
Thank you for sharing!!
Amber, I’m also shocked that the doctor they gave you the shots and wanted to keep you on prednisone for life which is bull. Crap didn’t look at any other medication‘s for you. This is such a rare disease I don’t think anybody really knows.
Bill, thank you for all that you do! This Mayo Connect is an awesome site!!
Autoimmune inner ear disease (AIED) has been defined as a condition of bilateral sensorineural hearing loss (SNHL), caused by an 'uncontrolled' immune system response.
Please share your experience with AIED if it has affected you or a family member.
Hi all,
I just stumbled on this community while searching for an ENT specializing in AIED. I’ll give a brief background of my journey in case it’s helpful to anyone. When my AIED onset in 2015, my GP thought it was a sinus and ear infection. When those meds didn’t work and I started to lose my hearing and had an awful vertigo flare (could barely get out of bed for a few weeks), they thought it was a virus. I was put on a short dose of steroids and all was well until about 9 months later, when I had another flare. Slight bilateral hearing loss but my left ear recovered quickly from prednisone and has been stable in the normal range since. ENT/rheumatologist thought it was Wegner’s, Cogan’s or AIED.
Since then, I’ve been on various doses of prednisone up to 60 mg, coming off of it only for 4 months early this year (am now back on it because of another flare). In the meantime, I’ve tried a number of steroid-sparing medications - Imuran, methotrexate, remicade, rituxan, cellcept, Arava and currently on Kineret. Methotrexate seemed to work better than most of the others but I couldn’t handle the hair loss. Once I flare, which presents for me as increased hearing loss in my right ear and minor vertigo spells (more minor as time has gone on), we assume the meds aren’t working and I increase my dose of prednisone and try a new med.
My ENT once told me that some people might need to stay on a low dose (5 mg or less) long term in addition to whatever med works the best for them.
As a positive for anyone reading this, while prednisone has awful side effects, it can help some people (including me) regain hearing. I’ve read that even steroid-responsive people become less so over time, but since I started back up 4 weeks ago (40 mg for 3 weeks, 30 currently and still tapering), my hearing has been better than it has been in years (per my audiogram today). Another positive, again with the caveat that this is in my experience but I’ve read it can be similar for others, is that my vertigo flares are significantly less intense than they used to be.
As far as prednisone, I can empathize with anyone taking it as I have 7+ years under my belt. I’m not a doctor, but some advice below.
Get a bone density test so you have a baseline and can monitor over time, as it can wreak havoc on your bones; talk to your doctor about how to counter that (eg calcium supplements).
**This could be a potential trigger for someone and is NOT for everyone.** But I found that tracking my calories, lowering my sodium intake and drinking a lot of water helped me to maintain my pre-prednisone weight and avoid moonface during some of the periods I was on a higher dose (though moonface did happen to me). It makes you ravenous, so I just wanted to be mindful of what I was eating.
If you’re experiencing mood swings, irritability, sleep deprivation, etc., which I have, I found giving my friends and family a heads up helped them to be a little more patient with me and helped me to feel a little less guilt/frustration over not being able to control the chemical changes in my body. Along those lines, I’m a big proponent of anxiety/stress-reducing techniques (breathing exercises, working out, meditation, etc.).
If you get heartburn, talk to your doctor about Omeprazole. It has its own negative side effects but might be worthwhile short term.
Some other random things to note:
Second opinions can never hurt, nor can a team of specialists (rheumatologist, ENT, audiologist, neurologist, etc.), especially if they’re in the same network and can easily communicate with each other and can all access each other’s notes. The longer you deal with AIED, the more doctors you might need to see because of side effects of medications. I also did a lot of tests (MRIs, X-rays, CT scans, tons of bloodwork) to rule out extremes like tumors, etc., and to monitor my body. This might look different for someone who’s older, but I know I’ll be dealing with it for (hopefully) many decades.
If multiple medications usually meant to treat AIED aren’t working, talk to your doctor about others that aren’t indicated for AIED.
Overall, hang in there. It’s tough to be in a minority of patients where there’s not a ton of info out there, but you’re not alone! This seems like a great support group.
Hello, for the most part, your situation is very similar to mine. You’ve given great advice. I agree with all of it. I am currently on 20 mg of prednisone tapering down to 15 mg in a few weeks. Still on methotrexate while the Humira kicks in. Thanks for sharing your story.
Hi Amber, just wanted to follow up and see what happen with that hearing test you had and how you’re doing.
They started me on Humira, but of course the left years failed now, which is probably almost gone. At this point they upped my prednisone again to see if I can get it back.
Let me know how you’re doing
Hello I want to check in and see how you’re doing. You gave some great advice and there were a lot of similarities to your situation as mine as of this weekend my left ear is failing so they’re upping the prednisone. I’m still on methotrexate and Humira waiting for the Humira to kick in to see if we can wean off anything else, but the left ears failed worse than it’s ever been. We are increasing the prednisone to see if it’s gonna bring it back. Just curious how you’re doing . Thanks Tina.
Hi Tina,
I’m so sorry that your hearing’s getting worse. Hopefully the increased prednisone will help! And thanks for checking in — I’m stable, as far as I can tell. Slowly tapering off prednisone (now at 20 mg) and will retest my hearing in the near future to check on it. I hope you have good news to come!