The other day I had a follow up appointment with the ENT, following my VEMP testt last month. It would seem that the VEMP test backs up the findings of the CT scan, and confirms the diagnosis of Superior Semi circular canal dehiscence. It also suggests that I am bilateral, ie have a hole in the canal in both ears.
This all means that they consider me a candidate for surgery. In the UK that pretty much exclusively means that surgery will be done by the Trans-Mastoid approach, and the hole itself will either be patched or plugged. Let me explain this a little further.
There are two surgical techniques that are used to fix SCDS. One approach is Mid-fossa Craniotomy, which is a very invasive procedure, which involves making an incision through the skull and coming at the hole from the top down. The plus side of this is that they can see the hole, the downside is that due to the invasive nature there are extra risks involved.
The second approach is the Trans Mastoid approach, where they come into the inner ear through the mastoid bone behind your ear. This is less invasive, it does not require having to lift the brain, and therefore carries less risk. But the downside is that they cannot visualise the dehiscence. The Transmastoid approach is the technique that is used in the UK. The US uses both techniques.
Once they have gained access to the inner ear, there are two ways of fixing it. They can either put a patch over the hole, or plug the entire canal. Putting a patch over the hole would maintain the function of the canal, but it is not as durable as plugging, and if it is not in the right place, then the surgery will fail. Plugging the canal, is where they fill the canal with a sealing substance, and therefore seals the hole. But by plugging the canal you lose the function of the canal, which could affect balance. However, with Vestibular therapy, it is hoped that they brain learns to compensate for that loss.
As you can see, it is not straightforward, and there will be various decisions to be made. The first decision for me, was getting a surgeon who had done this procedure before. As nice my ENT consultant was, he admitted he hadn't done this surgery as a consultant before, and I really didn't fancy being the first. So I am being referred to a consultant who has a few of these surgeries under his belt. The problem with having a rare condition that has only been known about for the last 20 years, is that even the experts are still learning about the condition, and not that many surgeries (in the scale of things) have been undertaken. Techniques are being improved upon all the time, and the longevity of the surgeries is still being discovered.
That being said, I have a condition that has a chance of being fixed. I have a chance of getting my pre-SCDS life back. So I await surgery.
Sunday 20 December 2015
Friday 27 November 2015
More tests
I realise that I need to bring you up to date.
I went to see the different ENT at the end of October, and this was the first time I felt that I was talking to a medical person who knew anything about this condition. I was in a room with the ENT, a physio, and various other nurses who between them all made up the balance clinic. Unfortunately the ENT didn't have access to the reformatted version of my CT scan, but he later wrote me a letter saying that once he had seen it, it became clear that I "had quite a good example of superior semi-circular canal dehiscence".
Another test he wanted to have done on me was a VEMP test. VEMP stands for "vestibular evoked myogenic potential". Which to the lay person means absolutely nothing! I tried researching it, so that I knew what to expect and to try to understand the purpose of the test. Unfortunately I was unable to make head nor tail of my findings. I gleaned that it involves electrodes and a pulsating noise in your ear, but the whys and wherefores of it all remain a mystery.
So the day came to go for my test. It was explained to me that the vestibular system is a complex system, and trying to find out where it is going wrong is like trying to find a needle in the haystack. VEMP testing combined with high resolution CT scan is used as a diagnostic tool in diagnosing SCDS. And even though I have had it done now, I still don't understand it, so all I can do is explain the process.
I was hooked up to some electrodes. Two each side of my neck, placed on specific muscles. I had further electrodes placed on the front of my neck, and my forehead. The electrodes were tested, and retested, and then moved around, until after what seemed like forever, they were happy that they were set up correctly. I then had to turn my head one way, to put my neck muscle under just the right amount of tension, which was determined by a traffic light type arrangement. Red meant that it was contracted too much, blue was not enough, and green was just right. They then, through and ear piece placed in my ear, sent a loud pulsating noise. This made my eyes bounce all over the place, but I had to concentrate on keeping my neck in the correct position. I had this repeated on the other side (which was much harder as couldn't maintain the correct tension in my neck). Then the test was repeated on each side again but at a lower sound level.
I was expecting to feel particularly disorientated after all that, but I think the overwhelming feeling was one of tiredness, and a slightly sore neck!!
The results of the test will be analysed over the next couple of weeks, and hopefully, when I get to see the consultant again in December, we will be able to formulate a plan of action to rid me of the symptoms of this condition.
I went to see the different ENT at the end of October, and this was the first time I felt that I was talking to a medical person who knew anything about this condition. I was in a room with the ENT, a physio, and various other nurses who between them all made up the balance clinic. Unfortunately the ENT didn't have access to the reformatted version of my CT scan, but he later wrote me a letter saying that once he had seen it, it became clear that I "had quite a good example of superior semi-circular canal dehiscence".
Another test he wanted to have done on me was a VEMP test. VEMP stands for "vestibular evoked myogenic potential". Which to the lay person means absolutely nothing! I tried researching it, so that I knew what to expect and to try to understand the purpose of the test. Unfortunately I was unable to make head nor tail of my findings. I gleaned that it involves electrodes and a pulsating noise in your ear, but the whys and wherefores of it all remain a mystery.
So the day came to go for my test. It was explained to me that the vestibular system is a complex system, and trying to find out where it is going wrong is like trying to find a needle in the haystack. VEMP testing combined with high resolution CT scan is used as a diagnostic tool in diagnosing SCDS. And even though I have had it done now, I still don't understand it, so all I can do is explain the process.
I was hooked up to some electrodes. Two each side of my neck, placed on specific muscles. I had further electrodes placed on the front of my neck, and my forehead. The electrodes were tested, and retested, and then moved around, until after what seemed like forever, they were happy that they were set up correctly. I then had to turn my head one way, to put my neck muscle under just the right amount of tension, which was determined by a traffic light type arrangement. Red meant that it was contracted too much, blue was not enough, and green was just right. They then, through and ear piece placed in my ear, sent a loud pulsating noise. This made my eyes bounce all over the place, but I had to concentrate on keeping my neck in the correct position. I had this repeated on the other side (which was much harder as couldn't maintain the correct tension in my neck). Then the test was repeated on each side again but at a lower sound level.
I was expecting to feel particularly disorientated after all that, but I think the overwhelming feeling was one of tiredness, and a slightly sore neck!!
The results of the test will be analysed over the next couple of weeks, and hopefully, when I get to see the consultant again in December, we will be able to formulate a plan of action to rid me of the symptoms of this condition.
Friday 6 November 2015
A wake up call.
This illness has forced me to make decisions about daily life. I had to decide to stop driving, I've had to choose carefully where I go, and what I do. I've had to cut back on what work I take on. By doing these things I can generally have a days that are manageable. Sure, I might feel a bit off, and some days are definitely better, or worse than others, but in general I am ok.
So it is easy to forget that I do have a condition that affects me as much as it does. Sometimes you go and do something and it makes you realise that things are most definitely not right.
Yesterday, I went to a small demonstration, as our local hospital's maternity unit was being shut. A cause I felt very strongly about, so I figured I would at least show my face. I had taken my stick, but forgot, of all things, my ear plugs. I was stood for 2 hours, with cars tooting, and sirens going off at times. Before I was ill, perhaps the most I would feel from that would be sore feet, and a bit tired. But, apart from staggering a few times, and suffering for the loud noises, I then started to feel very off after two hours, and had to make my way home.
Getting home was hard, I was staggering, really struggling to walk in a straight line, and my vision was really distorted. All I could think was how this is not how I should be. I should be able to do these things without feeling so unwell. How I need to get better. An hour of peace and quiet when I got home was enough to rejuvenate me enough to go do the school run, and I am glad that I could bounce back so quickly.
So today I am back to a quiet life, and I am doing ok. But life cannot be reduced to this all the time. I have to remember that when life gets busy, and noisy, I will feel unwell. Another thing I have to remember is to take my ear plugs with me!
So it is easy to forget that I do have a condition that affects me as much as it does. Sometimes you go and do something and it makes you realise that things are most definitely not right.
Yesterday, I went to a small demonstration, as our local hospital's maternity unit was being shut. A cause I felt very strongly about, so I figured I would at least show my face. I had taken my stick, but forgot, of all things, my ear plugs. I was stood for 2 hours, with cars tooting, and sirens going off at times. Before I was ill, perhaps the most I would feel from that would be sore feet, and a bit tired. But, apart from staggering a few times, and suffering for the loud noises, I then started to feel very off after two hours, and had to make my way home.
Getting home was hard, I was staggering, really struggling to walk in a straight line, and my vision was really distorted. All I could think was how this is not how I should be. I should be able to do these things without feeling so unwell. How I need to get better. An hour of peace and quiet when I got home was enough to rejuvenate me enough to go do the school run, and I am glad that I could bounce back so quickly.
So today I am back to a quiet life, and I am doing ok. But life cannot be reduced to this all the time. I have to remember that when life gets busy, and noisy, I will feel unwell. Another thing I have to remember is to take my ear plugs with me!
Wednesday 4 November 2015
An explanation by expert Dr Carey
I came across this video the other day that explains exactly what superior semi-circular canal dehiscence is, and how the symptoms come about. I found it very useful, and I hope you do too.
https://m.youtube.com/watch?v=i7zkb30-d50
https://m.youtube.com/watch?v=i7zkb30-d50
Tuesday 3 November 2015
So what is Superior semi circular canal dehiscence?
Any google search will give you details of this condition. However, I will summarise what I have discovered in my many hours of trying to work out what was wrong with me prior to official diagnosis.
SSCD has only been around officially since 1998, when it was described by Lloyd B Minor. Who noticed that patients were experiencing a bizarre collection of symptoms, which were sound or pressure induced. He discovered that a small hole in the bone that lies between the base of the brain and the inner ear was enough to create the vertigo and other symptoms. Rather than there being the usual two entrances/exit to the inner ear (oval window and round window) where sound would enter and exit the inner ear. This extra hole added another source of sound and pressure to the vestibular system. The source of all this was through the sounds and pressures of the body itself, and external noises and pressures too.
The thinking is, that as the bones in the head develop, not enough bone is put down at the location of the tegmen (between ear and brain cavity). This is not usually a problem in early life, as there is sufficient bone to prevent symptoms, but as the body ages, and through wear and tear, the bone is reduced, to the point that interference of the vestibular system occurs. Symptoms can arise through the bone being simply too thin, or through an actual hole (dehiscence). So the average age of occurrence is in a persons 40's. Although some people do obviously get it earlier or later than this, and some people get it through head trauma.
With it being such a "new" condition, it is not really known what the incidence of this is. At autopsy they reckon about 1-2% of the population could have a dehiscence, but diagnosis has been very tricky for some, with them often being misdiagnosed for many, many years.
Now, I am new to all this, and as a non-medical person I am trying to make sense of it all. But the basics of it are that they vestibular system is a finely tuned system, reliant on sight, the awareness of where your body is in space (proprioception) and the feedback from the inner ear. When something is not right with any of these, it can affect the vestibular system. One of the side effects of one part of the system not working, is that you rely far more heavily on the other parts. For me, this means that walking in the dark can be more difficult as I don't have the feedback from my eyes. And standing on uneven ground can also be problematic as the proprioception feedback is unusual.
For people with SSCD, the symptoms are varied and bizarre. I will highlight my own symptoms first, and then look at those that other people also suffer with. I plan to elaborate on all these in a later post.
Hearing internal noises (autophony) - this, for me, includes hearing my eyes moving, hearing my heart beat, hearing my neck joints moving, even when I don't think I am moving my neck, just the slightest movement can be deafening. Hearing my footsteps when I am walking, and hearing my voice as particularly loud.
Hyperacuasis (sensitivity to external sounds)
Tullio phenomenon - noises creating a sense of imbalance.
A feeling of fullness in your ear, as if your ears are blocked.
Vision jumping up and down in time to your heart beat, which gives the illusion of things moving. When this is particularly severe it is incredibly disorientating.
Feeling unsteady. This is like being on a boat, or how you feel after a little too much alcohol. But then I can have episodes where I am fighting against an unknown force to stay upright.
Bass noises making me feel unsteady and vibrating my vision.
Slight nausea
Tinnitus - ringing in the ear
Headaches
These symptoms vary from day to day, some days are far worse than others. On a bad day, the best thing I can do is go to bed and try to sleep. Yet on a good day I feel nearly normal, if a little unsteady and hearing a few odd things in my head when it is quiet.
Other symptoms that people can experience are
Nystagmus - Involuntary eye movements
Brain fog
Exhaustion
SSCD has only been around officially since 1998, when it was described by Lloyd B Minor. Who noticed that patients were experiencing a bizarre collection of symptoms, which were sound or pressure induced. He discovered that a small hole in the bone that lies between the base of the brain and the inner ear was enough to create the vertigo and other symptoms. Rather than there being the usual two entrances/exit to the inner ear (oval window and round window) where sound would enter and exit the inner ear. This extra hole added another source of sound and pressure to the vestibular system. The source of all this was through the sounds and pressures of the body itself, and external noises and pressures too.
With it being such a "new" condition, it is not really known what the incidence of this is. At autopsy they reckon about 1-2% of the population could have a dehiscence, but diagnosis has been very tricky for some, with them often being misdiagnosed for many, many years.
Now, I am new to all this, and as a non-medical person I am trying to make sense of it all. But the basics of it are that they vestibular system is a finely tuned system, reliant on sight, the awareness of where your body is in space (proprioception) and the feedback from the inner ear. When something is not right with any of these, it can affect the vestibular system. One of the side effects of one part of the system not working, is that you rely far more heavily on the other parts. For me, this means that walking in the dark can be more difficult as I don't have the feedback from my eyes. And standing on uneven ground can also be problematic as the proprioception feedback is unusual.
For people with SSCD, the symptoms are varied and bizarre. I will highlight my own symptoms first, and then look at those that other people also suffer with. I plan to elaborate on all these in a later post.
Hearing internal noises (autophony) - this, for me, includes hearing my eyes moving, hearing my heart beat, hearing my neck joints moving, even when I don't think I am moving my neck, just the slightest movement can be deafening. Hearing my footsteps when I am walking, and hearing my voice as particularly loud.
Hyperacuasis (sensitivity to external sounds)
Tullio phenomenon - noises creating a sense of imbalance.
A feeling of fullness in your ear, as if your ears are blocked.
Vision jumping up and down in time to your heart beat, which gives the illusion of things moving. When this is particularly severe it is incredibly disorientating.
Feeling unsteady. This is like being on a boat, or how you feel after a little too much alcohol. But then I can have episodes where I am fighting against an unknown force to stay upright.
Bass noises making me feel unsteady and vibrating my vision.
Slight nausea
Tinnitus - ringing in the ear
Headaches
These symptoms vary from day to day, some days are far worse than others. On a bad day, the best thing I can do is go to bed and try to sleep. Yet on a good day I feel nearly normal, if a little unsteady and hearing a few odd things in my head when it is quiet.
Other symptoms that people can experience are
Nystagmus - Involuntary eye movements
Brain fog
Exhaustion
Tuesday 20 October 2015
The journey so far
I have started on a journey. A journey that I wouldn't choose to make. A journey I wouldn't want anyone to make. A journey into the condition of "superior semi-circular canal dehiscence"(SSCD). That is my diagnosis. But I am kind of jumping the gun a bit.
Let me take you back to the beginning.
It was around Christmas time 2014 that I realised that at bed time I could hear my heartbeat. I didn't really think too much about it, until it became apparent that it wasn't usual to hear your heartbeat. So I finally went to the doctors. Bloods came back fine, ECG was fine, hearing test showed a little low frequency loss. By Easter I was feeling "off" I realised I could hear my eyeballs move, and was feeling out of sorts. When I exerted myself or got stressed, my hearing of my heart beat became louder, and my vision pulsed in time to my heartbeat.
A flippant comment on Facebook along the lines of "ooh, I can hear my eyes move" led to a friend pointing me in the direction of a rare vestibular condition of "superior semi circular canal dehiscence". I looked at the wikipaedia page about it, and low and behold there was a list of symptoms, of which I could tick off quite a few.
I kept this "diagnosis" to myself, as I figured the doctors would know more. But my visit to the doctor, in June, proved fruitless, with him admitting he didn't know what was wrong with me. He did however refer me to ENT, and gave me a prescription for prochlorperazine (which did nothing)
I got an appointment through for the end of August, which was frustrating to say the least, as my symptoms were progressively getting worse. My tolerance for noise was getting less, I was hearing my footsteps like a drum, when I walked. My balance was going awry, and I often felt as if I'd either drunk a bottle of wine, or was out at sea and everything was constantly moving, with the occasional wave coming along to really knock me off balance.
Driving was becoming difficult. Whilst I would never drive if I was feeling dizzy, I could never predict how I would be feeling by the time it came to the return journey, so I took the hard decision, just as the summer holidays approached to give up driving.
In my frustration at nothing happening (I'm not a very patient patient!) I got back in touch with the GP (a different one as the previous one had moved on) who referred me for an MRI scan whilst waiting for this ENT appointment to come through, and changed my prescription to Betahistine (which still did nothing).
By this time I am experiencing vertigo attacks which feel like I am being pushed. The children liken it to Keanu Reeves' character in the Matrix films, where he bends backwards in slow motion. I realise one morning when walking our dog, and having to hold onto his back to stop me falling over, that I need to use a stick. I borrow my father in law's spare stick for a while, taking it out when I feel wobbly.
An MRI scan was undertaken (with my eyes firmly shut!) and fortunately showed nothing. What they would have been looking for would have been a tumour, so I am very grateful that it came back clear.
Finally, the end of August came around, and I saw the ENT consultant. I explained everything that I was experiencing. I had a few tests, and he told me that he didn't think it was anything to do with my ears! I explained that I had come across this condition of SSCD, and that I felt that it fitted with my experiences, more so than any other vestibular disorder I had come across (the VEDA page is a fantastic resource for understanding vestibular problems). I explained that I would like a high resolution CT scan to at least rule out this condition. He did at least agree to that, even though he felt that it couldn't possibly be that as he hadn't come across a case of that in 20 years of practice.
Early September I went for my CT scan, and was anxious to find out the results. But, as you know, the wheels of medical bureaucracy turn incredibly slow for non urgent cases. I managed to get a cancellation appointment for the second week of October. In the meantime, I had another appointment come through, and whilst I was trying to work out why this was, it became apparent that I should have received a letter. A copy of the letter was sent to me.
The letter confirmed I had a left sided dehiscence of the semi circular canal. This brings me to the present day. I am awaiting to see a different ENT consultant to see where we go from here.
Let me take you back to the beginning.
It was around Christmas time 2014 that I realised that at bed time I could hear my heartbeat. I didn't really think too much about it, until it became apparent that it wasn't usual to hear your heartbeat. So I finally went to the doctors. Bloods came back fine, ECG was fine, hearing test showed a little low frequency loss. By Easter I was feeling "off" I realised I could hear my eyeballs move, and was feeling out of sorts. When I exerted myself or got stressed, my hearing of my heart beat became louder, and my vision pulsed in time to my heartbeat.
A flippant comment on Facebook along the lines of "ooh, I can hear my eyes move" led to a friend pointing me in the direction of a rare vestibular condition of "superior semi circular canal dehiscence". I looked at the wikipaedia page about it, and low and behold there was a list of symptoms, of which I could tick off quite a few.
I kept this "diagnosis" to myself, as I figured the doctors would know more. But my visit to the doctor, in June, proved fruitless, with him admitting he didn't know what was wrong with me. He did however refer me to ENT, and gave me a prescription for prochlorperazine (which did nothing)
I got an appointment through for the end of August, which was frustrating to say the least, as my symptoms were progressively getting worse. My tolerance for noise was getting less, I was hearing my footsteps like a drum, when I walked. My balance was going awry, and I often felt as if I'd either drunk a bottle of wine, or was out at sea and everything was constantly moving, with the occasional wave coming along to really knock me off balance.
Driving was becoming difficult. Whilst I would never drive if I was feeling dizzy, I could never predict how I would be feeling by the time it came to the return journey, so I took the hard decision, just as the summer holidays approached to give up driving.
In my frustration at nothing happening (I'm not a very patient patient!) I got back in touch with the GP (a different one as the previous one had moved on) who referred me for an MRI scan whilst waiting for this ENT appointment to come through, and changed my prescription to Betahistine (which still did nothing).
By this time I am experiencing vertigo attacks which feel like I am being pushed. The children liken it to Keanu Reeves' character in the Matrix films, where he bends backwards in slow motion. I realise one morning when walking our dog, and having to hold onto his back to stop me falling over, that I need to use a stick. I borrow my father in law's spare stick for a while, taking it out when I feel wobbly.
An MRI scan was undertaken (with my eyes firmly shut!) and fortunately showed nothing. What they would have been looking for would have been a tumour, so I am very grateful that it came back clear.
Finally, the end of August came around, and I saw the ENT consultant. I explained everything that I was experiencing. I had a few tests, and he told me that he didn't think it was anything to do with my ears! I explained that I had come across this condition of SSCD, and that I felt that it fitted with my experiences, more so than any other vestibular disorder I had come across (the VEDA page is a fantastic resource for understanding vestibular problems). I explained that I would like a high resolution CT scan to at least rule out this condition. He did at least agree to that, even though he felt that it couldn't possibly be that as he hadn't come across a case of that in 20 years of practice.
Early September I went for my CT scan, and was anxious to find out the results. But, as you know, the wheels of medical bureaucracy turn incredibly slow for non urgent cases. I managed to get a cancellation appointment for the second week of October. In the meantime, I had another appointment come through, and whilst I was trying to work out why this was, it became apparent that I should have received a letter. A copy of the letter was sent to me.
The letter confirmed I had a left sided dehiscence of the semi circular canal. This brings me to the present day. I am awaiting to see a different ENT consultant to see where we go from here.
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