News & Events

Philippa’s Story: Living with Superior Semicircular Canal Dehiscence Syndrome

Last Reviewed 13/07/2015

By Philippa Thompson

I have Superior Semicircular Dehiscence Syndrome (SCDS) which is a balance disorder. The developmental anomaly, present in 1-2% of the population, occurs during the first three years of life, and is thought to be caused by a lack of bone growth in the uppermost balance canal of the inner ear, which can cause a tiny hole to develop. In roughly 50% of cases this is on both sides of the head.

The Path to Diagnosis

By my twenties I was sure something wasn’t right. I often felt nauseous and unbalanced, and over time it had a very dramatic effect on my confidence. As a researcher for a book publisher, I regularly worked in the British Library, which I loved. But I began to dread the walk from my desk to the central collection area. The sounds of trolleys moving books around or shelves being restacked seemed to cause a feeling of unsteadiness and anxiety. I put this down to some sort of panic attack. The same sensations gradually extended to other environments and fluctuated wildly, which added further confusion. The problems with balance, anxiety and a general feeling of something making me feel unwell were difficult to deal with as they couldn’t be attributed to anything specific. For years, the condition affected my behaviour at work, as well as my social relationships. Then in 2003, when I was in my forties, a sharp change of pressure during the descent at the end of a flight triggered the full range of symptoms. The inside of my head felt like a balloon I badly needed to burst.

It took me four years to get a diagnosis, in part due to a lack of awareness among doctors, and over that period my symptoms were subtly changing. The headaches were becoming severe, the nausea persistent, my vision was often blurred, and it was extremely uncomfortable to raise my voice, sing, or even eat crunchy foods. All those sounds reverberated inside my head. I had great trouble hearing people in noisy surroundings, and yet I was also hypersensitive to certain sounds such as a banging door, my dog barking, or a spoon clattering on the floor. I had no idea that my years of unsteadiness were directly linked to these new problems, and that the long-standing creaking noises in my neck or the clicking sounds from my eyes might be significant. As time progressed, I also had quite severe tinnitus, with terrible whooshing and thumping noises in my ear. Everything had a knock-on effect on my brain, affecting my memory and concentration.

During this time, I was referred to a consultant to be evaluated for Patulous Eustachian Tube, an unusual condition where the tube connecting the inner ear to the nasopharynx doesn’t stay closed. By this point, I was having frequent freeze-on-the-spot attacks of vertigo, and no longer felt safe driving. My life was already a complicated one with the care of a severely disabled child, and I felt I could no longer cope.

Surgery

Having finally had my diagnosis of SCDS confirmed by a CT scan, I was referred for surgery, which should have set me on the path to improvement. Unfortunately the surgeon tried out a new method for the procedure and I came out in a worse state, with terrible vertigo and pain.

After 18 exhausting months of trying to get things put right in the UK, I went to see the world’s most experienced specialist, Dr Gianoli in Covington (Louisiana). He established that the SCDS was in fact bilateral and also explained all the anxious years: “Panic attacks are the result of too much of a particular chemical (e.g. catecholamine) surging through your bloodstream. Abnormal vestibular stimulation is a common trigger for their release.” He performed revision surgery on my left side.

Surgery was an easy choice for me as the condition had taken control of my life, but there were serious risks to consider, including hearing loss and nerve damage. These operations aren’t an overnight fix; the brain has a lot of adjusting to do. My right side was successfully repaired in 2012 but I still have whooshing noises from pulsatile tinnitus. The left side unfortunately remains problematic due to a rare nerve syndrome linked to the SCDS. I continue to manage ever-present symptoms from that.

Understanding SCDS

It was only relatively recently, in 1995, that Dr. Lloyd Minor, an ENT (Ear, Nose and Throat) specialist at the John Hopkins University School of Medicine in Baltimore, identified the syndrome, and doctors are still learning about all its complexities. Dr Minor made the discovery by connecting strange symptoms in a few of his patients to a study of pigeons, in which specific eye movements had been linked to damage to certain canals of the inner ear. In some cases very thin bones (even without the holes I have) in the ear can cause a range of symptoms.

In a speech he explained, “Some of my first patients were referred to me by psychiatrists. I would later discover they were experiencing symptoms coming from inside their heads – but not in the ways others might have supposed”. Sound or pressure can trigger abnormal activation of the balance canals in the ear, which in turn leads to a wide range of distressing and disabling symptoms.

An invisible condition of this kind is hard for other people to understand, and it is vital that public awareness is raised. Not everybody who has the anatomical defect is symptomatic, but it is estimated that approximately 10% of patients with chronic dizziness could have SCDS, with many undiagnosed or misdiagnosed.

Most people have never heard of SCDS and certainly have no idea how difficult it is to live with. The holes in the bone are tiny, but their effects are far-reaching.

Learn More

For more information on SCDS visit scdssupport.org.

Philippa

Philippa has been busy trying to raise awareness about SCDS and has written for the Guardian and Eurodis. (@PhilippaThomson)

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