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

Hey Tiane,

I’m going to send you a much longer detailed response. But a few coles notes.
Funny I am totally 100% a people pleaser! Hahaha And my joke was that this must have happened because I wasn’t hearing some message or not listening to something in my life.

Sooo I was on methotrexate for 17 years for my vasculitis. Oddly enough within about six months of finally coming off methotrexate,
(recommend by my rheumatologist) is when my sudden acute hearing loss happened.
I said let’s try and put me back on it and see if it helps. It did nothing went back on it for about 6 months.
I am also on a biologic (Remicade) for my vasculitis. I did think that the day after my infusion my hearing got a little better. But we debunked that. I had a hearing test the day before my infusion, and the day after, and there was no difference at all..
I’ve had so many hearing tests in the last two years I could be an audiologist myself lol.
When I had exploratory surgery, they scraped the lining of my inner ear, and sent it to pathology. It came back, saying there was a mass amount of inflammatory cells in the tissue. This was another reason they told me it’s a secondary autoimmune disease. My ENT did tell me one day in his office that he doesn’t understand why I haven’t gone deaf in my right ear yet, and that it was going to happen. It just hasn’t caught up.. HA!!! I told him he might want to make his delivery on that a little different.
So for the last year and a half I have been holding my breath hoping and praying I don’t loose my hearing in my right.

I can’t get a hearing aid because there is absolutely nothing to amplify. A hearing aid only works if you have some hearing in that ear. I have nothing. No word recognition, and can’t hear any tones low or high. If I put an ear bud in my ear and crank it with the base all the way up, I can’t even feel it.
We did discuss a crossover system, where they would put on microphone in my left ear and an aid in my right, and when someone was talking on my left, it would send it to the hearing aid in my right. However, they are thousands and thousands of dollars and was told that it would affect the amount of hearing I have in my right ear, like muffle it. And if I go deaf in my right ear, it would also be completely useless, and I would be out thousands of dollars.. so hearing aid are not going to help me unfortunately. I’d give ANYTHING to have an aid!
I mean I was over 3 months before a doc even looked in my ear. I saw 3 different ENTs. And they all said if you would have come in the first 3-5 days and we got prednisone in there it most likely would have helped.
I’d be so curious to see now if I started taking 60 mg of prednisone a day and had my ear injected, if it would bring it back to any degree?

I’m so sorry you’re going through this too. How old are you? Such a crazy thing. I’m just concerned I’m going to also loose my hearing 100% on my right….. fingers crossed I can have a hearing test tomorrow and get some answers about this fullness that started today on the right.

Lol I said this was going to be short. Sorry!
I’d love to follow your journey and see how things go for you. And what works and doesn’t.
Definitely keep me posted.
Good night 🙂

Hey Tiane,

I’m going to send you a much longer detailed response. But a few coles notes.
Funny I am totally 100% a people pleaser! Hahaha And my joke was that this must have happened because I wasn’t hearing some message or not listening to something in my life.

Sooo I was on methotrexate for 17 years for my vasculitis. Oddly enough within about six months of finally coming off methotrexate,
(recommend by my rheumatologist) is when my sudden acute hearing loss happened.
I said let’s try and put me back on it and see if it helps. It did nothing went back on it for about 6 months.
I am also on a biologic (Remicade) for my vasculitis. I did think that the day after my infusion my hearing got a little better. But we debunked that. I had a hearing test the day before my infusion, and the day after, and there was no difference at all..
I’ve had so many hearing tests in the last two years I could be an audiologist myself lol.
When I had exploratory surgery, they scraped the lining of my inner ear, and sent it to pathology. It came back, saying there was a mass amount of inflammatory cells in the tissue. This was another reason they told me it’s a secondary autoimmune disease. My ENT did tell me one day in his office that he doesn’t understand why I haven’t gone deaf in my right ear yet, and that it was going to happen. It just hasn’t caught up.. HA!!! I told him he might want to make his delivery on that a little different.
So for the last year and a half I have been holding my breath hoping and praying I don’t loose my hearing in my right.

I can’t get a hearing aid because there is absolutely nothing to amplify. A hearing aid only works if you have some hearing in that ear. I have nothing. No word recognition, and can’t hear any tones low or high. If I put an ear bud in my ear and crank it with the base all the way up, I can’t even feel it.
We did discuss a crossover system, where they would put on microphone in my left ear and an aid in my right, and when someone was talking on my left, it would send it to the hearing aid in my right. However, they are thousands and thousands of dollars and was told that it would affect the amount of hearing I have in my right ear, like muffle it. And if I go deaf in my right ear, it would also be completely useless, and I would be out thousands of dollars.. so hearing aid are not going to help me unfortunately. I’d give ANYTHING to have an aid!
I mean I was over 3 months before a doc even looked in my ear. I saw 3 different ENTs. And they all said if you would have come in the first 3-5 days and we got prednisone in there it most likely would have helped.
I’d be so curious to see now if I started taking 60 mg of prednisone a day and had my ear injected, if it would bring it back to any degree?

I’m so sorry you’re going through this too. How old are you? Such a crazy thing. I’m just concerned I’m going to also loose my hearing 100% on my right….. fingers crossed I can have a hearing test tomorrow and get some answers about this fullness that started today on the right.

Lol I said this was going to be short. Sorry!
I’d love to follow your journey and see how things go for you. And what works and doesn’t.
Definitely keep me posted.
Good night 🙂

Hi, I have totally deftness in my left ear. It was sudden acute. I had no idea this was an emergency. It started happening during Covid and nobody would look in my ear. Finally after three months I had a hearing test and two days later was sent to an ENT where they thought that I had a Glomus tympanicum vascular tumour in my inner left ear. while waiting for exploratory surgery to see if there was a tumour, the ENT injected my ear with prednisone several times and had me on 60 mg of prednisone a day, which brought my hearing up to 90% !!! One EnT wanted to keep me on prednisone for life. I didn’t like this option for obvious long term effects on the rest of my body. As soon as I stopped orally taking prednisone and having my ear injected, my hearing went completely. This took only a month. I am currently 100% death in my left. They told me this was a secondary auto immune disorder. I was diagnosed with Takayasu’s vasculitis since 2004.
I started noticing the hearing loss in September 2021. I was 39 years old. They have since told me to keep a close eye on my right, very precious ear. Oddly enough as of tonight I feel like I’m having some hearing loss and hoping to go for a hearing test tomorrow on my right side..

Tinae,
I can't contribute to your situation BUT I am hopeful you will get restoration and resolution. You will keep us posted I hope. I send my best to you. There are great people in this group to keep the dialogue going with.

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.

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.

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.

Hey Tiane,

I’m going to send you a much longer detailed response. But a few coles notes.
Funny I am totally 100% a people pleaser! Hahaha And my joke was that this must have happened because I wasn’t hearing some message or not listening to something in my life.

Sooo I was on methotrexate for 17 years for my vasculitis. Oddly enough within about six months of finally coming off methotrexate,
(recommend by my rheumatologist) is when my sudden acute hearing loss happened.
I said let’s try and put me back on it and see if it helps. It did nothing went back on it for about 6 months.
I am also on a biologic (Remicade) for my vasculitis. I did think that the day after my infusion my hearing got a little better. But we debunked that. I had a hearing test the day before my infusion, and the day after, and there was no difference at all..
I’ve had so many hearing tests in the last two years I could be an audiologist myself lol.
When I had exploratory surgery, they scraped the lining of my inner ear, and sent it to pathology. It came back, saying there was a mass amount of inflammatory cells in the tissue. This was another reason they told me it’s a secondary autoimmune disease. My ENT did tell me one day in his office that he doesn’t understand why I haven’t gone deaf in my right ear yet, and that it was going to happen. It just hasn’t caught up.. HA!!! I told him he might want to make his delivery on that a little different.
So for the last year and a half I have been holding my breath hoping and praying I don’t loose my hearing in my right.

I can’t get a hearing aid because there is absolutely nothing to amplify. A hearing aid only works if you have some hearing in that ear. I have nothing. No word recognition, and can’t hear any tones low or high. If I put an ear bud in my ear and crank it with the base all the way up, I can’t even feel it.
We did discuss a crossover system, where they would put on microphone in my left ear and an aid in my right, and when someone was talking on my left, it would send it to the hearing aid in my right. However, they are thousands and thousands of dollars and was told that it would affect the amount of hearing I have in my right ear, like muffle it. And if I go deaf in my right ear, it would also be completely useless, and I would be out thousands of dollars.. so hearing aid are not going to help me unfortunately. I’d give ANYTHING to have an aid!
I mean I was over 3 months before a doc even looked in my ear. I saw 3 different ENTs. And they all said if you would have come in the first 3-5 days and we got prednisone in there it most likely would have helped.
I’d be so curious to see now if I started taking 60 mg of prednisone a day and had my ear injected, if it would bring it back to any degree?

I’m so sorry you’re going through this too. How old are you? Such a crazy thing. I’m just concerned I’m going to also loose my hearing 100% on my right….. fingers crossed I can have a hearing test tomorrow and get some answers about this fullness that started today on the right.

Lol I said this was going to be short. Sorry!
I’d love to follow your journey and see how things go for you. And what works and doesn’t.
Definitely keep me posted.
Good night 🙂

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 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.