MAV or Perilymph Fistula

Specific Illnesses & Conditions
1

Well my Propranolol trial made no improvement in my symptoms after 5 weeks. My ENT still thinks I have MAV. But his reasoning is simply based on the fact that I dont have nystagmus on the hallpike-dix test. There is really no other reasoning for his suspicion. I dont think I have MAV because my symptoms are different than most MAV people. Most MAV people feel rocking, I have never experienced rocking because I only feel dizziness and unsteadiness/nausea when I move my head or walk. Most MAV people feel better when moving and worse when sitting still, I feel fine when Im still and dizzy when Im moving. Most MAV people have a history of either migraine pain or non-pain migraine symptoms, I have absolutely none of either. I have experimented with avoiding migraine triggers and it makes no difference.

All of my symptoms can be explained by **perilymph fistula. ** I have read case studies of perilymph fistula and my symptoms sound more like that than MAV.

First Occurrence of Dizziness
My first bout of dizziness started after a bad cold 2 years ago and it lasted only 4 days. I returned to completely normal.

Current Problem
My symptoms started 14 months ago immediately moving to a new house, this move involved lots of heavy lifting and straining. Several days after moving, my symptoms started with a spinning vertigo spell lasting 10 minutes. After the first spell, I was left with chronic symptoms that I still experience today:

  • Dizziness and nausea when I move my head, or by increased physical activity.
  • I often hear water sounds in my left ear. Certain head positions stimulate the sound and that causes ear pain and increased dizziness. I often get extremely sharp pains in that ear, sometimes randomly, and sometimes caused by altitude changes, yawning, or chewing.
  • Chewing on left side of mouth causes eyes to jump (oscillopsia)
  • I often feel heavy pressure in the back of my head.
  • Eyes frequently feel tired.
  • Consistent shift to left with Quix test.

Notes
These symptoms never go away completely, but I feel almost normal when I sit down or lay down.
No personal or family history of migraine. No history of motion sickness.

I am having an ENG test this week. If it comes back normal, my ENT will make an official diagnosis of MAV. But I am not comfortable with this because most perilymph fistula patients show a normal ENG. So the ENG probably wont help at all.

2

Hi Darren–

I was initially suspected of having a perilymph fistula (PLF) when I suddenly lost the hearing in my left ear and got dizzier, and I even had the exploratory/patch operation for it done. So I researched quite a bit about it in the “old days” (1999-2000). Although I cannot of course be sure, I don’t think your symptoms and history are very suspicious for PLF.

If you had given yourself a PLF from straining, that should have occurred immediately, right at the time you lifted something, not days afterward. Many of the symptoms you describe can be found in dizziness from several other causes and are NOT exclusive to PLF or even highly indicative of it. The only thing that sounds puzzling is the oscillopsia when chewing on the left side, but I haven’t heard of that being associated with PLF.

It is NOT true (as far as I know) that everyone with MAV has rocking and that they feel better while in motion. Many people have MAV without these factors.

I have never gotten a diagnosis in all these years, but I do believe I have MAV, as everything else has been ruled out and I am definitely a migraineur (have gotten the visual aura). I never have a rocking sensation (just dizziness with movement) and I feel worse in motion.

Ask your ENT again why he thinks you have MAV: lack of nystagmus on the Dix-Hallpike certainly isn’t enough to base it on! Also, is this a neuro-otologist you’re seeing? They are the real experts on dizziness, although oto-neurologists (neurologists–as opposed to ENTs–who specialize in dizziness and the inner ear) are the BEST experts on MAV.

Do you have any family history of migraine? If so, that makes migraine a likelier (though of course not necessarily definite) possibility. Migraine is HIGHLY hereditary.

Please be aware that the diagnosis of PLF can be highly controversial, because there simply is no good test for it short of surgery. The “gold standard” test is surgical exploration of the middle ear to look for leakage of perilymph through the oval and/or round windows. Even if no perilymph is seen, the windows will usually be patched just in case. The patient should have immediate and dramatic improvement from the surgery if PLF was the cause. (Obviously, I did not.)

Docs will highly suspect a PLF if dizziness and hearing loss started suddenly AT THE TIME OF a traumatic event (not later) such as heavy straining, diving or other pressure change, vomiting, Valsalva, and in my case–sneezing! However, a few ENTs (including notably one here in Portland; not mine, however) will diagnose spontaneous PLFs and often bilateral PLFs. Spontaneous PLF is still, I believe, a quite controversial topic and I would be VERY leery about such a diagnosis.

Definitely ask your doc what he/she thinks, but please be open-minded and don’t fix on PLF. Everyone wants a quick solution–I remember being happy thinking that I was going to get one with the PLF surgery–but it did not help, and this essentially ruled out PLF as the cause of my symptoms. (Which continue now 12 years later.)

Best of luck–believe me, I know this is all very difficult. Dizziness is often very tricky to figure out and the cause cannot always be pinpointed for sure. Keep us posted and let us know how the ENG goes!

3

Thanks for the response. Great info.

What do you think could cause the strange water sounds and sensations that sometimes cause dizziness in my left ear?

I am just seeing a general ENT because I dont have neuro-otologists or oto-neurologists in my insurance network. He hasnt made an official diagnosis, but MAV is his suspicion. He will make the diagnosis after the ENG. I emailed Dr. Wachym in Portland, I hope he responds because I think he treats PLF quite often.

I have no family history of migraine. It just seems unlikely that I would have MAV when I have no confirmed migraine mechanism.

4

Hi Darren. If I were you, I would make every effort to see a neuro-otologist or oto-neurologist–someone who specializes in dizziness. It’s possible that your ENT is knowledgeable about this stuff, but most aren’t experts in this field. You would probably do better with an ENT who has a certificate in otology/neurotology.

I think Dr. Wackym is in Dr. F. Owen Black’s enterprise, Dr. Black being in fact the doctor whose PLF theories and practice are so controversial in the field (or were, last I heard, which was a long time ago). So, do your research but please be very careful and get more opinions. San Francisco must have some good dizziness specialists. If your ENT is unable to help you (give him a chance, be willing to try different meds), ask for a referral to a super-specialist even if it means going out of network.

As for the strange water sounds in your left ear, I don’t know, but I do know (from reading dizziness forums for many years–am I pathetic or what! :slight_smile: that lots of people with various diagnoses describe all kinds of noise, fullness, pressure, pain, etc. in one or both ears. Be sure to let your ENT know about this symptom as well as all your others. PLF is actually relatively rare as a cause of dizziness.

Hopefully the ENG will provide some helpful information, but be prepared for it to be normal; it often is. Which obviously doesn’t mean nothing’s wrong. A balance-testing expert on another forum always used to tell us that if the ENG is normal and the doctor therefore concludes there is no vestibular problem, that’s like saying that since your keys aren’t on the table, they aren’t in the house! You have to keep looking for the source of the problem, with further testing or medication trials.

Good luck,
Nancy

5

Hi Darren,

— Begin quote from “ichbindarren”

I dont think I have MAV because my symptoms are different than most MAV people. Most MAV people feel rocking, I have never experienced rocking because I only feel dizziness and unsteadiness/nausea when I move my head or walk.

— End quote

I have never had rocking. The one thing I managed to escape. The dizziness I get will definitely get worse when I walk and move around – positional vertigo.

— Begin quote from “ichbindarren”

Most MAV people feel better when moving and worse when sitting still.

— End quote

Not true for me usually. Being still and relaxing improves things.

— Begin quote from “ichbindarren”

Most MAV people have a history of either migraine pain or non-pain migraine symptoms

— End quote

This is true for me although not classic stuff. I have had coat hangar tension type headaches for years which most GPs would never class as migraine. Others here (Kelley) never had any previous symptoms until a viral attack occurred.

— Begin quote from “ichbindarren”

I have experimented with avoiding migraine triggers and it makes no difference.

— End quote

Agree that this is unusual but again there are others here who have a very hard time finding clear triggers. That could be because there are none or because they have not yet identified something that is a trigger in their daily life. I’ve come across triggers that I never dreamed could set me off and yet they do.

Failing on propranolol is definitley not a clear sign that you are not a migraineur but perhaps you could seek a second opinion if you’re not convinced.

Good luck … Scott

6

Thanks for the info friends :slight_smile:

So it sounds like my symptoms and lack of headaches are less common features of MAV, but still possible.

I plan to try several more medications, most likely antidepressants. I was on Effexor for 1 day and I thought I felt better that day, but I cant say for sure. But I had to quit because of the extreme nausea side effect.

7

Hi Darren,
just wanted to say that I have MAV, and I would often feel better lying still rather than moving around (I would often need to lie down after too much movement). I also haven’t been able to clearly identify any dietary triggers.

— Begin quote from “ichbindarren”

I was on Effexor for 1 day and I thought I felt better that day, but I cant say for sure. But I had to quit because of the extreme nausea side effect.

— End quote

Were you literally on the Effexor for just 1 day?! Just wanted to say perhaps be wary of giving up on a med too soon, and try and reduce the dosage if possible rather than giving up…? I say this because I experienced really rough nausea for several weeks when I started the Topamax (about 9 months ago now!) - I literally didn’t want to eat anything as the nausea got worse - but once the body/system adjusts a lot of the side effects simply dissipate. The Topamax now looks like it might be working for me. I know it isn’t easy hanging in there, but just a thought…
Best wishes,
Tony.

8

Hi Darren,
I remember you from the healthboards inner ear forums. Your advice helped me a lot… Sorry to read that you’re still suffering :frowning: My GP diagnosed me with MAV and I don’t have the “rocking” feeling either. It’s just this constant lightheadedness/drunk feeling when moving/walking around. Mine is a lot better when I sit or lay down too. It’s interesting the spectrum of symptoms that MAV brings about… I feel like everyone feels something so different, no wonder it is often difficult for doctors to make the diagnosis!

PS - I also forgot to mention that while I don’t have water noises in my ear, I sometimes get very sharp pains in my ear too… Sometimes they will only last a few seconds, but the other night I had it for quite a while. I always attributed it to my allergies and ETD

9

Hi suki530,
Im glad that I was able to help. I have been suffering from MAV or something else, I dont have a definite diagnosis yet. I also have the lightheadedness when I move around.

I get really dizzy when I stand or walk when its dark. Does anyone else feel extremely dizzy and off balance in the dark?

10

— Begin quote from “ichbindarren”

I get really dizzy when I stand or walk when its dark. Does anyone else feel extremely dizzy and off balance in the dark?

— End quote

is it just when it’s dark outside, or does the same thing happen if you close your eyes or put on an eyemask?

11

Hi Darren–the balance system depends on three sources of sensory input: (1) inner ear, (2) vision, and (3) proprioceptors, the sensors in our muscles (such as the bottom of your feet–basically the sense of touch) that tell us the position of our body.

If one or more of these elements is compromised, the other elements have to try to take over. Thus, if your inner ear is damaged, you come to rely more on your vision, so when it’s dark or you’re in, say, a grocery store or other busy visual environment, you don’t have good vision to compensate for the inner-ear damage. This is also why dizzy people often feel better sitting down–you are getting a lot more proprioceptive input from the wide area of the “seat of your pants,” besides the fact that you’re not provoking dizziness with head movement.

Also, to maintain your sense of balance, your brain must be able to properly integrate the input from the three sources (ear, eyes, proprioceptors). If there is brain or brainstem damage, this cannot always happen correctly.

It is VERY common for people with dizziness problems to have a lot of trouble or even be unable to walk in the dark. Try to keep nightlights on, and if you have to walk in the dark, reach out for furniture or something to hold on to (to get that proprioceptive input!).

12

— Begin quote from “lorcalon”

— Begin quote from “ichbindarren”

I get really dizzy when I stand or walk when its dark. Does anyone else feel extremely dizzy and off balance in the dark?

— End quote

is it just when it’s dark outside, or does the same thing happen if you close your eyes or put on an eyemask?

— End quote

I get extremely dizzy if its dark and Im on a slanted or uneven surface. I think I would feel just as dizzy if I were to close my eyes too, but I havent tried it yet. This feeling goes away after if I sit down with my feet flat on the ground. Please share more of your thoughts :slight_smile:

13

Hi Darren,

It’s totally normal to feel very unsteady and unbalanced in the dark if you have any kind of inner ear problem. And the same if you are on an uneven surface.

It’s even normal for someone with no inner ear problems at all to be less steady without their sight to help balance them. You can see this if you get an obliging friend to stand still for a couple of mins with their eyes open, and then get them to do it with their eyes shut instead and you will find they will start to sway after a while. For those of us with inner ear problems it’s much worse, and can be to the point of not being able to stand at all. I was like this when my balance problems were at their worst, and had to turn the light on before getting out of bed.

Also, regarding your question about triggers, not everyone has them, or has obvious ones that can be identified. Although I have triggers for headaches, I never noticed anything that set off my dizziness, and the dizziness seemed to bear no relation at all to what I call my ‘normal migraines’. I’ve also struggled to accept the MAV diagnosis for this reason. However, I have eventually made a pretty much full recovery on migraine preventative meds, so I guess it was right (or else I just have something that’s eventually got better by itself).

Like TeeCee says, give the meds a longer go next time. Lots of people on here say about it taking a bit of time for side effects to settle down, so try to give things at least a couple of weeks. Also, a lot of the migraine meds need 4-6 weeks to see an effect, so a bit of staying power is definitely needed!

14

— Begin quote from “beechleaf”

Hi Darren,

It’s totally normal to feel very unsteady and unbalanced in the dark if you have any kind of inner ear problem. And the same if you are on an uneven surface.

It’s even normal for someone with no inner ear problems at all to be less steady without their sight to help balance them. You can see this if you get an obliging friend to stand still for a couple of mins with their eyes open, and then get them to do it with their eyes shut instead and you will find they will start to sway after a while. For those of us with inner ear problems it’s much worse, and can be to the point of not being able to stand at all. I was like this when my balance problems were at their worst, and had to turn the light on before getting out of bed.

Also, regarding your question about triggers, not everyone has them, or has obvious ones that can be identified. Although I have triggers for headaches, I never noticed anything that set off my dizziness, and the dizziness seemed to bear no relation at all to what I call my ‘normal migraines’. I’ve also struggled to accept the MAV diagnosis for this reason. However, I have eventually made a pretty much full recovery on migraine preventative meds, so I guess it was right (or else I just have something that’s eventually got better by itself).

Like TeeCee says, give the meds a longer go next time. Lots of people on here say about it taking a bit of time for side effects to settle down, so try to give things at least a couple of weeks. Also, a lot of the migraine meds need 4-6 weeks to see an effect, so a bit of staying power is definitely needed!

— End quote

Thanks for the reply. I have heard a lot of people with inner ear problems say that they experience symptoms exactly like what I described. Do MAV sufferers experience this too? Or is this more of an inner ear thing?

15

I feel worse in the dark. If I am bad, I cant go for a walk in the evening when its dark, I am staggering along the pavement like a drunkard. I sleep with a night light as I have to have some light in the room. The cinema drives me crazy, dark room and bright big screen.

Standing still on a slant makes me dizzy. Went to buy a house once and outside I was stood on a slant and felt very dizzy so dismissed the house.

Very open spaces can make me feel bad (another house I looked at had beautiful views but they went on and on) felt dizzy with that as well.

Not so bad with closing the eyes, for instance, closing eyes to go to sleep is OK. If I am having a bad vertigo attack, cant close eyes, keep them open and focus on one spot.

Christine

16

Christine, that’s funny about standing on a slant. I have become noticeably reliant on my feet to keep my balance, and I am very sensitive to what’s under my feet.

Once, in someone’s apartment, I told them their hallway floor slanted a tiny bit to the left. They said, “Naw!” But later they called me and said they’d taken a level to the hall floor and found out I was right!

I notice any slight unevenness or bulge in floors, whether linoleum, carpet, or whatever. Mostly seems to be a problem in public buildings such as stores, etc.

CANNOT stand on those spongy mats that are supposed to be good for your feet when standing. I feel uncomfortably off-balance on them.

Oddly, though, I don’t have much trouble moving around in the dark, as long as it’s not completely dark.

Nancy

17

So it sounds like these same symptoms are found in both MAV patients and vestibular damage patients. I am still waiting for an official diagnosis, I had a VNG today.

18

hi darren, i dont fit the MOULD exactly either. I had very bad ngstamus for roughly 10 mins, i have vest nerve damage in one ear and also have blood shot/dry eyes coinciding with onset of vertigo and floaters. I get headaches and havent had a major migrane in years. I can barely walk in the dark, i had bad balance for 6 months in which is getting better 2 yrs on. I still have a diagnoses of mav at this stage. i have no aniexty.I feel better only when i lie on my side.Somehow they say i got sjorgens syndrome,fibro and mav all at once!go figure?what else can you do other than try out what the dr suggest? Id definately get an opion from a neuro even if u have to pay. Mav is a matter of exclusion, so knock out everything else and thats what your left with. Vertigo can suck the money but who wants to live like this??Keep us posted every story helps get people sorted on the right track

19

— Begin quote from “beechleaf”

Hi Darren,

It’s totally normal to feel very unsteady and unbalanced in the dark if you have any kind of inner ear problem. And the same if you are on an uneven surface.

It’s even normal for someone with no inner ear problems at all to be less steady without their sight to help balance them. You can see this if you get an obliging friend to stand still for a couple of mins with their eyes open, and then get them to do it with their eyes shut instead and you will find they will start to sway after a while. For those of us with inner ear problems it’s much worse, and can be to the point of not being able to stand at all. I was like this when my balance problems were at their worst, and had to turn the light on before getting out of bed.

Also, regarding your question about triggers, not everyone has them, or has obvious ones that can be identified. Although I have triggers for headaches, I never noticed anything that set off my dizziness, and the dizziness seemed to bear no relation at all to what I call my ‘normal migraines’. I’ve also struggled to accept the MAV diagnosis for this reason. However, I have eventually made a pretty much full recovery on migraine preventative meds, so I guess it was right (or else I just have something that’s eventually got better by itself).

Like TeeCee says, give the meds a longer go next time. Lots of people on here say about it taking a bit of time for side effects to settle down, so try to give things at least a couple of weeks. Also, a lot of the migraine meds need 4-6 weeks to see an effect, so a bit of staying power is definitely needed!

— End quote

I would like to make a comment on something you said. You mentioned that your dizziness was not effected by migraine triggers, but your headaches were. I read something interesting that was published by Cutrer and Baloh. It stated:

— Begin quote from ____

Cutrer and Baloh suggested that when the dizziness is unrelated to the headache, the dizziness occurs from the release of neuropeptides (ie, neuropeptide substance P, neurokinin A, calcitonin gene–related peptide [CGRP]). Neuropeptide release has an excitatory effect on the baseline firing rate of the sensory epithelium of the inner ear, as well as on the vestibular nuclei in the pons.

Asymmetric neuropeptide release results in the sensation of vertigo. When neuropeptide release is symmetric, the patient feels an increased sensitivity to motion due to an increased vestibular firing rate during head movements. Cutrer and Baloh also proposed that CGRP and other neuropeptides may produce a prolonged hormonelike effect as these peptides diffuse into the extracellular fluid. This may explain the prolonged symptoms in some patients with migraine-associated vertigo, as well as the typical progression of persistent spontaneous vertigo, followed by benign positional vertigo, then motion sensitivity.

— End quote

I have not done a lot of research into the mechanisms of chronic MAV, but I think this explanation pretty much nails it. Continuous MAV symptoms are probably caused by this phenomenon resulting from inflammatory neuropeptides bathing the nerve cells. It is fairly reasonable to assume that anticonvulsants or antidepressants would stabilize the chemistry in the nerves, and I suppose theis action promotes the removal of the extracellular neuropeptides.

Reference

Cutrer FM, Baloh RW. Migraine-associated dizziness. Headache. Jun 1992;32(6):300-4.

20

darren thats the best explaination i have so far. I hope im in the boat where meds may fix me too, as you said its a life recker, im so over it 2 yrs and counting