meniere

Author Topic: hmsn and hearing loss  (Read 10789 times)

Offline jon

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hmsn and hearing loss
« on: Sep 16 2005, 11:17 AM »
At last someone has done some study into this,... When I suggested to my consultant that maybe Hnpp had been 'partly' responsible for my hearing loss and also subsequent vertigo, (I know others with both Hnpp and menieres) I was given the impression that I was not being reasonable....ie a troublesome patient.

HMSN and SNHL

"Sensorineural Hearing Impairment in Patients with Pmp22 Duplication, Deletion, and Frameshift Mutations.
Otology & Neurotology. 26(3):405-414, May 2005.
Verhagen, W I. M *; Huygen, P L. M +; Gabreels-Festen, A A. W. M ++; Engelhart, M ++; van Mierlo, P J. W. B ++; van Engelen, B G. M ++"



woohoo! I havent lost my marbles after all!!!  well maybe :shock:
Tinnitus and deafness left ear 1981 onwards.
Diagnosed with meniere's in 1993.
Retired from work 1995.
HNPP - Hereditary Neuropathy with Pressure Palsy

Offline Elaine

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hmsn and hearing loss
« Reply #1 on: Sep 16 2005, 01:42 PM »
Hi Jon ,
I had been told that I might have lupus but couldn't find vertigo anywhere in the lupus symptoms list .My specialists refused to have vertigo as part of my main condition (still undiagnosed) and sais I was suffering from 2 separate conditions .Current rheumy and neuro say it is rare to have more than one condition and I have since found out about a condition called relapsing polychondritis which - you guessed it - has vertigo as a symptom as well as the achey bits which were thought to be lupus.Yay I'm not going mad after all  :D
I think the vertigo in my case is inflammatory of some sort as it responded well to steroids .Left ear has started revving up like an angry wasp again since before out hols and grommet is not working this time  :roll:
Sometimes we know more than the docs don't we?  :wink:
Elaine xx

Offline jon

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« Reply #2 on: Sep 16 2005, 03:37 PM »
Elaine,
There seems to be quite a lot of people with possible inflammatory conditions and menieres, or connective tissue and menieres, I'm sure that there will be a gradual recognition of these conditions, and their wide ranging effects, especially with the rapid rate of progress in the Bio-tech and medical fields. It is quite startling.
Similarly, there's some neurological link in some cases, and there are so many neurological conditions, and some very rare, that it takes along time for all this to become evident.
I suppose for some there will be no clues, just more frustration.
We have to be patient I suppose, it's not always easy, at times I want to stamp my feet and shout, not that that would help......I might feel a bit better for it though! :evil:  :evil:  :twisted:  :twisted:  :roll:


jon
Tinnitus and deafness left ear 1981 onwards.
Diagnosed with meniere's in 1993.
Retired from work 1995.
HNPP - Hereditary Neuropathy with Pressure Palsy

Offline Elaine

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hmsn and hearing loss
« Reply #3 on: Sep 16 2005, 03:48 PM »
Hi Jon,
I agree with you and I've done my share of foot stamping lately ,especially when the latest rheumy suggested somatization disorder (I'll leave you to look it up LOL!)  :twisted:  :twisted:  :twisted:  .Am awaiting the results of an MRI done on monday to see if there is any venal interruption or irregularity .I told the rheumy it would helpful if it showed up something and he looked at me like I was mad and said he would want it to be clear if it was him  :roll:
Speak soon ,
Elaine xx

barbara

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hmsn and hearing loss
« Reply #4 on: Sep 16 2005, 04:22 PM »
Hi Elaine,
It's diabolical that they go back to infering that it is all in your head.
I know what you mean about it being useful if things show up on tests. It is not that we want there to be something wrong. Just that we know there is but no one can identify the problem.  It is so frustrating.

Offline Elaine

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« Reply #5 on: Sep 16 2005, 04:29 PM »
Thanks Barbara! I hit the roof when I got the letter from him  :twisted:  :twisted:  .He's not sure I have Hughes Syndrome because my bloods aren't always positive and I haven't done the miscarriage thing but I have done lots of the neuro symptoms of it .We had an emergency meeting about it and I told him about the things I have tested positive for  (he was even suggesting the vertigo was in my mind till I said my ECoG test was distinctly abnormal !!!).He's now keeping a more open mind to APLS but is cross I am closed to the idea of somatization disorder - don't know anyone who would agree to it all being in their mind do you ???  :shock:  Am off to see Graham Hughes in January privately ,if not before then on the NHS .
Elaine xx

Offline Elaine

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« Reply #6 on: Sep 16 2005, 04:31 PM »
PS forgot to say the poor man did something nobody has dared to do so far - he waded through my entire file of notes at that hospital (all 7-8 inches thick of them !) and summarised them !!
Elaine x

barbara

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hmsn and hearing loss
« Reply #7 on: Sep 16 2005, 06:45 PM »
Hi Elaine,
I think it is a good idea that you are going to see G. Hughes. At least he should be able to say difinitively whether it is APLS or not. My bloods were on the whole negative but I did have six miscarriages between the two girls. Have discovered that Kiwi fruit, which prevents jelly from setting, is also a good blood thinner. I try to eat a couple of these a week rather than take aspirin.

Offline Elaine

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« Reply #8 on: Sep 16 2005, 07:31 PM »
Thanks Barbara! As you know I've been toying with the idea for a while now but trying to avoid it because of the trek to London .Now I've decided to do it I find out he is retiring in November and my PCT have been dragging their heels about funding it  :roll:  I've booked thw private appointment but am hoping to get the funding through before then so I can see him on the NHS - wonder if the PCT will fund private appointments??
I've been off the aspirin since last October but then I starting getting memory problems and other strange things so they put me on Plavix an antiplatelet which seems to be doing OK so far .
Glad your tests were negative .I didn't know that about kiwi fruits .
Elaine x

Offline jon

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hmsn and hearing loss
« Reply #9 on: Sep 21 2005, 10:22 AM »
Elaine, sorry to hear you're getting abuse from your doctors. re. the somatization disorder rubbish. I think that is a reflection of your doctors abusive personality rather than any problem on your part. I wish these medics would leave the psychiatry to the psychiatrists. And you dont need to have negative test results to fall foul of them either. It is really a value judgement on their part, and has no medical validity, doctors other than psychiatrists should not be making these judgements, they can adversely affect the patients care, for many years.


sorry for the rant! I've seen it from both sides, and it isnt pretty!

Jon.
Tinnitus and deafness left ear 1981 onwards.
Diagnosed with meniere's in 1993.
Retired from work 1995.
HNPP - Hereditary Neuropathy with Pressure Palsy

Offline Elaine

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hmsn and hearing loss
« Reply #10 on: Sep 21 2005, 10:51 AM »
Hi Jon,
Thanks for your understanding .I am due to see them again next month but hopefully will have my referral to London soon .I had an MRI venogram last week so the results of that should be back anyday soon .To be quite honest ,I have decided that if it would shut him up I would agree to any psych tests (which will come out negative because I'm fine apart from a bit of relic anxiety from years back!) just to rule it out  :roll: My ENT thought my facial pain was one of those facial pain disorders and it turned out to be an arthritic jaw!
Am off for a pre-op chat with the guy who's sorting out my nose this afternoon ,
Elaine xx

Offline jon

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Re: hmsn and hearing loss
« Reply #11 on: Nov 16 2005, 11:04 PM »
Over the last two years I've realised that 'it' is active again. In the last few weeks I've had several disabling episodes of vertigo. I think this is my right ear going the same way as the left. 10 years of gradual high frequency hearing loss, now affecting the mids and lows. No fluctuations as such just a gradual worsening.
It was at that point the vertigo really started, and the pattern seems to be repeating.

I've heard it said that the vertigo is not as bad the second time. I'm not sure about that, it still feels quite hellish, but it is different, maybe not so much spin as fall (zero grav effect), still go cold and clammy and feel sick as a parrot, so far cinnarizine (a trusted friend) has got there in time.

I am quite convinced of a link between SN deafness / vertigo and HNPP. At first I wasn't but with others similarly affected, and recent studies finding some link, it does look that way. Though it may be that it increases our liability to develop SNHL. Our, as in those with HNPP. Is it secondary hydrops, meniere's or just a demyelinating process, or combinations of all. I suppose I should see a Doctor to see if they are interested in finding out what is going on. Somehow though, I dont think they'll have the resources for that unless of course there happens to be a research program. Should I bother, is it worth a consultation?

I found this today, it looks very interesting, and I'm so curious to know how the research is going, I'm tempted to write to the Doc for info....
Molecular pathology of deafness due to mutation in PMP22

PMP22 is the gene that I have an under expression of.

Sorry, this is turning into a bit of a ramble, but its just about the only place on the web that I can talk about both problems........Thx Alec :)



 
Tinnitus and deafness left ear 1981 onwards.
Diagnosed with meniere's in 1993.
Retired from work 1995.
HNPP - Hereditary Neuropathy with Pressure Palsy

Offline Elaine

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Re: hmsn and hearing loss
« Reply #12 on: Nov 16 2005, 11:12 PM »
Hi Jon,
Sorry your worst fears are confirmed .My ears don't behave (or should that be misbehave?) the same .My spins on my left ear are less frequent but worse and affect my balance more than my hearing .The ones on my right ear which was my second ear to go active are more frequent and affect my hearing more than my balance but make me feel more sickly  ::)
If your cause is autoimune like mine would a blast of Pred help at all ? I've done 2 courses now and it's really calmed things down .I'm getting more "near normal" days now  :)
I would go for the consultation and ask about the research - nothing to lose eh ?
Elaine xx

Offline jon

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Re: hmsn and hearing loss
« Reply #13 on: Nov 17 2005, 01:22 PM »
Not sure about the steroids. They do get used for CIDP sometimes, as that is an autoimmune inflammatory neuropathy (aquired). There are some similarities to the hereditary neuropathy, so maybe steroids may help. I'll find out soon enough. I've had breathing troubles for a few years now, smoking was the biggest cause, but it seems the Docs think I may have asthma, I'm on 4hourly peak flows, and depending on those results will be on either puffers or oral steroids.

It will b interesting to see what effect they have.

I dont hold out much hope of success, I'm fairly sure that my breathing problems are due to a weakened diaphragm, or rather poor phrenic nerve conduction. We'll see. Hope for the best. ie the steroids work.

Jon
Tinnitus and deafness left ear 1981 onwards.
Diagnosed with meniere's in 1993.
Retired from work 1995.
HNPP - Hereditary Neuropathy with Pressure Palsy

Offline jon

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Re: hmsn and hearing loss
« Reply #14 on: Nov 23 2005, 12:29 AM »
Bish!! Looks like I'm going to be on the oral steroids for 2 weeks. And Inhalers regularly. They think it's asthma, I hope so, if it doesnt clear up it will most likely be neuromuscular, and I dont think there's any treatment.

My ears have been unsettled recently, it will be interesting to see if the steroids have any effect on that. It would be a rare and welcome side-effect for a change.

j0n  -  Tired and apprehensive
Tinnitus and deafness left ear 1981 onwards.
Diagnosed with meniere's in 1993.
Retired from work 1995.
HNPP - Hereditary Neuropathy with Pressure Palsy

Offline jan

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Re: hmsn and hearing loss
« Reply #15 on: Nov 23 2005, 09:26 AM »
hi jon,hope the steriods have a good effect for you
                                           jan
keep smiling!!

Offline Elaine

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Re: hmsn and hearing loss
« Reply #16 on: Nov 23 2005, 10:21 AM »
HI Jon,
Are you going on prednisolone? if so make sure you taper off it .I did a quick blast first time with sudden stop and suffered really weird " withdrawal " symptoms ! Doing the taper was great .The worst side effect was being very sleepless as it all caught me up once I'd stopped and then all I wanted to do was sleep  ::) Otherwise my energy levels were great and it worked well for me .
Hope it helps you ,
Elaine xx

Offline jon

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Re: hmsn and hearing loss
« Reply #17 on: Nov 23 2005, 12:42 PM »
Thnks Jan, I hope so too, but really have my doubts. Trying hard to be positive about this, but it isnt easy.

Elaine, I think its going to be prednisolone, I pick up the script tomorrow.


Jon.

Tinnitus and deafness left ear 1981 onwards.
Diagnosed with meniere's in 1993.
Retired from work 1995.
HNPP - Hereditary Neuropathy with Pressure Palsy

Offline Elaine

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Re: hmsn and hearing loss
« Reply #18 on: Nov 23 2005, 02:51 PM »
Think positive Jon ! I was nervous too because of all the tales I'd heard on various groups but I was fine .Let's hope it works for you ,
Elaine xx

Offline jon

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Re: hmsn and hearing loss
« Reply #19 on: Nov 27 2005, 02:00 PM »
Aye, I'm on 30 mg/day for 14 days. Day3 so far. I'm actually feeling worse. No improvement in 'suspected' asthma, and I'm losing my voice on occasions. This does happen to me on occasions so it's prolly coincidence. Just an unwelcome one. Vocal cord dysfunction is a known asthma mimic, so I've just found out. Vocal cord palsies are not unheard of in HNPP, and I have a history of them. Hmmm??

Also feeeling quite dizzy today, though I think this continued breathlessness maybe to blame to some degree.

Now I'm begining to sound like Marvin the depressed android.!! I wish there was a smiley with an exploding head

Right, I'd better start thinking about getting the roast dinner on the go.

j0n

Tinnitus and deafness left ear 1981 onwards.
Diagnosed with meniere's in 1993.
Retired from work 1995.
HNPP - Hereditary Neuropathy with Pressure Palsy

Offline Elaine

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Re: hmsn and hearing loss
« Reply #20 on: Nov 27 2005, 02:17 PM »
Hi Jon,
3rd day is still early days to tell if it is working or not .I did notice something when I first ran it but not when I had the second course .In fact second time around I didn't notice anything till after I'd tapered off really  ::)
Keep an eye on the shortness of breath as it can be a side effect of the pred itself - gawd how do we work out if it's the disease or the meds ! ;)
Elaine xx

Offline jon

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Re: hmsn and hearing loss
« Reply #21 on: Mar 07 2006, 02:46 PM »
Well I finished the steroids which had no effect on the 'asthma', At the resp. nurses request I've kept up with inhalers, still no effect. Well no effect on breathing but ventolin does provoke a bad ear reaction if my ears are active. Which they are.

I've had a strong return of vertigo, sveral flare ups a week. My balance is completely zapped because of the motor and sensory losses in my legs and feet. So life in betweeen episodes is tricky to say the least. In fact Ithink the whole neuropathy thing has taken a step up in gear. My arms are worse than ever, lots of numb patches and parts of my body not working from time to time. I will get quite dizzy and off balance which seems to be triggered by sensory goofiness.

I see a neurologist on friday, yearly out-patients appointment, I'm hoping I can get to discuss a few of these things. I havent seen this chap before, he's apparently the MS expert in the Bristol area, so hopefully he'll be open to sensory goofiness triggering vestibular symptoms. But then again maybe not. Best not to have too many expectations.

I've also found out that the severe vomiting caused by vertigo really upsets my chest and abdominal muscles, and it has just occured to me that it might be one reason for the HMSN affecting my intercostal nerves and muscles. It really gives them a good yank and stretch, which my nerves have a particular weakness for. I know one other with HNPP and menieres syndrome, and they have rib troubles as well, so it might not be such a totally barking mad idea afterall. I bet the doc doesn't agree.

CMT UK are trying to get HNPP included in their literature, this will require a Doc to review the evidence, I am hopeful. It will be the first written info (for public comsumption) in the UK.

bye for now,
jon

Tinnitus and deafness left ear 1981 onwards.
Diagnosed with meniere's in 1993.
Retired from work 1995.
HNPP - Hereditary Neuropathy with Pressure Palsy

Offline Christina

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Re: hmsn and hearing loss
« Reply #22 on: Mar 07 2006, 03:06 PM »
Hi Jon

It sounds like you're having a really bad time at the moment, I hope things start to balance (sorry for the pun!) soon. Let us know what the neurologist says.
Best wishes
Chris
not waiting for the storm to pass, but learning to dance in the rain ...

Offline latou

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Re: hmsn and hearing loss
« Reply #23 on: Mar 07 2006, 05:05 PM »
Jon, this is all a real bummer isn't it?   Hang in there, buddy and let's hope Himself is helpful on Friday.
Linda

www.latou.co.uk

Offline jon

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Re: hmsn and hearing loss
« Reply #24 on: Mar 07 2006, 05:34 PM »
Thanks chris, Linda,
 the neuro appt. should be interesting if I'm still spinning, they've never seen me during an active phase before. Hope I dont chuck!

jon


Tinnitus and deafness left ear 1981 onwards.
Diagnosed with meniere's in 1993.
Retired from work 1995.
HNPP - Hereditary Neuropathy with Pressure Palsy

Offline latou

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Re: hmsn and hearing loss
« Reply #25 on: Mar 07 2006, 08:55 PM »
Take a plastic bag with you just in case.   It's as well for them to see the full array of delights you have to cope with.
Linda

www.latou.co.uk

Offline Elaine

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Re: hmsn and hearing loss
« Reply #26 on: Mar 07 2006, 11:44 PM »
Hi Jon,
Sorry you're having such a rough time .Ihope yoru appointment goes ok and is helpful ,
Elaine xx

Offline jan

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Re: hmsn and hearing loss
« Reply #27 on: Mar 08 2006, 10:22 AM »
hi jon,sorry you are having a rough time.hope your appt goes well,and you find him helpful.
                   take care                           jan xx
keep smiling!!

Offline jon

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Re: hmsn and hearing loss
« Reply #28 on: Mar 08 2006, 07:29 PM »
Thx Jan, elaine,

the good news is that I have landed today.  so I'm much better, clearer head. But I have a back ache from hell. Still thats better than the vertigo, I really cant cope with vertigo at all.  I've had a few jolts today but nothing sustained. phew, what a relief.....

thanks all for the good wishes for Friday


jon
Tinnitus and deafness left ear 1981 onwards.
Diagnosed with meniere's in 1993.
Retired from work 1995.
HNPP - Hereditary Neuropathy with Pressure Palsy

Offline jon

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Re: hmsn and hearing loss
« Reply #29 on: Mar 10 2006, 10:02 PM »
Well he didnt want to know about my ear and vertigo at all, some one else's territory.

Even funnier, my problems arent Hnpp they are postural.....lol.....well at least they're not all in my Head.!!

One step forward two steps back.....and round and round the mullberry bush we go...whoopeee!!!!



Jon
Tinnitus and deafness left ear 1981 onwards.
Diagnosed with meniere's in 1993.
Retired from work 1995.
HNPP - Hereditary Neuropathy with Pressure Palsy

Offline Christina

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Re: hmsn and hearing loss
« Reply #30 on: Mar 11 2006, 02:31 PM »
Sorry it was such a frustrating visit to the neuro Jon.  Doesn't it make you want to spit?
Chris
not waiting for the storm to pass, but learning to dance in the rain ...

Offline jon

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Re: hmsn and hearing loss
« Reply #31 on: Mar 11 2006, 10:47 PM »
Hi chris,
I've had time to think things over, there were a few positives. I have to take part in physio pain management course, to correct my posture. I don't think it is going to be that easy, because of HNPP but that is something they will have to learn. I'm sure I'll learn aplenty, and I might be surprised if they can correct my posture.
As for vertigo and deafness, I'll just have to adapt, nowt else that can be done. I'm not going to bother to see the ENT bods, it always seems a waste of time.
I dont think I'll bother going back to the neuro after next year, again there is nothing they can do. I'll just see my GP for any problems I have.

Are you still seeing a neurologist, or has that come to an end, or are you still waiting for an appointment..

Jon
Tinnitus and deafness left ear 1981 onwards.
Diagnosed with meniere's in 1993.
Retired from work 1995.
HNPP - Hereditary Neuropathy with Pressure Palsy

Offline Christina

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Re: hmsn and hearing loss
« Reply #32 on: Mar 12 2006, 11:24 AM »
Hi Jon
I have a follow up appt on 2 April - that's about a year since my first appt, so I'll let you know what he says.
Chris
not waiting for the storm to pass, but learning to dance in the rain ...

Offline jon

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Re: hmsn and hearing loss
« Reply #33 on: Mar 12 2006, 07:16 PM »
Good luck chris

Tinnitus and deafness left ear 1981 onwards.
Diagnosed with meniere's in 1993.
Retired from work 1995.
HNPP - Hereditary Neuropathy with Pressure Palsy