It wasn't MAV

Specific Illnesses & Conditions VM/MAV
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Hi everyone,

I havent posted on here in a longtime but I do come through once in awhile and read the posts. The last time I posted I was in the process of being diagnosed with either MAV, PLF or SCDS.

I am 4 weeks post op now from a PLF and SCDS repair on my right ear and Im a 75% symptom free and feeling human again!! Of course ear surgery bring its own new “symptoms” so Im hoping things get even better as I reach the 6 week and 3 month recovery stages.

It was a very hard road to get to these diagnosis… I saw 9 doctors in 2 states over the course of almost a year. When I returned to the States a month after my symptoms started I was repeatedly told I had VN after 3 months went by and I didnt get better only worse then the doctors kept saying it might MAV or Mdds. I just knew it wasnt right especially when all the preventive’s were making me feel worse and couple of these meds I had been on years ago for another issue. I always knew I had a PLF but finding a doctor who believes in PLF’s can be damn near impossible. Finally, after seeing a doctor who specializes in PLF’s, I redid my VEMP test and it was positive for Superior Canal Dehiscense but he also said abnormal VEMP test can be positive for PLF’s as well. I had my CT re done but they couldnt see a hole in the bone just a “severely” thin right canal. Now I was in between 2 doctors one thought its just a PLF and the other thought its SCDS… I decided to go ahead with the SCDS repair and asked the doctor to patch the round and oval window also, he agreed he would. When he got in there he was able to visualize a tear in my oval window. He was glad I insisted on the PLF repair too!!

My surgery for the SCDS was a Transmastoid Resurfacing. Eventhough I didnt have a hole sometimes the bone can become so thin that its not functional anymore. There are few doctors that are knowledgable about SCDS and few that believe thin canals can cause SCDS symptoms. I look back now at how many Neurotologists looked at my CT scan and never even noticed how thin the canal was or asked me to repeat the scan because there are very special parameters for a SCDS temporal bone scan.

Of course I wonder if it was really just the PLF… who knows. I am 75% dizzy/vertigo/rocking free, no nauseau, no headaches, no more skakiness/inner vibration, no oscillopsia, brain fog is 50% better. I still have pulsatile tinnitus and regular tinnitus but it is probably from the packing in my ear.

Im not writing this post to discourage anyone that they dont have MAV. I 100% believe its real and a horrible condition to suffer from. This just my story and I want to share with anyone who might be suffering for years and not responding to MAV treatments. I had to do a lot of my own research and force doctors to listen to me but in the end Im glad I pushed.

Take care everyone!!

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wow thanks for that update!!! Did your Dr. say that you should keep improving as you heal? How did you feel immediately after surgery- any better? Has your balance improved at all since the surgery? That’s pretty amazing that he actually saw a tear in the oval window- most plfs are too small to see- good to have evidence that you really had that. What type of activity restrictions do you have now? Can you ever fly again? Are you back at work?

So happy to hear that you are doing better and I hope that things continue to improve for you!!!

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Its a long recovery from a SCDS repair but I chose the less invasive approach which is a transmastoid and he resurfaced the canal instead of plugging it which blocks of the canal. I have zero balance problems. The floor doesnt move when I walk anymore, no more trampoline feeling.

Im still on my 6 week recovery restrictions, I go back to work on July 26th. No bending, no lifting, no straining, no sneezing with my mouth closed, no water in my ear… I actually had ALOT of complications after surgery and my 24 hour overnight stay turned into 5 days. I just had problems with the anesthesia, vomitting, rash from the steriod and low BP. The first week or 2 after the surgery was HELL and I thought I had made a huge mistake. Now I feel more human than I have in the past year. I’ve been at the beach with my kids everyday, driving, going to the zoo, going to bbq’s,shopping, reading, things I couldnt do the past year.

I cant fly for a year and Im super scared to do that. I still havent been on boat since Im very scared to try it. Although walking on sand used to make so dizzy/vertigo but now I feel it for like 10 mins and its just gone.

The doctor said he was suprised when he saw the tear and leak because he was doubtful about the PLF. He told me I should feel very proud for not giving up.

Thanks Sarah!!! how about you? Any changes?

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that’s terrible about your complications after surgery- did your dr. think that the vomiting could have messed up the fistula repair? I can only imagine the panic and fear about feeling worse after surgery- thank god things have turned around for you. That is strange how your dr. said he didnt think you had a fistula- why did he not think this??? that sucks that you basically had to diagnose yourself and suggest treatment- that is sad on the part of the medical profession.

For your fistula repair what type of tissue did he use? How has your hearing been since surgery? That is awesome about the balance part- was your balance really bad before the surgery?

Yea I hear you about flying…did your dr. say that there is a risk of it re-opening with flying? how likely would that be?

I’m so glad to hear you are doing better- I imagine you will keep improving as you continue healing- did your dr. say this should happen?

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He was just more sure about tthe SCDS. I think he didn’t think my symptoms fit the PLF textbook symptoms but history was suggestive. After seeing & speaking to 2 docs who specialize in PLF’S they told me that fistula’ s are way more common than most doctors believe.

Im not sure what he used for the fistula but I know he used fascia & cartilage from my ear for the resurfacing. Yes, before the surgery I was basically bed bound and on Zofran Q8hrs to control my nausea.

I can fly in 1 year because I opted not to have a ear tube inserted.

I saw your other post Sarah and all my fistula tests were negative too. They are completely useless and I just want to add I didnot have any of the autophony or internal sounds that people typically have with SCDS except for the pulsatile tinnitus.

Honestly, so many of my symptoms were exactly what I would read people experiencing here. These vestibular conditions are so difficult to diagnosis and so many symptoms overlap.

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Thank you for taking the time to post your story and hopefully help others…glad you got most of your life back.

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Hi there–glad you have gotten improvement from the surgery; that’s great! I hope your recovery continues uninterrupted and that you will feel normal, or close to it, again. Then you can have a continuing certainty that these conditions were the cause of your problem and not have to be on the diagnosis merry-go-round anymore!

I was suspected of having PLF initially due to history (sneezing with sudden hearing loss and suddenly increased dizziness) but the surgery did not help and thus PLF was ruled out. I’m always glad to hear a PLF success story!

Take care and keep getting better! Please keep us updated on your recovery.

Nancy

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You had no hearing loss to begin with, right? How is your hearing now?

All I can say is that with no hearing loss and with negative fistula tests, it is almost impossible to get a surgeon to operate on you. In a way you were lucky to have the SCD finding- this gave the surgeon a reason to go in- otherwise at least with the drs. I have seen, no one would dare operate on me. Because of that, patients like me are basically left to rot…In all honesty though I think my fistula may have healed on its own, and I am almost positive that I have MAV too…so even if my fistula did get fixed, I would still have MAV to contend with…confusing.

They need to come up with a better test for that…I have had the fistula test done on me many times, 2 times it made me dizzy, but I never had nystagmus- also every dr. used a different looking instrument which I thought was weird. Drs. all seem to have a different idea on how accurate it is- it is just a mess.

Anyway, I hope things keep improving as time goes on and please keep us updated!!!

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No hearing loss. I have some distorted hearing now but its from the massive packing in my ear. I go in for my 6 week hearing test on the 23rd.

The hardest part is finding a doctor who knows about PLF’s… my doctor and the other 7 doctors didnt think my symptoms fit the “typical” PLF symptoms either but really when your dealing with something uncommon how do you know whats typical? I was so dizzy, off balance all the time it was hard to pinpoint what made it worse. Fistula tests are pointless… I always got dizzy with them but never had the nystagmus either. Have you ever had the tuning fork test?

I also had a VNG in the beginnging of this that said I had a 42% weakness in my left ear and knew this was wrong but no doctor would listen till the last 2 I saw told me how crude and barely useful these VNG’s are…

I just thank God, that I found a doctor who was a specialist in PLF’s and then another doctor who believed thin canals can cause SCDS symptoms. There is 1 doctor who specializes in both and does more testing than any other docs and if i could have afforded the travel I would have went to him. If you dont respond to MAV treatment maybe people should look into these rarer conditions… but not alot of ENT/Neurotologists know or specialize in these conditions so you have to travel to see the experts.

If you look online there are alot of articles by a Dr.Jeremy Hornibrook and he has alot of research & info about PLF’s and thats what really clued me in.

I truly hope you get some relief soon Sarah!!

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Many years later, I’m circling around to see how everything panned out for you.
I have been experiencing chronic dizziness for the last few years, and I’m still trying to find answers. At the stage now where I’m taking a medicine called acetazolamide to treat a possible perilymphatic fistula.

My symptoms are right ear fullness, pressure, and disequilibrium, but I can’t say I have full-spin vertigo anymore. It’s more non-spinning vertigo. I do have a history of BPPV but that vertigo is now gone, I’m just left with the constant sense of motion and bobbing, like I’m on a boat. Dealing with this off and on for over two years. I’m a weird case because I also have MS and had a stapedectomy in my right ear in 2016. So many layers of possible issues here. I’m just curious what your exact symptoms where and how you are now. Thanks!

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Sorry to interject. I’m curious why you had this procedure?

That’s very … interesting. Acetazolamide is a diuretic and whilst I can imagine it might be employed to help resolve a case of secondary hydrops, how is it supposed to counteract an active PLF?

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Hello and thanks for the response.

A little about me. But I’m an odd case; I’ll try to keep it as short as possible.

I’m a 42-year-old woman with a history of inner ear issues plus Multiple Sclerosis. I had tubes throughout my childhood up into my early 20s. History of infections, even today almost every year, Tinnitus, TMJ. (I even went to jaw therapy for TMJ in my 30s, hoping to relieve the Tinnitus, which was laughable.)

I have been told that I have otosclerosis in both ears. In 2016, I had it so bad in my right ear that my ENT proposed a Stapedectomy to correct the hearing, which it did. My right ear sometimes hears a little too well. It even made my Tinnitus a bit louder which was great. However, I still have that feeling of fullness and pressure often, especially during allergy/cold season. It’s now my left that has significant hearing loss.

I have seen 6 ENTS and 2 Otoneurolgists, and I have 1 Neurologist for MS.

I have a history of BPPV which turned into PPPD. I had a spinning episode on Jan 8, 2021, and I haven’t been the same. I no longer have active BPPV as far as I know. My symptoms include chronic lightheadedness, the feeling of rocking/on a boat, being pulled around from the neck up, involuntary eye movement (my environment twitches and usually goes with my heartbeat), moving walls (I hate bathroom stalls and the shower), uneven ground, overall disequilibrium.

I’ve been through two rounds of vestibular therapy. I feel better for a bit then it all comes back.
This last person had me keep doing the Epley maneuver, but while doing this maneuver, I would not feel the spinning right away, but I would feel a .25-second spin about 30 seconds in, and that’s it. The maneuver hurt my neck more than anything, so we stopped treating BPPV and continued the eye movement therapies. My 6 weeks are up she turns me over to a naturopathic physician for blood work to check anything else that may be happing internally.

Anyway, life happens, holidays come and go, work is busy, and I power through all the minor symptoms daily. I’m fine one minute, and the next minute everything changes.
It does change my quality of life because it will keep me from driving, flying, and being social. Your symptoms sound much more severe than mine, so I’m happy you have found relief.

Current remedies and practices: Anti-inflammatory eating (not 100%, but I try to eat pretty well for MS purposes), exercise when I can, elevated sleeping (I can’t sleep on my sides), plenty of rest and water, vitamins for MS-related purposes, walking, and when it gets awful, I have a prescription for Alprazolam (Xanax) which I don’t care to take long term. My neurologist suggested this. “Here slap this bandaid on.”

Right now, I’m back to square one, feeling my worst. Hard to walk and stay up straight. A double ear infection a few weeks ago set me off. Antibiotics and two rounds of Prednisone, and I’m still not 100%. Very woozy all the time, wobbly environment, hard to focus, and foggy brain; screens are annoying, and I’m a freelance graphic artist. Tolerating computer work right now, obv.

So I find a new ENT(#6), and he says words like perilymph fistula (PLF). And has me start this diuretic called Acetazolamide. He claims it can relieve pressure in the head/ears and eliminate possible fluid build-up. I have read the same. They told me to take it for 3-4 weeks. I’m on week 1. This PLF talk brought me to this forum.

I had never heard of the term hydrops before. So that is interesting. I read your comments about this, and it makes all the sense that the BPPV maneuvers could exacerbate my symptoms.

Thank you!

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Wow, that’s quite a journey!

I went through a long phase of believing I might have a PLF (backed up by the opinion of a very well qualified oto-neurologist who also told me this could lead to secondary hydrops). My best bet now is it was a metabolic inner ear issue all along (but very possibly still secondary hydrops). I’m very glad I didn’t have that surgery as it almost guarantees a degree of hearing loss. I spontaneously lost most symptoms (all of the balance problems are gone) and am left with a bit of fluctuating tinnitus and some very occasional minor neurological symptoms. If I’m very lucky, this will go too. I put this down in part to a reduction in stress levels by leaving my very stressful job. More lately, significant but not draconian modification of my diet (essentially intermittent fasting with the intention to keep blood sugar levels at reasonable levels) has shown some promise.

Explore the conservative solutions as much as possible is my advice, because those are usually reversible if it doesn’t help.

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Yeah, I’m with you there. Surgery is the last thing they would probably want to do, but we will see.
I find it interesting that Amitriptyline helped you for a long time. I’ve been told that going on some kind of SSRI would help my issue, but again, I want to approach this a bit more holistically before jumping into medication. Perhaps that’s only hurting me. As I said, I do not like taking Xanax but it seems to be the only relief these days.

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