Auto Immune Inner Ear Disease - CI

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Paullys50

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Hey all, I'm new. Writing this one day post op of my Cochlear Implant. My case seems to be pretty unique and I had difficult time finding others in my situation. I experienced sudden hearing loss in my left ear 1.5 years ago, within 3 days I was completely deaf in my left ear. Tried steroids and injections, nothing. Thought it was a virus that caused it, doc's said it would be like "lighting striking twice" for something to happen to my right ear.

Well, after a year I had learned to cope with the hearing loss (had lost vestibular function too) and felt like I was back to myself only to get hit with problems in my right hear. Thankfully my right ear has been fluctuating for the past few months, it's bad then good then bad again. It's given me time to understand Cochlear Implants and become a candidate for one on my left ear. Ultimately they now feel it is Auto Immune Inner Ear Disease, I have a history of Ulcerative Colitis and other "auto-immune" issues. My vestibular function is shot in my right ear as well so my balance is all based on vision and feel under my feet. My vision is very blurry as it just can't filter vibrations out anymore.

Seems like I am a very rare case, and as such it was difficult for me to find information. This has been very traumatic for me, as I had excellent hearing prior. Been a rough road but I'm excited for my Cochlear Implant. At this point it is very likely my right ear hearing will continue to decline despite the steroids and auto-immune treatments I have been pursuing. Very happy to get a jump on quickly. I hope to adapt well to the CI, however my expectation is simply speech, to be able to communicate. I know that music and sounds as I once heard are gone.

So I'm 1 day post op, being activated on March 26. I choose Med-El. I choose them for a few reasons, one I liked the small size of the implant and the modular battery options. I liked the remote, and after discussions with my surgeons I feel this is the least traumatic implant from a surgical perspective. I was also very pleased to hear about the "Rondo" processor. You receive two processors with Med-El, one for a "backup" and I can exchange that backup for the Rondo come April so I can have each type which I think is a pretty cool option to have.

Originally I thought AB, but after having each device in my hand it just Med-El just seemed to fit me. I know I'm in the minority in-terms of the 3 companies, but I have high confidence that all 3 would serve the purpose just as a Ford, Dodge or Chevy do.

Looking forward to activation! Hopefully others don't get hit with what I got hit with, completely turns your world upside-down it seems!
 
didnt realize the Rondo was coming out so soon. That's pretty cool. Hope your recovery goes smoothly and that you have a great activation!

I don't know anything about auto immune inner ear disease, so I can't help you with that.
 
I'm VERY interested in learning from you. My case is similar, sudden hearing loss-18 years ago but never got totally deaf. I have reasonable success from hearing aids but things are getting worse--speech recognition especially. I've been trying to find people that were hearing well before CI and how they hear after the fact. Good luck with your recovery. I say on my thread you were at Mayo--whats the skinny on how they do things--same day or did they keep you overnight? I have no known cause for my hearing loss.
 
Paully, were CI surgery buddies! We had surgery on the same day :) My activation is sooner than yours, March 20, but how cool!

I've never heard of your loss, I'm sorry. That must be very hard to lose your hearing so quickly and suddenly as an adult. I lost my hearing instantaneously at 8 years old. It was mostly scarey. But I was young and adapted well. I wish you great luck with your CI!
 
HOH-ME said:
I'm VERY interested in learning from you. My case is similar, sudden hearing loss-18 years ago but never got totally deaf. I have reasonable success from hearing aids but things are getting worse--speech recognition especially. I've been trying to find people that were hearing well before CI and how they hear after the fact. Good luck with your recovery. I say on my thread you were at Mayo--whats the skinny on how they do things--same day or did they keep you overnight? I have no known cause for my hearing loss.
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Mayo was very efficient. I was not kept overnight. I checked into the hospital at 9:00am, and was on my way home at 4:30pm. I feel like I am recovering ok, get some discomfort but the pain meds take care of it. Feel pretty tired still, but it's only been 4 days. I did not look into any other CI facility, quite frankly Mayo acted so quickly that I just went with it. I had been seeing an ENT doctor in St. Paul who was great, but was reserved when it came to moving forward with a CI because I was not a clear cut candidate and insurance coverage was a concern (due to my right ear working so well on "good" days, didn't have much tangible data on my "bad" days). Long story short, I went to Mayo for other consults (trying to mitigate the auto-immune issue) and as a result I requested a CI consult just to have the conversation as I felt at some point I may be a candidate. Next think you know the ENT gives a green light for a CI, I have the CI testing performed (thankfully my right ear was having a very bad day) and 10 minutes after the CI test I had the green light from insurance and I'm talking about the 3 CI choices. I went from having zero expectations of having a CI to having surgery scheduled all within about 4 days. It's been very very quick!

Getting anxious for my activation, thinking about calling to see if I can do it a week sooner, I have a be family outing on March 23 and it would be cool to have it activated by then to show everyone.
 
Paullys50 said:
Mayo was very efficient. I was not kept overnight. I checked into the hospital at 9:00am, and was on my way home at 4:30pm. I feel like I am recovering ok, get some discomfort but the pain meds take care of it. Feel pretty tired still, but it's only been 4 days. I did not look into any other CI facility, quite frankly Mayo acted so quickly that I just went with it. I had been seeing an ENT doctor in St. Paul who was great, but was reserved when it came to moving forward with a CI because I was not a clear cut candidate and insurance coverage was a concern (due to my right ear working so well on "good" days, didn't have much tangible data on my "bad" days). Long story short, I went to Mayo for other consults (trying to mitigate the auto-immune issue) and as a result I requested a CI consult just to have the conversation as I felt at some point I may be a candidate. Next think you know the ENT gives a green light for a CI, I have the CI testing performed (thankfully my right ear was having a very bad day) and 10 minutes after the CI test I had the green light from insurance and I'm talking about the 3 CI choices. I went from having zero expectations of having a CI to having surgery scheduled all within about 4 days. It's been very very quick!

Getting anxious for my activation, thinking about calling to see if I can do it a week sooner, I have a be family outing on March 23 and it would be cool to have it activated by then to show everyone.
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I'd ask this in a private message but you don't have enough posts yet--do you mind elaborating on your auto-immune disease. I'm curious as to what you have specifically. I've wondered if my loss was autoimmune related. I had some funky test results initially but nothing was investigated further because they just didn't think that was the cause (at Mayo). Since then several autoimmune diseases have surfaced in various family members and I've been thinking I should retest.
 
HOH-ME said:
I'd ask this in a private message but you don't have enough posts yet--do you mind elaborating on your auto-immune disease. I'm curious as to what you have specifically. I've wondered if my loss was autoimmune related. I had some funky test results initially but nothing was investigated further because they just didn't think that was the cause (at Mayo). Since then several autoimmune diseases have surfaced in various family members and I've been thinking I should retest.
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I don't mind elaborating.

Basically, the auto-immune inner ear disease is really not a solid diagnosis. Basically comes down to using other issues within your body to conclude that the hearing loss is related. It's rare, so there is not much out there to support it, however there are doctors out there aware of it.

I have Ulcerative Colitis (which is a well known auto-immune disease). Years ago I had my large intestine removed to mitigate this illness, which was the best thing I could have done for it. I was extremely healthy. Over the past 5 years or so I started to develop weird manifestations such as muscle aches and pains, as well as odd skin rashes. None of which had a direct causes, but could possibly be classified as a "manifestation" of my Ulcerative Colitis (some could argue I really have Crohn's disease). Over the years I just coped with those issues best I could, but once I started to lose hearing in my right ear odds of it being a random virus went south and odds that something else was happening in my body went up. Ultimately the ENT will refer you to a rheumatologist if they suspect auto-immune related hearing loss, as the root causes is the auto-immune system and rheumatology is responsible for that treatment. I am now on an immuno-suppressent medication to hopefully combat any further hearing loss. Now, not all rheumatologists are aware of a connection between the immune system and hearing loss. So it's important you find one that knows what you are talking about.

I have had a few MRI scans as well, and there is some indication that my inner ears are affected, which support the "auto immune inner ear disease" diagnosis, however still seems pretty soft in-terms of evidence.

Ultimately, you need to ask yourself what other symptoms you experience besides hearing loss. If you have a bunch of auto-immune related, or symptoms of unknown causes maybe it is an auto-immune issue. However, all the treatments end up being the same. Steroids and immune-suppressents of some type. Treatment success rates aren't solid and you basically take the drugs to see what happens unfortunately.

I'm really concerned about completely losing my hearing in my right ear, very scary to figure out how to cope in the world I'm in the prime of my career, mainly concerned about my job, getting married, family...adapting to life without those pleasure I once had. Granted I hope a CI helps me move forward with the fundamental communication skills that I require, but knowing that I'll never hear the same again really hits hard.

For the record, it took awhile before I felt Mayo was doing me any good.
 
Wow. As if they implanted with having a "fluctuating loss" even it its only in one ear...I don't know where you live but I would imagine its in the states.
 
haha no lol ...at least at my centre they won't. Our government healthcare pays for the CI so they make up all these excuses why you're not approved when really it's to save money.
 
Paullys50 said:
Hey all, I'm new. Writing this one day post op of my Cochlear Implant. My case seems to be pretty unique .

Seems like I am a very rare case, and as such it was difficult for me to find information. This has been very traumatic for me, as I had excellent hearing prior. Been a rough road but I'm excited for my Cochlear Implant. At this point it is very likely my right ear hearing will continue to decline despite the steroids and auto-immune treatments I have been pursuing. Very happy to get a jump on quickly. I hope to adapt well to the CI, however my expectation is simply speech, to be able to communicate. I know that music and sounds as I once heard are gone.
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I hope the CI Works for you! I'm trying to get my insurance to approve a CI for me.
You really aren't that unique in having sudden SNHL in one ear and a few years later, sudden SNHL on the other side.
Last year I lost nearly all the hearing I had left in my left ear within a ay or two.
I already had severe hearing loss in both ears but I didn't realize how disturbing it could be to suddenly be even more deaf in my "good" ear.

There are many conditions (EVAS, autoimmune hearing loss etc) that can cause random and severe or complete loss of hearing in one or both ears.

I hope you do well without CI!
 
Wait, I just re-read your first post.
You only have fluctuating in your contralateral ear?

Recently I've seen a few stories about people with profound deafness in only one ear getting cochlear implants and honestly, that pisses me off. I have severe to profound SNHL in both ears but insurance won't cover a CI but some people with single sided deafness can be approved (albeit in different countries)? Not fair.
 
I lost 100% of my hearing overnight in both ears. Seems there are many ways this can happen. Mine is due from a sudden onset of Sepsis. Doctors are not quite sure why because it is not a common side effect of Sepsis but that is when it happened. I had some other serious issues when I was sick but I was deaf before I reached the ER.

I have a CI (surgery May 2012)in one ear only and even though my CI journey was difficult I am happy that I did it.

Good luck!!!
 
ecp said:
Wait, I just re-read your first post.
You only have fluctuating in your contralateral ear?

Recently I've seen a few stories about people with profound deafness in only one ear getting cochlear implants and honestly, that pisses me off. I have severe to profound SNHL in both ears but insurance won't cover a CI but some people with single sided deafness can be approved (albeit in different countries)? Not fair.
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This is why I was surprised he was even implanted. I have a severe/profound loss with less than 66 percent (last time I checked) speech discrim and I won't even be approved....
 
Couple of things, yes I am in the States, and I am very fortunate and grateful for the insurance that I do have.

Left ear: Completely Deaf
Right ear: Downward spiraling fluctuations.

ecp said:
...
You really aren't that unique in having sudden SNHL in one ear and a few years later, sudden SNHL on the other side...
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I have seen multiple doctors at various institutions and all have said to me that sudden hearing loss is quite uncommon for both ears. You would think that if it was very common, that a CI would have more leverage in SSD cases if long-term medically it was a known that bilateral loss was on the horizon given unilateral loss exists. Why would anyone even consider a BAHA type solution if they had SSD and a good chance of becoming bilateral? I had a conversation early on about a BAHA to cope with my SSD, thankfully I choose not to do that! How much more complicated would it have been both with insurance and surgically had I gone ahead with a BAHA right away?

Surprised to hear some pushback on my situation, after all I have not given any details of how much my "good" ear fluctuates. The reality is that on my bad days/weeks I can maybe understand 20% of what is said to me if you are 6 inches from my head. This includes using my $$$$ Phonak Ambra HA (I do have a CROS setup) and cranking the volume up as high as I can. Distortion is my biggest problem, don't get me wrong the decibel level drops off, but distortion kills any hearing I have during those bad days/weeks. On my "good" days/weeks speech is pretty clear, but tones are all off. I'm lucky to understand the TV on my "good" days.

The day I had my CI evaluation testing, my "good" ear just happened to be having a really "bad" day. I meet all the qualification criteria, don't remember all the testing but at one point during the test my speech discrimination was maybe 18%? If they were to re-test another day would I meet it criteria if I has having a "good" day? Guess we won't know.

For insurance, I was not expecting a green light this quickly. I fully understood that I may not be a candidate given the situation with my "good" ear. However we prepared to present a good argument to insurance for a CI, many factors were brought up such as the length of time, the prognosis of maintaining my "good" ear, the 20+ audiograms I've had over 3 months showing the fluctuations, my current health....blah blah blah. Thankfully it has worked in my favor. I fully understand the "life is not fair" statement, and I wish that others would have the opportunity that I have. If I was "stuck" waiting for my right ear to completely go before I would be considered a candidate life would be....a straight up bitch. I fully sympathize with anyone in the same situation. What do you do? Continue to work and hope it doesn't affect your performance. Mentally prep yourself? But how? Learn ASL? Try to become a member of the "deaf" community even though you really aren't? such a mental game to play....
 
lovezebras said:
This is why I was surprised he was even implanted. I have a severe/profound loss with less than 66 percent (last time I checked) speech discrim and I won't even be approved....
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With our health insurance I don't even need to get cochlear implants pre-authorized. If my Dr says today that CI's will help me, I could have surgery tomorrow (assuming they can get everything scheduled at the hospital and the implants are available). The Mayo clinic technically isn't in our network of providers (a choice our employer made unfortunately) so I would have to get authorization to go there. Not sure how likely that is or not.
 
Yes but the CI surgeon/CI audi themselves still have to approve you even if insurance isn't a problem and that's what I'm talking about. People with a fluctuating loss or single sided deafness are getting approved when there are people like me and ecp who have trouble and are severe and/or profound and struggle with hearing aids and we aren't getting approved.
Fluctuating and ssd isn't apart of the criteria for a CI so it kinda sucks that they'll make exceptions for some and not others is what we're saying.
 
John Hopkins has been researching the use of CI for SSD, it's a very recent thing.

Also, each center/surgeon will vary on who will be accepted for CI and who won't. Perhaps the center you go to is more strict. Not all centers use the exact same criteria.
 
Tell me about the Rondo and why you are excited about it. What does it have that the current one does not?
 
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