Thud, thud, thud. The sound of my feet boomed in my ears as I ran up the steps of the community centre. Rushing in late, I joined a meeting of parents. As I sat listening, I realised they were being drowned out by a new noise. I was still breathing fast and it slowly dawned on me that the sound I could hear was the pounding rhythm of my heartbeat. Unable to focus on the discussion, I felt an overwhelming sense of despair.
I have superior semicircular canal dehiscence syndrome (SCDS), a balance disorder. It’s a developmental anomaly that occurs during the first three years of life and is thought to be caused by a lack of bone deposition in the uppermost balance canal of the inner ear, which can cause a tiny hole to develop. I had two holes, one on each side, like about 50% of sufferers. 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. The holes in the bone are tiny, but their effects are far-reaching.
SCDS was discovered in 1995 by Dr Lloyd Minor, an ear, nose and throat specialist at The Johns Hopkins University School of Medicine in Baltimore. Minor connected strange symptoms in a couple 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. As he explained in a speech in 2011: “Some of my first patients were referred to me by psychiatrists. One man said he got dizzy when he sang in the shower. Another said he could hear the sound of his own eyes moving. I would later discover they were experiencing symptoms coming from inside their heads – but not in the ways others might have supposed.” The fact that there is great variation in symptoms means that many more cases could be going undetected or misdiagnosed.
From my 20s, I was sure something wasn’t right. I often felt nauseous, unbalanced, or both, and little by little my confidence was eroded. As a researcher for a book publisher I often worked in the British Library, which I loved. But I began to dread the walk from my desk to the central collection area. The sound of trolleys moving books around or shelves being restacked seemed to be causing a feeling of unsteadiness and anxiety. I put this down to some sort of panic attack. These sensations gradually extended to other environments – and fluctuated wildly, which added further confusion.
This continued into my 40s. Then in 2003, a sharp change of pressure during the descent at the end of a flight triggered the full range of symptoms. A brutal pain in my left ear gradually morphed into a permanent feeling of pressure. The inside of my head felt like a balloon I badly needed to burst.
My troubled ear led me to a GP, followed by a series of ENT consultants, at first near my home in Edinburgh, then further afield in London and Oxford. A clear MRI scan seemed to be my cue to disappear, despite tests showing evidence of hearing loss, but I was determined to persevere as my symptoms not only remained, they were subtly changing. I had been getting severe headaches; the nausea was becoming persistent, and it was extremely uncomfortable to raise my voice, sing or eat crunchy foods. Over the months the list of symptoms kept growing, with blurred vision and vertigo among them.
I had no way of knowing that my years of unsteadiness were directly linked to the problematic ear, and that the creaking noises in my neck or the clicking sounds from my eyes might be significant. Months of research to find an explanation resulted in an email to Dr Dennis Poe, a renowned Boston ear specialist. He directed me to a doctor in Oxford to be evaluated for another unusual condition – patulous eustachian tube, where the tube that connects the inner ear to the nasopharynx doesn’t stay closed – but I came away instead with a diagnosis of SCDS, later confirmed by a CT scan.
Referral for surgery should have set me on the road to recovery. But an experimental procedure proved disastrous. I came out of surgery in a worse state, with terrible vertigo and pain. After 18 exhausting months, I went to see Dr Gerard Gianoli at The Ear and Balance Institute in Louisiana, who has carried out more than 200 successful SCDS procedures. It was he who established that my SCDS was in fact bilateral and performed successful revision surgery on the 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 as the brain has a lot of adjusting to do and the inner ear has to heal. But now, following a further repair on the right side, I’m at the start of a new, and quieter, life – one without holes in my head.
A Hole in My Life: Battling Chronic Dizziness by Philippa Thomson is available from Amazon
For more information, visit scdssupport.org
