SSHL - an auto immune issue?

[Freedom2B]

For those with SSHL - sudden sensorineural hearing loss - there is a long, highly informative thread here:
http://www.medhelp.org/posts/Hearing-Loss/SSHL-recovery-timeframe/show/1478159?camp=watch_list

A brief recap of my situation:
After a severe emotional trauma (dog hit and killed by car), I woke 2 days later with my left ear feeling 'full.'  Minimal hearing in that ear.  No tinnitus, no dizziness or nausea.  Saw my PCP same day, she diagnosed Eustacian tube issue and put me on Flonase.  After 10 days of no improvement, she referred me to an ENT.

ENT diagnosed SSHL, I had several hearing tests, left ear showed severe hearing loss, primarily in the lower frequencies.  I had 3 intra tympanic injections of decadron, one per week, waited one week, redid hearing test and major improvement.  So ENT advised to wait 3 months and redo the hearing test as further improvement could be expected.

I found the conversation in the thread I linked above, and on my own I started the Magnesium, Vit B, etc.  ENT said these would not have any impact on my situation.  I also asked him about the CIST - contra induced sound therapy - which appears later in that thread, and he felt that was not going to make any difference for me as this is now pretty clearly an auto immune issue.

ENT also explained this was most likely either a viral infection or an auto immune issue.  The auto immune diagnosis came based on family history and my own health issues, specifically Hashimoto's hypothyroiditis.

Returned for hearing test after 3 months (that was 6 days back), and my hearing in left ear and declined back to where it was before the injections.  ENT authorized an MRI to rule out an tumors - MRI clear, no issues.  

At this point, it has been 5 months since onset.  ENT advises this is no longer a virus, this is an auto immune issue.  I am having a 'flare up' with stress as partial cause.  We will redo the injections and, if hearing test shows improvement again, ENT will insert a tube in the ear, so I can put in drops at home.  Drops will be 5 daily to start, and after a few months, we will redo the hearing test (oh gosh I already know all the words by heart lol), and see if we need to continue the drops (if the hearing has been sustained) and perhaps taper them, maybe to every other day.  

I've not seen anyone else post about SSHL as an auto immune issue.  This is a bit scary as it means long term hearing issues in this left ear, possibly same issue in right ear, and lots of other things will / can happen to my body.  During the wait for the MRI results, I was even MORE stressed, and my first spot of vitiligo appeared on my left hand -- have a family history of this, and it is an auto immune issue.  

Family history, my Dad has this auto immune syndrome and has: vitiligo, diabetes, Hashimoto's, pernicious anemia, Addison's disease, and reactive arthritis.

Would like to hear if anyone else has this auto immune connection.

[Clark99]

If you research the blood brain barrier, you will find the Prednisone doesn't penetrate.  Why ENT practitioners use this as a first line of defense for SSHL is not understood.  Dexamethesone is designed to penetrate the blood brain barrier and should be prescribed.  The problem however is these are catabolic steroids and have severe side effects, despite short term.  If you are trying to decrease inflammation, you will need to reach the cochlear nerve and the one very small vessel that supplies blood to it.  I have SSHL and only via my internist came to realize the prednisone is like splashing water on a house fire.  I went on a high dose of Dexa, unhappily, but willing to suffer the physical atrophy of my body in the chance that I would regain my hearing.  After 3 1/2 weeks began dexa intertempanic injections, on the 2nd one now.   I would recommend the intertempanic from the beginning rather than salvage - it gets the steroid to the right place needed without the side effects.  Also, regardless of cost, if you're losing your hearing permanently what is the real cost of hearing?  I'm glad to see a place like this to share these things.  Helpful resources are also pubmed.org where you can search for sshl and see clinical studies, albeit these are not very joyous.  Get treatment immediately.  See a neuro ontologist.  ENT's rarely know what this is and with only 4000 cases per year, yours probably hasn't seen it before.

[Freedom2B]

Thought I would update this.  I have been trialing a HA since April 22, and finding I like it very much.  Had 3 adjustments thus far.  While it is not like 'normal' hearing, it is much preferable over not wearing the HA.

[Freedom2B]

Had my hearing test last Wed, saw the ENT doc on Thurs.  Hearing has declined.  So , he says no point in continuing the injections, not responding.  No point in putting in the tube.  Time for hearing aid.  Saw audiologist today, for one hour info meeting.  Tried 2, liked the Opticon Nera.  Need to figure out how I will afford it.  My insurance pays a max of $700, and it is more than twice that for the cost.  LOVED having the demo in, I could hear properly again!  Oh what a tease, hated to take it out and leave not hearing.  Of course it was just a demo, couldn't have walked out with one today anyway.  

[Nancy T]

The only thing I've noticed is that when I'm very tired--underslept--the tinnitus is louder. In fact, it's kind of a tip-off to me that I really need to get more sleep!

Haven't noticed any connection to anything I ate, but your hearing and tinnitus seem to be more variable than mine, so I'm sure it is indeed a good idea to keep track of what makes it better than worse.

Take care, good luck!

[Freedom2B]

Thank you Nancy.  For some reason, that day it was REALLY LOUD, unusually loud.

Been dealing with this since Oct and yes most days I just keep going and as you said, sometimes I will stop short and wonder, "what's different?"  The answer is the hissing is almost non-existent.

But 2 days back, the entire day, it was a blaring hissing sound in my ear, impossible to ignore.  Someone else told me this may be stress related, or it could be due to some food I ate (her tinnitus gets loud in response to sulfates?).  Guess if / when it happens again I'll have to compare what I ate in the 24 hours prior, both times.  (I am using myfitnesspal online to track calories, so I do have a record of what I eat daily).  

Thank you for the support!

[Nancy T]

So sorry the tinnitus is driving you nuts. It can be frustrating (understatement of the year, right??).

I don't know how loud yours is, but I made peace with mine by acceptance--which sounds like a cliche--but I just began thinking of it as my "friendly neighborhood tinnitus", just a part of me the way I was. I know that sounds corny but it did keep me from thinking about it, worrying about it, and letting it intrude on my life. Now I don't even think about it unless something draws my attention to it, and on the rare occaions when it seems to stop or lessen greatly for a couple of hours, THAT's when I notice it and think, what's wrong??

You MUST find a way to make peace with it and start ignoring it. It may very well lessen over time. But even if it doesn't, you CAN'T let it get a toehold and become an anxiety issue for you, because then the vicious circle starts. You can't let it intrude on your life. You have to dismiss it from your mind and concentrate on something else. You have to stop thinking about it as some evil, alien, outside, uncontrollable thing and start to "own" it.

This is probably easy for me to say because mine is not very loud.

AND, I hope you are aware that if you cannot make peace with the tinnitus yourself, there IS help--various therapies that you could try. There are clinics that deal specifically with tinnitus. They won't make the tinnitus go away, but they can really help you deal with it and make it a non-issue in your life. Here is one link to specialists:

http://www.ata.org/members/doctor-search

Take care and keep us posted!

[Freedom2B]

I am going inSANE with the constant hissing in my left ear today.  Can not concentrate on ANYTHING!  This is nuts!  GAH!!!!

*runs from the room*  **sound follows me out of the room**

[Nancy T]

Hi Freedom 2B, glad that your appointment today seems to show that you have a doctor who is knowledgeable and on top of things and good at working with you. That is half the battle!

I can imagine it must indeed be depressing to realize that you may have to be dealing with hearing changes into the future, but I think your ENT's "slow down" approach makes sense. No one has a crystal ball, but lots of times things don't turn out as bad as we fear.

I too had a time of terrible fear when a neurologist (14 years ago) all but told me I had MS. I remember thinking, am I going to even be walking in a year? My whole life looked different. But I never actually got a diagnosis and nothing really bad ever happened like I expected. Our bodies and diseases are unpredictable. You do have a hard road ahead but I hope you will be able to deal with things well with the help of your good doctor. Wishing  you very best of luck.

[Freedom2B]

Back from the ENT visit.  Had the first injection for this series.  

Doc thinks I am rushing things and way over the top over reacting.  He advised me to slow down, calm down, and let him guide me as to when I need to start making future plans.  He said that the testing in this area is not specific, it just provides guidelines.  He is still quite confident I will recover most if not all of my hearing in the left ear, and that the other ear will not be at risk any time soon.  One thing he said was that I didn't notice any improvement when I clearly had plenty according to the hearing tests after the first round of injections, and I didn't notice my hearing deteriorated to get in sooner than the scheduled 3 month retest.  He said that is going to dictate some of what he does in the future; he can't rely on me to recognize what is going on.  He has one patient who keeps a supply of 2 mg prednisone, and if he notices hearing changes, he takes one pill a day for 3 or 4 days, and that clears it up.  That will not be part of my care plan, as I don't seem to know when my hearing changes.  

When I saw him (the ENT doc) last week, I had taken in the link for the CIST study, which was posted in the previous thread.  Here is the study: http://www.nature.com/srep/2014/140129/srep03927/full/srep03927.html

Today he brought that up right off the bat.  He thanked me for bringing it to his attention as he had not seen that one.  He felt the study was well run, he liked that they include the MRI results.  He printed it out and circulated it to all the docs in the practice (there are 7 ENTS total).  He encouraged me to work with some headphones and follow the plan in that study.  He said the practice is going to investigate what equipment is out there so they can offer more specific guidance to others in future.  So this was a really fantastic help from this forum!

I will have the second injection next Friday and then redo the hearing test; he doesn't think we need to do all 3, if there is going to be improvement we should see that after 2.  Then he can insert the tube and I can put in drops here at home, rather than going in for the injection.  So that is where things stand for me at this time.

[Freedom2B]

I would also appreciate more specifics as to what you mean, what happened, when you write:  my disease worsened.  If you don't mind sharing.  Do you mean your hearing got worse?  The second ear was involved?  I'm not sure what you meant with that statement.

[Freedom2B]

Thanks for posting.

Not redoing the MRI, I'm not sure what I wrote which suggested that.

My cochlear is fine, ENT has already tested for that.  My issue is clearly the nerve(s) not working, or not working properly.

I will look at the new link you provided shortly.  I have searched 'auto immune diseases' in the past 2 weeks, and saw a long list but hearing is not part of any of the list.  (My Dad has about 80% of them at this point).  

None of those things (CRP etc) have been tested.  I did discuss testing with the ENT, when he learned I have hypothyroid.  There is a test for this which would specify whether it is auto immune.  His response was very helpful: "for women, more than half the cases of low thyroid ARE auto immune.  You really do not want the test and have this documented in your medical file, for insurance reasons.  At some point, you may need to pick up additional insurance, or long term care insurance.  Once you have the diagnosis of auto immune documented, any insurance you add after that will not cover ANY auto immune issue -- even if in your case it is not auto immune, they will argue that it is and you won't be covered as they consider it pre existing so there would be no point in you adding the insurance."

I can totally relate to what you wrote, BC about how you felt when you got the diagnosis.  That's it in a nutshell: this isn't going to go away, there is no cure???  Yep, that is where I am at, shocked and saddened.

So, got to get ready to see the ENT, talk, ask questions, have the first-of-3 intra tympanic injections again.  Maybe I will come back with more information.  

[beachcomber13]

If your auditory nerve is spared, a cochlear implant would be an option. This would be down the line, I suppose. If your auditory nerve is involved, they are doing amazing things with auditory brainstem implants.

Here is some more info, including a list of systemic autoimmune diseases know to have inner ear involvement sometimes. Not that you need anything else to worry about, but others who have or may have autoimmune inner ear disease may want to check their other symptoms against these diseases.
http://www.asha.org/aud/articles/innereardisease/

I don't know how you feel, but I know how I felt when my autoimmune disease was diagnosed. "Wait, I might wake up one day blind? Unable to walk? This pain is not going away? There's nothing you can do about any of the problems I have already?" Not pretty.

I began working with a psychologist a few weeks after my diagnosis. It was not a miracle cure, but it really helped. I learned strategies for the worries and for the times when my disease worsened (and it did, about a dozen times). I grew up hearing that head shrinking was quackery, but I was willing to try anything for that horrible anxiety after diagnosis.

A diagnosis where there is not much to be done flat out stinks. I'm sure we can all imagine the words that really describe it, which are not used in polite company.

There is not much going on right now, but I encourage you and others to visit clinicaltrials.gov and search for autoimmune inner ear. You can see clinical trials recruiting, so that you can contact the researchers. But I think the terminated trials are also of interest. I like to track the results, see if anything is moving toward FDA application. I would hope that these applications would follow the "orphan" process, which means a faster decision. When an application is filed, patients are often asked to testify how much their disease stinks, how they would rather have a drug that has even a small chance at working and preventing further damage, because right now there are no good options.

Has your doctor checked your CRP? C-reactive protein is a measure of general inflammation in the body. I would have it monitored. If it starts to rise, steroids might be considered. Dietary supplementation is also important. I know you did not mention it here, but I feel like you and others have mentioned supplements somewhere on the forums. Any supplements used to bring down inflammation certainly cannot hurt, since autoimmune diseases tend to worsen as inflammation rises.

Freedom, I wish the best for you. I hope you do not lose more hearing and that a hearing device can help. I hope the drops and injections help (keep in mind that tubes and drops can give you a bit of conductive hearing loss, which would be temporary). I hope your MRI will be clear again--somehow I think it will be.

Please keep coming back and updating us. You may be a ray of light for someone googling the same problem, helping them not feel so alone. (If anyone else is in a similar situation, do not be shy about posting!) Take care.

BC

[Freedom2B]

Yes, I was remiss in not posting a thank you for Nancy, for providing that link.

I did see it and read it the day it came through in my digest.  And I did further reading once I had a 'name' to search in Google.

My ENT has said right along I had either a virus or an auto immune issue.  He based the virus on what many cases of SSHL are; he based the auto immune based on family history, and what other health issues I have.  The fact that my hearing declined after the injections is why he has now ruled out virus. So in my case, the doc did NOT just throw out the virus issue because he had no idea.  

The reason I did not reply (again, very sorry Nancy!), is that, while the prognosis in my case for SSHL is poor, as I read  more about aied (autoimmune inner ear disease) the prognosis for me seems even worse, and I became quite depressed.  While I have only 1 ear involved currently, it seems aied will impact both ears, that hearing loss will progress with time, will become profound loss, and on and on.   It also sounds like, while the BAHA is an option for sshl, it is not for aied.  Again, I may be misreading the online info.

I have my next ENT appt this afternoon (re starting the injections and hoping that those help again), and I want to discuss my 'diagnosis' with the ENT more, now that I have more information.  There are things I need to think about NOW, if I am reading and understanding aied correctly.  Perhaps he has no clear cut diagnosis, just information which suggests this or that.  Fine, but what expertise he has I need him to share with me.

I am also being forced to face the fact that, no matter which form of ear issue I have, it seems clear that I HAVE the auto immune syndrome my father has.  The stress of waiting for the MRI brain scan results brought on my first spots of vitiligo, on my left hand.  (And the fact that it is my left ear was not lost on me, that my left side is being impacted most at this time)  Which means I now have 3 auto immune health issues, withOUT counting the ear issue.  Needless to say, my head is spinning.

[Nancy T]

What Beachcomber said about doctors. Ditto!! Great post, thank you. You are spot on with the thing about throwing "virus" at you when they have no idea.

[beachcomber13]

First, thanks to Nancy for posting the link about hearing loss and dizziness as an autoimmune inner-ear disease.

Freedom2B,

SSHL or SSD can absolutely be autoimmune. In fact, we do not even know how common this is, because statistics on it are difficult to gather. People can have gradual or sudden hearing loss due to it, and audiologists/ENTs may not look for reasons for gradual hearing loss in people over age 50. Age-related hearing loss is assumed.. but age itself is known not to be a cause of hearing loss (unless only Europeans and Americans loss hearing naturally due only to aging, because we know that Africans do not).

Another issue is that doctors love to think that things are viruses if they do not know what is going on. I remember that when I was in college, I had a persistent cough, and I went to the doctor after two weeks. He looked me over and said it was a virus, drink plenty of water. Two months later, I was back in his office, and he did a simple blood test. I had a bacterial infection. Five days of antibiotics, and I was as good as new. My point is that doctors tend to throw the word virus on everything and assume that it will stick. No one likes to say, "I don't know," doctors least of all. So people on these forums are unlikely to come here with "My doctor said it's an autoimmune issue," because their doctors are unlikely to say that to them.

And the last issue is that I think doctors don't care what it is (for lack of a better phrase), because there is not much they can do. Steroid injections and oral steroids are what are typically prescribed, and they are generally considered appropriate for autoimmune disorders. So they are doing what they can for an autoimmune disorder or virus, hoping that it works and not really caring why, because there is not much else they can do. Speaking of the medical community in general--I know that some practitioners are creative and forward-thinking, but sadly, many are not.

I think that unless a doctor is punched in the face with a history of autoimmune disorders (if they even ask), they are unlikely to think in that direction. But folks with hearing loss are not alone in that. I have MS, and most of us hearing "stress" or "anxiety" before our actual autoimmune disease, despite symptoms perfectly aligning with MS and the fact that stress does not cause that constellation of symptoms.

Doctors. We have to stay on top of them in order to get appropriate diagnoses and services. Kudos to everyone who visits this forum and others like it.

BC

[Nancy T]

Not sure exactly what you have, but here is an article from a very well known hearing and dizziness expert about autoimmune inner-ear disease (AIED).

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

Sorry you are suffering from this--it must indeed be scary. Best of luck to you.