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Secondary Endolymphatic Hydrops or Perilymphatic Fistula?

#1

Hi there. I am so glad I found this forum as I have read some excellent posts thus far. I would like to get your 2 cents on my situation. I sincerely look forward to your feedback!

My story:

I attended a Justin Bieber concert here in Chicago with my daughter in December of 2010 only to be exposed to some very serious acoustic trauma I believe is the culprit of my current situation. As we awaited for JB to come on stage, Nelly (hip hop artist) come on stage first with some very loud music. As Nelly’s first song progressed, I started to feel there was something seriously wrong with my right ear. I heard loud sound distortions at first and then I could not hear much from my right ear. I also felt slightly off balance. I decided to leave the premises (My daughter stayed with my wife of course) and noticed I had lost about 60% of hearing capability in my right ear. I also noticed a roaring tinnitus settle in. I immediately went to see an ENT and they conducted some hearing test which all came back normal so the doctor made nothing of it. Over the course of the next month or so, the tinnitus completely went away and I recovered my hearing to a capacity of 80% of what it was prior to this incident.

Over the course of the next 2 years, I experienced on my right ear once or twice a month a very peculiar swooshing sound that would start out at a mid tone frequency and disappear at a lower tone frequency for about 5 seconds. The frequency of these weird sound incidents increased to about 3 to 4 times a month during the last 6 months of this 2 year period.

On May 29th, 2013, a permanent low tone tinnitus (still hear it today) in my right ear settled in at night. 2 months later, Aural fullness and dizziness (non-rotational) settled in as well. 2 months after this, I experienced aural fullness on my left ear but no tinnitus and the dizziness invoked by it was mild. My initial doctor diagnosed me with Meneire’s Disease but I obtained 2 more diagnostic opinions consisting of a Perylimphatic Fistula. One of these second opinions came from Dr. Timothy Hain (Highly Acclaimed Otoneurologist in Chicago) whom is currently treating me. Dr, Hain recommended a conservative approach to allow my PLF to heal. 6 months after consulting with Dr. Hain, my dizziness and aural fullness on both ears disappeared and I thought my PLF had healed - side note- Dr Hain believed my PLF was on the right ear and for reasons he himself does not understand, my left ear “was jealous” and thus mimicked my right ear to a lesser degree however. The only symptom I was left with was low tone tinnitus in my right ear which varied in loudness- Loud in the winter - almost inaudible during the summer and fall.

Fast forward 6 years to December of 2018- my tinnitus loudness was the highest its ever been and my mild to moderate dizziness (non-rotational) returned after a really bad allergy season (allergic to ragweed). An important consideration here as well is that I started a very hard core exercise program in July of 2018 that physically stained me more than ever before.

I returned to Dr.Hain and he then reversed his original prognosis and instead believes I may have Secondary Endolymphatic Hydrops (aka Atypical Menieres- A more constant but milder form of symptoms associated with Meniere’s Disease) I have been taking Betahistine 48 mg daily and it seems to help reduce my unsteadiness \ mild dizziness. Nevertheless, I now experience daily Aural pressure on both ears and really annoying mild to moderate lightheartedness

Thanks for reading this far! Here is the climax to this story- The more I think about my symptoms, the more I believe a may have a minor case of bilateral PLF related to the acoustic trauma I experienced 9 years ago. I feel immediate relief when at rest, I cannot tolerate elevators or any major pressure fluctuation including barometric pressure (which is why things seem to go bad in the winter when the barometric pressure increases significantly here in Chicago). I cannot bend over forward or even do light exercise without feeling lightheaded or mildly dizzy. Most recently, i discovered that simply placing an ear plug (noise reduction plugs I use for attending concerts) on my left ear, my moderate symptoms turn into very mild ones.

So here is the million dollar question- Should I have an exploratory tympanatomy performed to once and for all determine what I have and this take the corrective measures? I have been waiting since December of 2018 and although my symptoms are rather tolerable and mild, they are continuous on a daily basis.

Tony

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#2

Hey Tony, welcome aboard!

So sorry to hear about your terrible experience.

I’m going to try to be brief: my personal opinion, having suffered an ear injury myself and done a lot of research into the anatomy of the ear, is the following:

  • PLF will generally heal spontaneously (as per Hain, 90% is his hunch)
  • You are almost guaranteed to have a degree of hydrops after PLF because it messes up your endo/perilymph balance
  • 66% of PLF operations fail according to Hain, but my surgeon says only 20% fail (but there’s no science to determine if the successes wouldn’t have worked out spontaneously anyway).
  • The lack of efficacy of the operation makes sense - a patch of the windows cannot magically fix the complex 3 dimensional fluid imbalance.

So I’m with Hain on this one. I would be wary of surgery as it might upset things more.

What we don’t know if there is a natural recovery process. There’s no deep understanding of how the fluid maintenance works.

In my experience, with what sounds like a slightly less severe breach than you had, I initially had only a balance problem for 5 weeks, which resolved. Unfortunately after a glorious 5 months of no symptoms I was hit with the MAV truck and it took me over 3 years to get my balance back, and there are loads of signs that things have improved. I’ve done nothing but follow the ‘MAV’ treatment like Hain suggests and this has worked for me so far. I still get fluid sensations though they have lessened a lot, but I’m usually neurologically sound these days. My tinnitus is purely a high hiss which varies in intensity and I’ve lost some HF hearing in my bad ear but I suspect this is a dual result of a change in the resonant frequency of my ear (due to the fluid imbalance) and the pressure, which would obviously dampen the window vibrations.

Here’s another interesting case:

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#3

Thank you so much for your speedy response James. I too have spent countless hours doing research I almost feel I am ready to get certified as an associate specialist of the anatomy of the ear. I appreciate your advice to stick to a conservative approach preached by Dr. Hain. I too have come across conflicting reports ranging from 50 to 90 % post surgical success rates. It is scientifically very difficult to come up with a an accurate percentage as it would entail accounting for all related surgical procedures done in a particular geographical location. In other words- we need better statistical sampling performed.

Meanwhile, I was contemplating the possibility of taking diuretics as a supplement to my current Betahistine regimen mentioned above. Betahistine is a H3 receptor antagonist which is uses a vascular dilation approach to improving blood circulation in the inner ear. I would imagine that a diuretic like Triamterine HCTZ would act in tandem to reduce endolymph fluidd volume in the Semi Circular Canals and the Cochlear memebranous ducts. I currently exercise a low sodium diet with less than 2K mg. It seems to help but my goal is to potentially eradicate lightheadedness \ mild dizziness. thoughts?

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#4

Hi Tony,

Welcome! I just wanted to cautiously mention an observation that has come up on this board- the observation that several have had their symptoms show up after intense exercise/weight training. I believe @turnitaround has brought this up as well. James, would this suggest a recurrence of PLF? My guess is that those who get PLF are somehow prone to it? Just wondering about the timing with the exercise you mention…

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#5

Explore advances in PLF surgery where folks use liquid bone to seal the patch. Also there is a blood patch done in one University of California. My guess is if the patch is fixed the fluid imbalance should fix itself by the endolymphatic sac releasing the excess pressure (posted a while ago on this, happy to dig it up).

#6

Hi Napagirl,

Yes- that has crossed my mind indeed. I am currently pausing my workout and I am going to wait at least a couple of months to see if my symptoms improve. If they do, then indeed it may be a recurring PLF on my left ear this time. Thanks for your input.

Tony

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#7

Interesting GetBetter. Please do (if you can) post that link. Thanks for your feedback!

Tony

#8

Let me be clear by no means am i saying go for the surgery. I am just trying to educate you on the various options available.

Here you go on the endolymphatic sac

One more on the blood patch

Blood patch

A procedure known as a blood patch is the first line of treatment. This can be can be performed in the office, and you will go home the same day. The blood patch procedure usually works the first time. Occasionally, it has to be repeated.
http://www.ucihealth.org/medical-services/ear-nose-throat-ent/hearing-ear-disorders/perilymphatic-fistula

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#9

PLF may have caused the problem in the first place but what you likely end up with is Secondary Hydrops. This raises the pressure of the ear and intuitively probably makes you more at risk of continuing breaches as blood pressure hooks up with ear pressure and there is less slack in the system. Therefore the pressure on the inner ear windows must be more influenced by exacerbations of blood pressure than in a completely healthy ear. So yeah, you could be more at risk from straining than normal.

But note this is not the PLF causing most of the risk, it’s likely the hydrops. If there was just a healing PLF and zero hydrops given we know normal pressures in the ear are fairly low the PLF would surely normally get on with healing undisturbed. But unfortunately the hydrops probably interferes. When you get a healing PLF being reopened intermittently because of excess blood pressure the result could be a never ending problem. Hopefully the Hydrops reduces slowly and this risk reduces over time. Maybe intermittent leaks are a normal part of the healing process! After all you only need a trend with hydrops reduction and recovery would then be inevitable. Who knows if, perhaps some cases, when the PLF and Hydrops is so bad, surgery might help by artificially helping to keep the PLF closed for long enough for the Hydrops to die down before it’s no longer reinforcing the PLF. That may be why some surgery works where spontaneous healing was not an option?

There must be a mechanism by which the Reissner membrane sustains its position. This must be based on osmosis and pressure. It’s probably a slow balancing influence. If you get trauma the balance might be kicked out quite far and I speculate that the rebalancing takes years given my experience and that of others.

The anatomy of the ear is far from simple. Abstractly, the Reissner is kind of like a balloon inside an egg. The balloon can expand and retract based on competing pressures/volumes of the fluids inside and outside of it. The eggshell (bone) doesn’t move. Leak one of those fluids significantly and you have a recipe for long term upset to homeostasis. Loss of homeostasis inevitably causes instability and changes the bad ear’s mechanical and electrical response to head movement resulting in the ears disagreeing and neurological fallout eg dizziness.

Note that PLF is surely only one cause of hydrops. There may be congenital issues that make people develop it or there may in some people be even a peripheral biochemical element that causes the perilymph region to shrink that is a result of some breakdown in a process in another part of the body, eg diabetes, hypertension

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#10

I’ve got much better despite not taking a diuretic. I did try one when I was quite ill but gave up as didn’t see any obvious benefit (although this could simply have been that I was so ill nothing seemed to help). I think you could try it. I know @Young_Lee tried a diuretic at one point, perhaps he can chime in?

Both this approach and betahistine are quite old school approaches. Neither drug survives Cochrane Review I believe?

To reduce dizziness symptoms you might explore Amitriptyline. That drug helped me a lot although it’s not without its side effects.

Has Hain recommended any low dose antidepressant that might help with that side of things?

Do you get any fluid sensations in your ears?

Can you increase your tinnitus temporarily by clenching your jaw?

#12

I have taken diamox for 4 months and don’t think it really helped. Maybe it worked for the first week but that could have been placebo. I hated the side effects (tingly and numb hands and feet). Ami is a better med for me.

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#13

Thanks for the insight James. We can sit here and try to deduce precisely in which form homeostasis is being disrupted by hydrops but unfortunately, the greatest challenge medical scientists have is getting undisturbed clear access to the main culprit of this disruption- The human Endolymphatic Sac. It is well protected by bone tissues and thus very difficult to analyze under an electron Microscope. It was great to observe in @GetBetter post where we can actually see the way in which the Endolymph flow is cointrolled by lamellar barriers.in the endolymphatic sac of zebrafish. But until we can observe the same in a human, only then we can learn how to control the overflow of endolymph which is the main causant of hydrops.

Regarding your question about Dr. Hain’s medication recommendations- he has not recommended Amitriptyline. He mentioned that if I need additional meds, he would prescribe a Diuretic but I am concerned about the side effects. I took Diamox at some point only to develop a Kidney Stone 3 months into the regiment :expressionless:

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#14

@Young_Lee- see my previous post. I developed a kidney stone 3 months after taking Diamox 6 years ago…

#15

Youch!

#16

Yes indeed. It’s nice to know there is possibly a pressure valve though! It might be one of the ways homeostasis is restored: when peri/endolymph reaches a certain pressure this valve opens to release some of the latter. I guess this could have ‘evolved out’ as it wouldn’t be the first time humans have lost something useful over time :smiley: . But surely perhaps because it’s been replaced with another mechanism that leads the pressure to be kept within certain bounds so the valve ended up not being used (so no longer critical for survival and procreation)

I’ve written earlier about my thoughts on this organ in fish (where it surely first evolved?) : there must surely be a way this organ copes with the varying pressures of depths.

#17

There is currently a regulator valve surgical procedure for eliminating Vertigo in Meneire’s Disease called - Endolymphatic Shunt Insertion -Endolymphatic Sac Surgery for Ménière's Disease – Current Opinion and Literature Review

According to Dr Hain- It is only about 50% successful.

Tony

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#18

Not very good odds: with that kind of invasive procedure I’d want 95% success.