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

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.