Finally got my diagnosis... NOT MAV! PLF

Specific Illnesses & Conditions
1

Just thought I’d give a little update…

In November, I finally got an appt with a neuro-otologist, Dr. Lehrer in Teaneck, NJ. He was great, almost taking 3 hours with me on my first visit. I did the balance (romberg) testing as well as all the hearing stuff. He didn’t believe I have MAV. He think it had something to do with too much fluid in my endolymphatic sac OR in my perilymph. He wanted to do the lasix test but I am allergic to sulfa drugs so he had me come back yesterday for the glycerin test.

The test consists of drinking glycerin which is supposed to suck all the fluid out of your head. Then every 45 minutes [for 3 hours], you get your balance and your hearing tested, and compare the results to baseline [he tested me before I drank the glycerin]. I did the glycerin test. and I got better with it in my system than without (less swaying on balance tests). He said this indicated that there is a leak in the perilymph. He dx’d me with perilymph fistula, in both my ears. He gave me a diuretic and he said my only other option is surgery. I am going to see him again in February.

I thought this was strange cos I read that PLF is very rare. Even rarer than meniere’s. And in both ears? Super duper rare. What’s stranger is that I’ve NEVER had any head or ear trauma [I told the doctor this]. No car accidents and I never played sports. I’ve never gone scuba diving, only snorkeling, over 2 years ago, and never had any ear issues from it. I read people with PLF have trouble flying. I fly 2-3 times a year, and this past time (over thanksgiving), I didn’t even use “ear plane” ear plugs the whole time and I felt fine. Got the usual ear clogging that even my husband felt upon descent. Most of my “dizzies” with flying come from walking around the actual airport. However, as the PLF symptoms do describe, I do feel worse with more activity, which is only then relieved by rest. But that’s only sometimes… ie: Some days I can marathon shop at the mall and feel perfectly fine. Other days, I have trouble working around the house. Mind you my dizziness isn’t true vertigo, just this lightheaded/“drunk” feeling.

While I was doing my glycerin test I snuck a peak at my chart and my visit notes from my very first visit, which included all those testing results. I was wondering if someone out there could help me with the technical terms for the audiometry portion:

*Exam showed slight shakiness on the romberg and shakiness on the quix with a sway to the right. There was no spontaneous or positional nystagmus, but she tilts back on the quix after the positional testing.

The CAE shows a slight drop at 6k in both ears with some asymmetry on the BSAS on the right. There was positive fistula testing in both ears, more so on the left. She will return for a glycerin test*

I know that the 1st paragraph is those balance tests and I understand that. Was wondering if someone could explain what the second paragraph meant. I’m a bit confused because in the office when he showed me my hearing test chart he said I had the hearing of a 16 year old! (I am 26).

I am so confused cos I was so positive I have MAV. I’ve been on propranolol since the end of July for mav… but it hasn’t done much for me. I’ve only been on 40mg/day though cos anything above that I get major insomnia. I know PLF is a very controversial diagnosis and the only way to really know is surgery… but I don’t want that! I am so frustrated. It’s ironic how all I wanted this whole time was proper testing and a diagnosis, and now that I got it, I feel even more confused and upset. I guess I’ll start the diuretic and see how I feel. Just wondering if anyone else out there got a PLF diagnosis without any head trauma.

*this post is x-posted to the “inner ear disorders” healthboards board

2

Hi Suki,

It is very rare. I’d get a second opinion before I let anyone operate in there. In the meantime, have you adhered to a migraine lifestyle and has that made any impact?

S

3

Yeah I will for sure get a second opinion. I spent my afternoon reading PLF forums and I was shocked to see how many people have actually gotten the surgery. I’ve never had any kind of surgery before so I will save it as a very very very last option.

I have tried to adhere to the migraine lifestyle – I bought the heal your headache book and I definitely found that certain foods made a difference in how dizzy I felt-- mostly nuts, chocolate, & alcohol.

How do people with a diagnosis of MAV do on the romberg/balance tests usually? I was way off. As in, I was standing there feet together and as soon as I closed my eyes I started to sway… it was scary!

4

I had the PLF surgery on the left ear 18 days ago. Here is what made my Neurotologist suspect PLF.

  • Valsalva induced vertigo
  • Fistula pressure test induced vertigo
  • no personal or family history of migraine activity
  • I was able to hear a small about of fluid in the left ear at times
  • I was doing lots of heavy lifting a few days before symptoms started
  • Before surgery I always fell to the left during Romberg test, Quix test, and Singleton Turning test (I now test normal after surgery)

The surgeon didnt see any leaks during surgery, but this is very common because the leak is often microscopic. For the first few days after surgery, I still had the same symptoms as before surgery but they were magnified about 10 times worse. During the last 2 weeks I have got a little better, but still worse than before surgery. I have heard that this is a pretty common reaction, so I have to be patient. I feel that my balance is a little better, but I now have a constant slow spinning and rocking sensation that I never had before surgery, this could be endolymphatic hydrops. I might ask to try a diuretic.

The surgery is not a big deal really, I only had a little pain for a few hours after surgery. Then I had no pain at any point after that. But I would give the migraine meds a good try before doing surgery.

5

I completely agree with Scott about getting a second opinion on the PLF diagnosis. As you found out, spontaneous PLF (especially bilateral) is very controversial (although I haven’t done any “research” on it for a decade, I guess). Your symptoms and history sure don’t sound like typical PLF.

I did have the surgery in 1999, but in my case I think it was justified because my sudden hearing loss and signifcantly worsened dizziness occurred when I sneezed hard. The surgeon found no leakage and I didn’t have any clear improvement. So it probably wasn’t a PLF that I had after all, although it was worth looking BECAUSE OF THE HISTORY.

If your doctor follows the regime of the famous Dr. F. Owen Black of Portland (NOT my doctor), who diagnoses a lot of bilateral spontaneous PLFs (at least that used to be the case; I don’t know about now), you will have a lot of post-op restrictions including strict bedrest and I don’t know what all.

I would be very doubtful and definitely get other opinions–second, even third if needed.

Good luck,
Nancy

6

Darren, sorry to hear you had no improvement with the surgery. Supposedly once the hole is patched (if there is one), you should feel much better almost right away. At least that’s what I was told.

The problem is that even if a PLF patient gets better only months after the surgery, how do you know it was due to the surgery and not some other factor(s)? It shouldn’t take months to feel better.

Good proof of PLF would be seeing a leak (although you can have two doctors look into the same ear during surgery and one will see a leak, the other won’t; apparently some different kind of fluid besides perilymph can be present too, so it’s tricky unless it’s a clear-cut case) or having the patient see very fast, significant improvement.

Caveat: all my “knowledge” about PLF is many years old.

Nancy

7

Nancy, I would have thought that recovery from PLF surgery would be quick, this is especially true in acute cases that just occured. But this was actually the exception in my research, most of the research I have done says that recovery from chronic PLF takes up to 6-8 weeks, and sometimes more if hydrops complicate things. Did you feel a lot worse after PLF surgery? If so, when did you get back to the “baseline”?

8

Hi Darren–I felt a lot dizzier than usual only immediately after the surgery, while still in the hospital for the afternoon. I went home late in the day, and in the subsequent days my dizziness was about the same as always–fluctuating but not really worse than before the surgery. It was the same old, same old. (I just looked it up in my old journal to confirm my memories of it.)

People who’d had the surgery kept telling me I’d soon feel my ear “clearing out” as my hearing returned, but that never happened. I had absolutely no hearing improvement. My hearing loss was probably caused by an inner-ear stroke when I sneezed (best theory).

I hope you soon begin to feel better! I was quite disappointed when I realized, the day after the surgery, that I WASN’T any better, because I had expected to be.

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What does PLF stand for?

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PLF stands for perilymh fistula.

Nancy, your reaction is what I would expect from an MAV patient after PLF surgery. I wonder why my symptoms got severely worse for a week after surgery, and they are still a bit worse and different than before surgery. The good news is that I never fall over to the left during romberg or quix test anymore. But I still have motion sensativity, and a new symptom of 24/7 slow spinning…

11

Darren–I don’t know why your symptoms got worse after surgery; that is a good question for your ENT.

It must be very upsetting to have symptoms WORSE after surgery. Ugh. Quite the opposite from what one would hope and expect.

I can’t remember, were you ever under suspicion for SCD?

Interestingly, with respect to your no longer falling on the Romberg, I will say that about 10 days or so after my surgery, a certain bit of imbalance that I’d been having for several months did clear up. (When turning around in the kitchen, for example, often my foot would not land quite exactly where I intended it.) But this may have been completely unrelated, as I had other odd coordination symptoms, including a summer-long bout of missing certain things when reaching for them, that came and went as they pleased. And my head felt no different after the surgery.

Have you been back to the surgeon for follow-up? I would let him/her know that your symptoms are worse as soon as possible.

Good luck to you–hope you see some improvement soon.

Nancy

12

Nancy, thanks so much for sharing the info :slight_smile:

It is quite upsetting to feel worse after surgery. I am under suspicion for perilymph fistula. My neurotologist insisted that I didnt have MAV, but I cant be quite so confident. I had a post-op with her and she said its normal to feel much worse for the first 3-5 days after surgery (I was a lot worse the first 6 days). She wasnt surprised that I dont feel better yet, she said I should start feeling better in another week or two.

Ive been having pretty bad vertigo, its a slow rotation that I notice mostly when Im not moving. I need to let her know about this too, I will email her.

Thanks for letting me bounce around some ideas! :smiley:

13

Hi Darren,
How is your vertigo feeling now? Though surgery is still a couple months out for me (if I choose to go ahead with it that is), I am still so nervous that it will make me feel worse/bring on new symptoms like with you and the vertigo

14

Im 4 weeks out and still struggling. I’ve had a few really good days, but otherwise Im still as bad or worse than before surgery.

I just dont know why my symptoms got so much worse after surgery. This flairup makes me reconsider the possibility of MAV. But If I had MAV, I dont think my symptoms would be effected by surgery. If I did have PLF, then secondary hydrops could explain everything Im still experiencing. I now have 24/7 slow spinning vertigo, tinnitus, and some hearing loss in the left ear, I didnt have any of this before surgery.

Id suggest giving several MAV meds an adequate trial before doing PLF surgery. If several meds make no difference, then do the surgery. Its not a serious surgery, I only had a little pain for 2 hours after surgery. I wouldnt worry about the surgery permanently making symptoms worse, but temporary worsening of symptoms is expected, especially if you actually have a fistula. Scholarly literature reports no occurrences of permanent worsening of symptoms, and I havent seen any personal accounts of this happening. However, I have seen many accounts of people who felt worse for a few months because of hydrops, and later made a successful recovery.

15

Darren, I have always been under the impression that secondary endolymphatic hydrops is something that develops a long time (months?) after an inner-ear injury or disease. BUT I could be wrong. The usual caveat: My “research” is all many years old, and my knowledge was never expert or complete anyway.

16

Nancy,

From my understanding, SEH can occur either immediately after closure, or years after. Both have been documented in literature. Dr Black and Dr Gianoli are big proponents of immediate SEH after PLF surgery. Their reasoning is that chronic PLF causes the inner ear to overproduce fluid (probably both perilymph or endolymph) in order to keep up with the constant leak. Once the leak is abruptly closed by surgery or successful bedrest, the ear still has increased fluid production, and it builds up and causes SEH. It generally takes a few weeks to months for this to calm down. But there have also been cases of SEH starting years after successful PLF closure, and this flaw in the ear is likely the cause, of the previous PLF (in my opinion).

17

Just wanted to bump this, because I had trauma induced dizziness for 5 weeks, then a clear 5 months and subsequently an onset of MAV.

I wonder if MAV is actually SEH!

SEH is definitely distinct from Menieres as there is no significant hearing loss, but in other respects it’s very similar.

I wonder if the SEH pressure is high enough it can temporarily reopen a fistula ‘from the other side’? Or perhaps exploit a congenital weakness that fell foul of the original trauma. Perhaps such a ‘reopening’ might cause the vertigo we experience?

I’m convinced that migraine symptoms are secondary to the cause, possibly SEH.

I wonder if this mechanism is the evolved ears response to implosive trauma to help ‘fix’ the implosive wound? It becomes an exacerbation and can cause chronic fluctuation of pressure: periodic breaches of the round window then a repressurisation ?

Given the labyrinth evolved first in fish I wonder if the pressure regulation was developed when fish lived at various depths that might have applied different levels of pressure on the inner ear windows … requiring an ability to increase pressure within?

18

I would also get a second opinion - if your symptoms get worse after heavy lifting it sounds like you may have neck or back issues causing too many false signals to your cerebellum (MAV). Nothing to do with your ears that part I think - so get a second opinion bedfore surgery, I have had two bad relapses in symptoms because of lifting something heavy and going ten pin bowling - but have tested nil for any ear canal problems…

paul.

19

Oh yes I don’t think surgery can fix SEH and most are sceptical about it fixing PLF (most heal spontaneously they say but noone can be sure)

20

Darren, if you ever return could you point me towards a reference or two of this … I’m now diagnosed with SEH post PLF (well the PLF is still healing but leak is very much less than it was) and wondering if you know of any success stories where SEH calmed down. My worst symptom is now tinnitus as much of my vestibular symptoms are well controlled by Amitriptyline.

Hope you are still doing well!

Cheers!

James