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Chiari malformation and superior canal dehisence

Hi, I have had a horrible 6 years medically. I was diagnosed in 2007 as having celiacs disease and in 2009 as having a rare disorder called superior canal dehisence syndrome (SCDS) - google it if you haven't heard about it before - anyway some of the symptoms were dizziness, fullness in ear, unable to tolerate noises, hearing your own heartbeat or eyes blink etc. I live in Australia and in the state I live in I was the first person to be diagnosed with the condition (the condition was only discovered in 1998 by Lloyd Minor) and my operation to fix the piece of bone which covers the top of my right semicircular canal in my ear was done using a neurosurgeon to do the craniotomy and retract my brain so the the ENT could have access to the area that needed to be fixed. This was the first time the ENT had performed this operation but the neurosurgeon was very experienced. What happened next was while I was still in hospital recuperating I noticed my left big toe become sort of tingling and numb. The neurosurgeon was contacted and as the tingling and numbness didn't get any worse he released me from hospital the next day. Over the next 4 months the numbness, tingling, aching and burning sensations (nerve pain) gradually moved up my leg and to my hand and arm and occasionally my face. My GP referred me to a neurologist immediately. This was majorly left sided but occasionally I would get weird sensations on my right side that wouldn't last long. I was still dizzy most of the time and I developed a wide gait when walking, I also had/have memory problems and trouble finding words (which is quite distressing as at the time I was a professional management consultant and had to give up that profession as I was petrified that I would be in a meeting with corporate executives and have a blank out with words or not remember people's names or information I was gathering from meetings.) Eventually the pain in my leg and arm and hand became so bad I had to start taking seizure medication along with a very old anti depression medication used to treat nerve pain. The problem with that is that while the medications controls most of the pain (some breakthrough pain) I am like a walking zombie who spends most of her time resting or sleeping so I am energised enough to look after my 3 boys when they weren't at school. I was unable to work as a consultant but battled on trying to teach casually at primary schools (I have an education degree as well as a business degree) but eventually after 2 years I could not sustain that work. My neurologist (and about another 3 he sent me to for their opinion) didn't know what was wrong with me despite having multiple MRI's to see anything (I think they were looking for lesions to rule In Or out multiple sclerosis) - my neurologist knew there was something wrong with me due to abnormal responses to neurological tests he would do every time I had an appointment with him.  Soooooo yesterday I was watching a show on TV which was about rare conditions and chiari malformation was one of them. I was interested to know more so I did some 'Google medicine research' and found out that people who have superior canal dehisence are 23% more likely to also have a CM1. That intrigued me more so I looked at several web pages to learn more. Basically because of my Superior canal dehisence, it means that the thickness of my skull is around 60% thickness of what a 'normal ' persons skull would be. I have just undergone brain surgery 5 weeks ago as I have trigeminal neuralgia since January 2014 - yes I must have been a bad person in a previous life to have such rotten health all at once after being perfectly healthy till I was 39 - and I was on more epilepsy medication for that as well from my pain specialist, until my neurologist ordered me to have another MRI to focus in on where the trigeminal nerve is at the base of my brain and it showed an artery had wrapped itself around my trigeminal nerve which over time had worn away the myelin sheath that protects the trigeminal nerve which is why my left side of my face was being bombarded with electric type shocks and feelings of an ice pick digging through my cheek into my teeth (they call trigeminal neuralgia the 'suicide disease' as those unfortunáte people who never get diagnosed and treated usually take their own lives the pain is so debilitating.) So far after 5 weeks I am pain free in my face but the surgery could last as long as 10 years or as little as a few months - I'm praying for the 10 years before I may have to have it again. I still suffer from the nerve pain, wide gait, being unbalanced and terrible memory and loss of words from my superior canal dehisence surgery 6 years ago. Soooooo after all of this waffling I guess my question is 'has anybody else with Chiari Malformation also had superior canal dehisence?' I have sent a few documents for my neurologist to read to see what he thinks as I do have a lot of the symptoms of CM1.  I sent my neurologist a few months ago, some documents about retracting the brain during surgery and in 10% of cases it leading to damage of the brain or brain stem injury, as when I saw my new neurosurgeon who did my trigeminal neuralgia surgery he said he believed my left sided problems I am having was probably due to brain retraction causing damage to my brain stem by being retracted too far. When my neurologist received the documents he emailed me back to say thank you as he was unaware that brain retraction during surgery could cause damage. Thanks for reading my looooong post - hopefully a solid answer will be found for my predominantly left sided problems as I have been battling with my income protection insurers for 3 years as I am no longer able to work and have been diagnosed by my neurologist and my GP of 13 years as being TPD (totally and permanently disabled) which my different superannuation institutions haven't questioned and have paid out my superannuation but I've had to hire solicitors to keep battling with my income protection insurers who just hope if they draw out a case long enough that the person who is insured (me) will not be able to afford keep paying their monthly fee (yes I pay them $300 a month even though I have an ongoing claim with them.) So I'm hoping in a way that if it is a chiari malformation this will give my insurers no more excuses not to pay out my income protection as they will be able to "SEE" the problem in an MRI. I am also desperate to get some of my quality of life back (even if some of my problems would still be permanent if it was a CM1) at least I could have some closure and get on with being as healthy as I can under my circumstances, because I hate not knowing what the cause of my predominantly left  sided problems are.  Regards Linda
1 Responses
620923 tn?1452915648

  Hi and welcome to the Chiari forum,

Your DX  of SCD does have many of the same symptoms of Chiari.....and there are even more conditions that share those symptoms along with Chiari....MS, lymes, lupus....

Chiari is often times over looked as a incidental finding....it is possible many of us do have SCD but it is not been the focus as to the cause of our symptoms.....once Chiari is found and you have a Dr well informed and experienced with Chiari they tend to rule out ALL related conditions.

I do not know if you are able to get copies of your MRI to send to Drs for review.....or even your OR report to know for sure if your brain was retracted too far.....but it may help give answers as to if you do have Chiari.

My question is what part of the brain was retracted?If it was the cerebellum and it was herniated already, pulling it up and stuffing it into a too small compartment ( skull malformation that denotes Chiari is present)

I do hope you are able to get answers.....I am adding a link to a very small list of Drs from Australia for you to ask more questions to-
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620923 tn?1452915648
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