Autoimmune Inner Ear Disease (AIED)

[Juan]

I have been reading a bit about the AIED and I am wondering if it could be related to hyperacusis or tinnitus when there is progressive hearing loss.

There is a link here:

http://american-hearing.org/disorders/autoimmune-inner-ear-disease-aied/

I was wondering if someone in the forum may have personal experience about the AIED and if they found any relation to hyperacusis. Apparently AIED is pretty much diagnosed by a blood test, together with audiogram, OAEs, and a test called "ECOG (electrocochleography)".

I am curious because in the last months I have noticed a gradual deterioration of my hearing, this is, hearing loss progressing faster than it is normal, and only after a couple of isolated episodes of accidental loud noise exposure. I have had hyperacusis for many years, but this new and sudden drop in my hearing is quite a new phenomenon to me, and I am trying to find out if it can be caused by some other factor on top of just noise exposure.

 

[PaulBe]

I've not seen auto-immunity as a cause referenced much at all here. Its a very hard one to prove, to the point where you would have to do virtually all your own research and present it to a medico just to get to the right kind of testing/diagnostic process (and I don't really know what they are).

 

[Juan]

I've not seen auto-immunity as a cause referenced much at all here. Its a very hard one to prove, to the point where you would have to do virtually all your own research and present it to a medico just to get to the right kind of testing/diagnostic process (and I don't really know what they are).

Maybe doctors are so reluctant to dedicate time to hyperacusis or tinnitus because as of today there are very few options for treatment, and none of them very effective and with quite individual results in each case. I read that even if AIED was the diagnosis, the treatment is basically corticoids like prednisone.. and I had prednisone in the past without noticing any significant improvement.

 

[PaulBe]

I guess though that it would be dose and duration related as most of the time, so far as I know, steroids are used only as an acute phase treatment in T & H onset (if used at all). Auto-immunity is a whole different idea, though not one I'd dismiss entirely. Its not impossible, but it is hard to demonstrate, especially in an area of medicine that offers so little support even to existing models.

 

[Ring Ding]

I contracted an autoimmune disease via a vaccination before my new hearing problems started ( I had mild T but it was not much of a bother ever ) and I would say it's certainly related. It had an effect on every single part of my body so why not my hearing too. My perspective is a little different on this now as I have tried to cure my ailments. Many autoimmune sufferers have hearing sensitivity and tinnitus. I belong to a group of people doing chelation therapy on facebook and it comes up quite frequently.

 

[Juan]

I contracted an autoimmune disease via a vaccination before my new hearing problems started ( I had mild T but it was not much of a bother ever ) and I would say it's certainly related. It had an effect on every single part of my body so why not my hearing too. My perspective is a little different on this now as I have tried to cure my ailments. Many autoimmune sufferers have hearing sensitivity and tinnitus. I belong to a group of people doing chelation therapy on facebook and it comes up quite frequently.

Is there specific medication for an autoinmune disease?

 

[bill 112]

Stemcells are known to be very effective against autoimmune disorders,whether you want to go down that path or not is your decision of course but it's something I would consider.

How bad is your hearing loss,in what way is it affecting you?(Can't hear tv etc.)

 

[PaulBe]

Is there specific medication for an autoinmune disease?

Generally they use steroids long term, which raises whole new sets of issues.

 

[Juan]

Stemcells are known to be very effective against autoimmune disorders,whether you want to go down that path or not is your decision of course but it's something I would consider.

How bad is your hearing loss,in what way is it affecting you?(Can't hear tv etc.)

I dont know much about stemcells; would have to research that, but I am not very adventorous as far as treatments are concerned. I would rather try medication or some way of adjusting my life, or supplements, than surgery.

My hearing loss, last time I checked was like +10 dbs for all frequencies until 6.000 and like +25-30 at 6.000 and around +55-60 at 8.000. It went down fast because last year it was only +35-40 at 8.000 and lately I am feeling worse, headaches, pressure behind my eyes and deep inside my ears. It is getting worse, new and more lasting symptoms; H is still shocking after many years.

 

[Juan]

Generally they use steroids long term, which raises whole new sets of issues.

Prednisone never did much to me, but most ENTs recommended using prednisone for like 10-15 days, starting with a high dose and then tapering.. some doctors said that the high dose of the first days should be maintained for a longer time, and this would mean being on prednisone for around 30-45 days.

Have you used prednisone for longer than a month?

 

[Mario martz]

This is what might be happening to me.

i have had constant tinnitus and i guess ive lost some decibel in the lowest range.
but thank god i havent had vertigo or any of that stuff.
and ive been experience morning pain, and weird vision, but these symptoms doesnt fit the MS criteria.
mm interesting

 

[PaulBe]

Have you used prednisone for longer than a month?

No, but I've looked after long term users with diseases like SLE (Lupus) for example (which I might add often involves tinnitus). If you want to know what medical steroids do long term, search the words "Cushings syndrome".

 

[Ring Ding]

Is there specific medication for an autoinmune disease?

Not that I know of unless you mean Cymbalta and all that junk. I was given that by a Neurologist but I refused to take it. The side effects seemed too scary. Allopathic medicine has no idea what the cause or cure is for autoimmune conditions. The reasons are many and in the end the answer is pretty sad. Probably in most cases it's heavy metal toxicity. The CDC and FDA have been fudging vaccine data for decades to hide the link between vaccines and Autism. The ADA is also complicit in this as well by hiding the fact that dental amalgam fillings are made of 50% mercury and the fillings off gas mercury into your organs and brain as long as you have them.

I began treating my condition on the principle that it was heavy metal poisoning a year ago. Six months into the process I was at a concert when the hearing issues began. Don't know if my hearing is just messed up or if it's related to the condition. I'm assuming it's at least partly related to that. many others with these kinds of conditions have the same problems. Could be deep stores of mercury in the brain tissue. I'm doing better but not nearly cured as of yet. I can pretty much deal with the pain and fatigue ok but the hearing issues are just a nightmare. The stinging feeling and the fullness and the goddam ringing are very powerful. Been 9 months with no change in the sting or the ring but the H is starting to fade. it's not gone yet either but enough so that I can function semi normally.

 

[Juan]

Not that I know of unless you mean Cymbalta and all that junk. I was given that by a Neurologist but I refused to take it. The side effects seemed too scary. Allopathic medicine has no idea what the cause or cure is for autoimmune conditions. The reasons are many and in the end the answer is pretty sad. Probably in most cases it's heavy metal toxicity. The CDC and FDA have been fudging vaccine data for decades to hide the link between vaccines and Autism. The ADA is also complicit in this as well by hiding the fact that dental amalgam fillings are made of 50% mercury and the fillings off gas mercury into your organs and brain as long as you have them.

I began treating my condition on the principle that it was heavy metal poisoning a year ago. Six months into the process I was at a concert when the hearing issues began. Don't know if my hearing is just messed up or if it's related to the condition. I'm assuming it's at least partly related to that. many others with these kinds of conditions have the same problems. Could be deep stores of mercury in the brain tissue. I'm doing better but not nearly cured as of yet. I can pretty much deal with the pain and fatigue ok but the hearing issues are just a nightmare. The stinging feeling and the fullness and the goddam ringing are very powerful. Been 9 months with no change in the sting or the ring but the H is starting to fade. it's not gone yet either but enough so that I can function semi normally.

Hearing issues are very complicated. Most probably, doctors do not even know why some people improve from their hearing issues and others dont, or why some people are more prone to hearing damage than others.

In my case I was always a very healthy person until H started. Actually, I seldom went to doctors at all, or have vaccines, not even those they deliver every year for the flu or stuff like that. All my life I tried to stay as far from any kind of medication or drugs as possible. When H started I tried a couple of things, but none of them really worked.

My H symptoms also evolved over time. I have been like 2 months with pressure; it had been a long long time since such a long episode of H hit me, but this time I have lost hearing too. Actually, I can feel very intense pressure in my ears (and sometimes behind one of my eyes) if I try to move my face in certain ways, like frowning hard, or smiling broadly, so there must be something muscle-related there, but doctors do not seem to find anything unusual that could explain H symptoms and this latest quick loss of hearing. I was wondering if this could have something to do with the facial nerve, or some nerve issue..

 

[Frédéric]

Autoimmunity and Otolaryngology Diseases

 

[Juan]

Autoimmunity and Otolaryngology Diseases

Thanks! It is an interesting read. However, most autoimmune diseases have a long series of symptoms, some of which are pretty obvious on the skin, nose etc and it is hard all the collection of symptoms and markers fully match a case that starts just with tinnitus, hyperacusis or hearing issues.

Taking Granulomatosis with polyangiitis (GPA), formerly known as Wegener's Granulomatosis (WG), as an example, at Wikipedia, the sensorineural hearing loss cannot be explained, the cause and origin of the hearing loss is unknown, and the treatment is corticoids and other drugs. WG also seems to have very different degrees of severity..

 

[Frédéric]

Autoimmune inner ear disease (AIED): A diagnostic challenge

 

[Juan]

Autoimmune inner ear disease (AIED): A diagnostic challenge

It basically explains nothing, like most medical literature on hyperacusis or rare hearing disorders over the last 30 years.

The only interesting idea is that corticoids do not really work. 14% success rate after the use of corticoids seems realistic, understanding "sucess rate" by patients showing some improvement (even partial improvement) after corticoid use.

Pure tone audiometric tests are useless, since they measure whether the patients hear or does not hear a tone, a little sound. The pure tone audiometric test cannot measure whether the patient hears the correct pitch for a tone (this means that a patient may hear 8khz at 30dbs but is hearing it with a sound pitch for 4khz, this is, hears the sound lower than it is) or the ability to understand language.

The ability to understand language has to be measured with a verbal discrimination hearing test, with or without background noise.

 

[Juan]

How bad is your hearing loss,in what way is it affecting you?(Can't hear tv etc.)

It's harder to hear tv for instance. I would get what they are saying in general but miss out many words. Also it is harder to understand high-pitched voices, the voices of women for example. And it's harder to understand anything with background noise. I feel I am missing speech that other friends understand perfectly when we are in a group.

 

[Frédéric]

Demystifying autoimmune inner ear disease

Introduction

Autoimmune inner disease (AIED) is an uncommon cause of sensorineural hearing loss and poses a diagnostic challenge. The present study aims to review the existing knowledge on the clinicopathological aspects, the diagnostic challenges, and therapeutic interventions in AIED.

Discussion
The incidence of AIED is less than five cases per 100,000 population. There are no definite seromarkers which make diagnosis of AIED difficult. Even though various markers have been studied, their sensitivity and specificity have not been replicated in the clinical scenario. The treatment of the condition is also an enigma. Corticosteroids are the drug of choice and require long-term use to prevent relapse. Various other therapeutic agents have been studied in a small cohort of patients, but the efficacy of these drugs needs to be validated in a large multicentric trial.

Conclusion
Timely intervention can restore hearing loss in AIED patients, but the clinician has to find a delicate balance between the hearing outcome and the potential side effects resulting from long-term use of the drugs. Treatment of steroid resistant AIED is a challenge and there are no universal guidelines for the same. AIED being an uncommon diagnosis, multicentric trials and collaboration are required to formulate diagnostic criteria and therapeutic guidelines.

Keywords
Inner ear Autoimmunity Hearing loss Sensorineural Diagnosis Inner ear antigens Circulating antibodies Positron emission tomography Magnetic resonance imaging

Source: https://link.springer.com/article/10.1007/s00405-019-05681-5

 

[xland]

Hi, I believe I have AIED, which is diagnosed more by exclusion than direct diagnosis. A month of Prednisone if you have AIED will dramatically improve symptoms. Tinnitus is not my major problem, it's extreme fatigue, vertigo, headaches and nausea. It's vestibular symptoms that cause me to sleep or lie down for 12 to 14 hours per day. I am not tired, just dizzy, feel like a low grade fever a lot of the time and need to lie down. Even sitting up lasts only 2 hours or so.

Not all AIED has vestibular/vertigo symptoms, but 50% do.

AIED is autoimmune disease which affects the soft tissue connected to the inner ear (essentially bone), easier to say AIED is arthritis affecting tissues around the inner ear, so it's like having a permanent inner ear infection with labyrinthitis.

I have had quite serious psoriatic arthritis since 1997, this requires quite strong rheumatoid drugs, like Sulfasalazine, Methotrexate, Duloxetine. I have lost the use of 3 or more joints; all my feet and hands (I mean fingers and toes).

Nowadays this type of soft tissue arthritis can also be treated with "biologics" e.g., Remicade etc. Very expensive bi-monthly injections.

So usually an underlying autoimmune condition would be a prelude to AIED. And hearing loss in my case is the least of my concerns.

I am going down the path of my local doctor and my rheumatologist to decide on the best course (drug) for treatment.

Hopefully a biologic injection which I now qualify for under public health.

So to stress again, AIED is a rheumatology disease, requiring rheumatologist based treatment and monitoring, not ENT as rheumatology disease is not an ENT's area of expertise.

Regards,
xland

 

[xland]

This video has the best treatment description for AIED I have found.

 

[Ela Stefan]

Hi, I believe I have AIED, which is diagnosed more by exclusion than direct diagnosis. A month of Prednisone if you have AIED will dramatically improve symptoms. Tinnitus is not my major problem, it's extreme fatigue, vertigo, headaches and nausea. It's vestibular symptoms that cause me to sleep or lie down for 12 to 14 hours per day. I am not tired, just dizzy, feel like a low grade fever a lot of the time and need to lie down. Even sitting up lasts only 2 hours or so.

Not all AIED has vestibular/vertigo symptoms, but 50% do.

AIED is autoimmune disease which affects the soft tissue connected to the inner ear (essentially bone), easier to say AIED is arthritis affecting tissues around the inner ear, so it's like having a permanent inner ear infection with labyrinthitis.

I have had quite serious psoriatic arthritis since 1997, this requires quite strong rheumatoid drugs, like Sulfasalazine, Methotrexate, Duloxetine. I have lost the use of 3 or more joints; all my feet and hands (I mean fingers and toes).

Nowadays this type of soft tissue arthritis can also be treated with "biologics" e.g., Remicade etc. Very expensive bi-monthly injections.

So usually an underlying autoimmune condition would be a prelude to AIED. And hearing loss in my case is the least of my concerns.

I am going down the path of my local doctor and my rheumatologist to decide on the best course (drug) for treatment.

Hopefully a biologic injection which I now qualify for under public health.

So to stress again, AIED is a rheumatology disease, requiring rheumatologist based treatment and monitoring, not ENT as rheumatology disease is not an ENT's area of expertise.

Regards,
xland

What tests do you need to do to find that out?

How would you know if a long-term middle ear inflammation, which could affect the soft tissue connected to the inner ear (essentially bone), started a sort of local AIED thing?

Is there a medication that you could try, if the tests don't show any results?