Migraines, Trigeminal Neuralgia, Vestibular & Auditory symptoms and HNPP

This blog post, is incomplete, I should really check for references and the veracity of some of my comments and inclusions… with these caveats in mind… please continue to read…  This isn’t a whinge or moan, and those that like to beat up on those who talk about health problems can hop off now and play with their  prejudice elsewhere.

I was looking at some posts on an HNPP group the other day, and a question came up regarding links between migraine and HNPP and bouts of persistent facial pain. The poster was asking whether migraines were directly linked to HNPP, ie caused by the PMP22 deletion or some of the other deleted genes in the deleted region on Chromosome17.

I didn’t think that there was a direct link, although of course not all the genetic causes of migraine have been found, but those that have appear to involve genes on other chromosomes other than 17. These genetic variants tend to be calcium channelopathies. Whilst I’m sure I don’t have a known genetic variant, ie familial hemeplegic migraine, it’s intersting to note that the medication that I find very helpful in reducing the effects of migraine, is a calcium channel blocker amongst other effects such as anti-histamine, and some actions attributable to it’s relationship to the major tranquillizers. The drug BTW is Cinnarizine, and was originally prescribed for me as a vestibular sedative with effects on the micro-circulation of the inner ear, which I believe is also due to calcium channel blocking.

I digress, or perhaps I just witter!

Anyway, Before I was diagnosed with HNPP, I was diagnosed as having Meniere’s Disease, or at least I had three of the four classic symptoms of Meniere’s disease, namely, sensori-neural hearing loss, tinnitus and bouts of rotational vertigo (severe dizziness). I didn’t think that there were any links between the two diseases, but I was quite surprised to find that Meniere’s Disease was far more common in the small group of people with HNPP than would have been expected. It could of course be just coincidence or misdiagnosis, but as Meniere’s Disease is supposed to be relatively rare (2 to 5 per 10,000) I found it surprising that there were approx 3-5% of people with HNPP that had meniere’s disease. BTW the incidence of HNPP is approx. 2-5 per 100,000.

Another curious thing is that at least one of these people who has HNPP and Meniere’s disease, also suffers from Trigeminal Neuralgia, and perhaps the linch pin in all this, Migraines.  The chance of this happening by coincidence must be extremely small, which doesn’t mean that it isn’t but surely begs the question, is there a causal relationship?

I was diagnosed with Migraine when I was in my teens, but it was also noted that there was an element of neck pain and, as the neurologist at the time mentioned, occipital nerve impingment due to weak and over-tightening neck muscles. My Teenage years were also the time when HNPP started to become more pronounced than just numb finger tips when carrying shopping bags, and numb thumb and forefinger when using scissors. I started to experience ulnar nerve involvement, numbness of little and third fingers which seemed to just come out of nowhere, and sudden episodes of foot and lower leg weakness, not to forget the ever increasing episodes dead arms and legs. I once lost all power in one arm after a minor bicycle accident that took nearly 9 months to fully resolve.

A few years later, aged 21, I awoke with what I perceived as high tone hearing loss (HL) and unbearable tinnitus. I also experienced intense facial pain for three or four months with this HL and tinnitus. My GPs were unsympathetic, and assumed it was just a viral illness, and after repeated visits totally dismissed the symptoms as fictitious. Migraine’s were also troublesome but less so than earlier in my life. I remember all this very well as I was training to be a psychiatric nurse, and my ill health was getting me into hot water with the management. But unfortunately without support from my GPs for my unusual symptoms, this became a very unhappy period of my life.

I really have digressed or wittered far off the beaten track….

Back to Migraine and Trigeminal neuralgia. A few years ago the migraines stepped up in frequency, and this time I had the classic tell tale signs of scintillating scotomas during the aura phase. Sometimes the auras became persistent with visual snow, blimps and bright spots (scotomas) and impression that I could see ‘space’, like a multi-faceted mesh. Also extreme ataxia and balance disturbance with feelings of depersonalisation (sorry it’s the psych nurse lingo coming out). This bore some resemblance to at least some of the episodes of vertigo I experienced during the very active phase of Meniere’s disease, but perhaps that is just similarity rather than causality.

Then the bouts of TN started, though on relflection maybe I had had this before, and my reading of the new info (bearing in mind I hadn’t read much about migraine for some 30 years), led me to think whether there was some mechanism by which migraine, through it’s involvement of the T.nerve, was triggering bouts of Trigeminal neuralgia. But is the TN I suffer from due in part to the HNPP, as HNPP is a demyelinating peripheral neuropathy, which has been reported as causing TN in some rare cases.

So consulting Dr. Google with extreme caution as always, I started probing for possible links, and I was stunned to find this article which takes it’s info from the book, “Striking Back: The Trigeminal Neuralgia Handbook”, pp. 113-115.


(Unfortunately the above link is no longer active, a google search for “Striking Back: The Trigeminal Neuralgia Handbook”, pp. 113-115 , reveals the following link and a few others.


Some quotes from the article
“For some migraine headache sufferers, there is a risk for sustaining permanent cranial nerve damage. When this type of complications persists, there can be long term health complications aside from the chronic migraine pain…..”

“One of the most common long term nerve complications seen in migraine headache sufferers involves a condition known as trigeminal neuralgia.”

Caveat: Without access to the book I cannot vouch for the accuracy of this articles interpretation, or the references from which the book asserts this link.

If this is the case, and TN is seen as a rare complication for some migraine sufferers, then I would think that having HNPP actually increases this risk. The T.Nerve is inherently affected by this disease i.e. all peripheral spinal nerves including at least 10 of the 12 cranial nerves (omitting the olfactory and optic nerves which are thought to be CNS tracts rather than belonging to the PNS) have a deficit of the PMP22 protein which normally helps to bind together the layers of PNS compact myelin.

It is also interesting to see that over the years Doctors and researchers are begining to narrow down the potential causes of Meniere’s disease, there are quite a few different flavours of the disease, some thought to be due to inner ear fluid imbalance, others due to trauma, nerve compressions, perilymph fistulae, auto-immune disease, and of interest to me, Migraine.

Migraine associated vertigo (MAV) has now been well established as one cause of vertigo and tinnitus, it was thought not to cause hearing loss, although recent studies (as reported by a long time expert into vertigo) do seem to suggest that hearing loss is possible in some cases of MAV, possibly due to ischaemia or changes in blood flow during the attacks of migraine. I have yet to find confirmation of this so yet this should be treated with caution.

I must add link and references to this… but I wanted to get this train of thought written down, so that it doesn’t just pass me by and forgotten, though I don’t think I’d ever be able to forget, due to the many symptomatic reminders.


14 thoughts on “Migraines, Trigeminal Neuralgia, Vestibular & Auditory symptoms and HNPP

  1. Good read. Although I do not think I have what you have, I was lead here from the Key words Visual snow. I suffer from something called Palinopsia where I see after images. This started after daily headaches/Migraines. I am trying find a cure for myself….. which doesn’t seem to be in reach. I also get the TN I believe. The shooting pains in the side of the head. I also have severe scalp sensitivity. I am being pushed around from dr. to dr. with no answer.

    Best of luck to you,

  2. Hi Candi, good luck with your search for treatment. Being bounced from Dr. to Dr. is pretty unpleasant, continuity of care suffers.
    Have you seen the migraine aura website, I found it very useful, and reassuring to know that others experience similar symptoms. I do find some of the migraine art difficult to look at without it triggering, if I am in an active phase. But without that website I would have found it difficult to understand my own symptoms.

  3. Reading your story prompted me to share the following experience with you. About 6 mos ago I had what, on the surface, appeared to be a minor stroke or sudden onset of Meniere’s disease–vertigo, nausea, immobility, excessive pressure in my ears/temporary deafness–keeping me in the hospital for 4 days. After several blood tests, neurological exams and an MRI, my doctors concluded that I’d had a serious sinus infection and treated me with a steroid and 2 grams of valacyclovir daily, since the only virus I’d ever had before was herpes simplex I, and they could find no other explanation/etiology for the infection. After about 1 month I felt better but sustained hearing loss within a certain range in my left ear. My audiologist suggested to me that since HS1 resides on the trigeminal nerve, its swelling during the sinus infection may have caused pressure on both the vestibulochoclear and facial nerves, since I had (initially serious) decreased sensation in my face during this time. If you are interested in reading about HSVs and how it may affect your own illness, and you would like to visit an excellent online site, here is the link: http://pathmicro.med.sc.edu/virol/herpes.htm. Best of luck to you. I do hope you feel better every day. ~a reader

  4. Hi Diane, Thanks for the link. Many times in the past I have felt that viral infections were partially responsible for my hearing loss and vertigo, way before I was diagnosed with Meniere’s disease. In fact it was after a fairly hefty bout of flu that I noticed that I had lost all my hearing in one ear, I then had fairly severe attacks of vertigo often lasting 24-36 hours.
    Two years before that I had what was thought to be glandular fever, epstein-barr, never quite sure what it was but I did not feel fully well for nearly a year. I had several attacks of rotational vertigo and drop attacks during that recovery phase.
    Thanks again for commenting,

  5. Finally, all these years of suffering with Tinnitus and the symptoms of Trigeminal Neuralgia I’ve come to the realization that this all started with sinus surgery gone wrong in 1984!
    Has anyone experienced an increase in the intensity of Tinnitus after being exposed to very loud noises, like from a commercial grade interior fire alarm?
    Any shared experience will be warmly received.
    thank you

    • It could well be Neal. A few years before tinnitus became a constant problem for me, I was working as the wood stacker on the back end of a moulder. The noisiest machine that I have ever had to work near. And all without ear defenders, this was the 1970s. My left ear bore the brunt of that noise, and it was my left ear that failed first.
      My son has started to experience bouts of tinnitus, and he plays bass guitar. They always play loud, and despite using musician grade ear plugs, it is damaging his hearing. He is 19 years old.

  6. Hi, thank you for posting this…

    I have been diagnosed with HNPP, which, has been quite an annoying part of my life…I have accepted that I cannot do many of the things I used to love doing, and my husband and I have even considered using donated eggs to not pass these gene mutation on.
    The part that concerns me the most is that, starting January 2010, I developed headaches that the doctors now call ” new daily persistent headache”. They have done most life threatening tests ( MRI, CT, Lumbar punctures…) so they are not concerned I am in any serious threat.
    In July of 2011 my left foot dropped. This is common in HNPP patients…. Now, in October 2011, I find myself in constant pain, and the headache of January 2010 has not left. It spikes every two weeks or so, this in turn has left me off work and unable to eat as the nausea gets so bad, eating becomes impossible.

    I’m starting to feel a tad crazy as seem to be the one who can never go out, have people over or travel at all…. I feel as if I do not live…I just watch the clock from one day to the next, hoping that the next day will be a bit better than the day before.

    I would love more info on this as dr. Google does have a lot to say….

    Very frustrated….

    • Hi Katie, sorry it’s taken me so long to reply, last week was quite a busy week for me, one way or another. It’s hard to know if HNPP is involved with cranial neuralgias, I certainly have some definite problems which seem to fit that pattern, but it hasn’t been confirmed, and I don’t know how it could be confirmed. I have heard of others with similar problems so it’s possible that it is just one of the many presentations of HNPP, but then again it could be just coincidence again. With HNPP being fairly rare and even rarer that it gets successfully diagnosed, I can’t see this situation changing.
      Trigeminal neuralgia or trigeminal neuropathy, is also quite rare, and seems to be poorly understood, with numerous ideas of the causative processes. I sometimes feel that no matter what the cause, treatment of symptoms is the most important thing. This can be difficult, if the medical profession can’t decide on cause, but when all known problems are discounted and all that’s left is persistent neuralgia or neuropathic pain, then that needs to be treated. I would imagine that some of the surgical treatments and procedures would be ruled out because of the risk of causing further problems with HNPP, so that mostly leaves medical treatment. Most of the drugs used to treat Trigeminal neuralgia/neuropathy, and also migraine, are the same for generalised neuropathic pain, so there is some hope that pain relief can be achieved.
      Finding the right medication can be very difficult, there’s such a large trade off between beneficial effect and side effects, it can take a lot of trial and error to find the right medication for you, and that takes time, and often as not time is a premium due to family and employment commitments.
      One thing is for certain is that you’re not alone, there does seem to be quite a number of people with HNPP with these other or additional problems. Hopefully it won’t be long before the medical profession start to recognise that HNPP can be more than just a few painless palsies in a lifetime.
      Are you a member of the yahoo HNPP group or the facebook HNPP (Hereditary neuropathy and CMT) group. You may find that there are others there who share similar problems, finding treatments may still remain elusive but at least you’ll be able to share your experiences.
      tta for now

    • frustrated too. I’ve seen more than a dozen dr’s, ENT, Neurologist, Neuro surgeons, pain specialists, & Migraine specialists over the past couple of years. No one has been able to help me. The three neurologists all contridicted themselves–with most lacking in empathy and almost being very critical of me.

      Been diagnosed with Meniere’s Disease (shunt, vest. nerve section, & laby surgeries); very acute Tinnitus (pulsates with eye movements); migraines (daily); Trigeminal Neuralgia (all three branches)–now spreading to other side of face (mimicking my pain). profound deafness (left ear). I’ve been found disabled for 9 things after filing for disability. Lost 60k when I just couldn’t work any longer.

      Life is hard, but it’s only temporary. I’ve really enjoyed getting to know my wife and 4 children much better over the last year. I’m about to get a companion dog (labradoodle) to improve my emotional state and assist with exercise. I need someone to be with me when I have VERY, VERY, DARK days. When everything hits me all at once and is an 8-10+… I don’t know how many more days I can take that. I’m 44 years old and this past ytear has felt like 5+ years.

      I really hope all of you can find some answers. I’m trying my best to deal with my body thru various meds and am learning to accept and live with the pain. I never thought this would be me after working as a sportscaster on TV for several years and a decade 1/2 in public relations.

      Whenever I start complaining… all I have to do is think of my sister who has taken chemo every week for the last 5 years and is still surviving. Doctors gave her months to live (inflammatory breast cancer). Because of her situation my wife got tested in her late 30’s and was found with breast cancer. She had a double mastectomy at the start of the school year and was back to work a month and 1/2 later.

      I have so MUCH RESPECT & LOVE FOR MY WIFE. We love each other unconditionally. She’s the kindest person I know. It’s no wonder she’s the most requested teacher at her school. I’m one of th luckiest people alive. There is so much love in our home with all the trials we’ve been thru. My favorite part of the day is seeing people when they come home. I’m like a little puppy dog.

      Many God bless everyone with improved health and spirits.
      …..Just thought I’d share.

  7. Very interesting to read all of this! There MUST be link to all of this.

    Here’s my story (in short):
    At the age of eighteen, I’ve had an sinusinfection (forehead). It took me several years to get rid of, that is, it feels like it moved to my left middle ear.When I was about 21, I’ve had a sort of meniere-stroke, only I didn’t know it then.
    I’ve had middle-ear problems since the time after the sinus-infection, and I’m now 46 years old…
    A year ago things went seriously wrong when I was hospitalized with an attack of menieres in combination with Trigeminas neuralgia.

    Short list of my problems:
    – Eustacian tube that’s blocked most of the time.
    – I’ve had chicken pocks for the first time at the age of 40 (!)
    – attacks of meniere’s, started seriously a year ago, at the same time I got
    – Trigeminas neuralgia (hospitalized for a few days, after months (!, couldn’t eat, speak, etc) started with Carbamazepine wich is very helpfull, hallelujah!)
    – Migraines, which I have for many years and seems to be related to my cycles, so is the meniers by the way.

    I’m having about 1 or 2 meniere-attacks a week, take Betahistine, which is helpfull in the way that the attacks are more bareable.

    I know for sure that it’s no coinsedence to have menieres and TN at the same time, however that’s exactly what the docters told me.

    Sorry for my use of language, tried my best 😉

    Ellis, Netherlands, Europe.

  8. Read all your comments and I definitely think there is some kind of relationship. Was diagnosed with HNPP at age 65 (last year) which finally put a concept behind all my awful, Dr. denied symptoms.(all of my dr.’s have never heard of HNPP except my neurologist). Also have a left side consistent/reappearing stabbing headache in my skull Have sinusitis which if I am not careful (regularly take sinus nose spray and allergy medication) turns into a sinus infection and an ocular migraine. It seems to me that I have a pressure palsy in my left sinus passage which feels like a very strange numbness that goes away after a while, but always comes back. Have had herpes simplex I as long as I can remember which manifests itself through cancer sores/fever blisters that often can be stopped by taking lysine in time. Apparently it is my love for nuts that overwhelm my body with arginine? I am gluten and dairy intolerant and tinnitus. I so wish that someone could finally come up with an explanation for all of this .

    • Unfortunately HNPP seems to be poorly understood even today. Many doctors hold to the opinion that it is a painless condition. That is notmy experience, nor is it that of the large number of people who are members of the various HNPP groups. It is a common complaint, ie doctors don’t listen, don’t accept pain as part of the whole HNPP ecxperience, and are generally dismissive and appear to have little time for us.
      As for the links with other nerve syndromes, although there might not be a direct relationship, however because HNPP is so easily aggravated by simple trauma, which can include the effects of other diseases, HNPP can easily become a complication. I am very curious as to whether migraine (unrelated to HNPP) might be a contributary factor in the development of trigeminal nerve pain, this seems a rare association except within our HNPP groups, where it seems not uncommon. So many questions…

  9. Hi, Thanks for the article. I stumbled across it while looking for a link to MAV and trigeminal nerve disorder. Surprisingly, there is nothing about this on any of the migraine disorder websites, but clearly there is enough anecdotal evidence and at least one published source to prove there is. I am relieved to have an answer. I will look for the book you mention. Thanks again and good luck to you!

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out /  Change )

Google+ photo

You are commenting using your Google+ account. Log Out /  Change )

Twitter picture

You are commenting using your Twitter account. Log Out /  Change )

Facebook photo

You are commenting using your Facebook account. Log Out /  Change )


Connecting to %s