I need this like a hole in the head.

A musician reflects on living with Semi-circular/Superior Canal Dehiscence Syndrome (SCDS)

A few years ago I started to notice a change in my hearing. Weird stuff started happening. One day I realised the strange scratching noise I kept hearing was the sound of my own eyeballs. Watching bands I’d get the sensation of falling backwards, like I was blacking out when a certain note was hit. My ear felt like it was stuffed with cotton wool. I had a constant cold but only in one side of my head. It was all a bit weird. I wondered if I was losing my mind.

Getting diagnosed was a two year long slog of GP appointments, false trails, drugs, hearing tests, before finally MRI scans, CT scans and a thing called a VEMP test provided the answer. Turns out I have a very rare condition that has caused a hole to appear in the bone between my ear and my brain. The hole is called a dehiscence and my condition is SCDS (Semi-circular Canal Dehiscence Syndrome)

This condition was only officially discovered in the 1990s. Prior to that patients were thought to be delusional and referred to psychiatric care. But modern diagnostics like MRI scanning make it easier to detect the tiny hole with the big impact. As someone who makes his living through music, I need this like the proverbial hole in the head. It affects me in so many ways that are often hard to articulate. So I’ve written this blog as a means of answering the questions people have most commonly asked me.

What does it do to me?

Over the years the symptoms have made themselves known to me, slowly one by one. At the moment I’m one of the lucky ones, these can get a lot more severe. Although getting a simple head cold can mean these symptoms ramp up considerably.

Here’s my Top 10 symptoms associated with the Syndrome.
1) Autophony
Hearing your own internal body sounds. I hear my heart beating loudly at all times, I can hear my eye balls moving, my voice doesn’t come through my ears but through my body which makes it sound incredibly loud like a distorted speaker or a kazoo.
2) Tullios (sound induced vertigo) & hyperacusis
Certain sounds  make me feel like I’m falling over or about to blackout, a common one is my own voice when I perform. Other sounds are just hugely painful (this is called hyperacusis) Common culprits include dogs barking, kids shouting or police sirens.
3) Balance problems
Some days I can’t walk straight. I walk into doors and veer into walls. It’s like having had 8 pints.
4) Nausea
This is like getting sea sickness on dry land. I just feel sick a lot of the time.
5) Brain Fog
In order to keep me standing upright my brain is having to work extra hard so it starts to shut down other functions. This makes concentration harder. I often forget words or get muddled up and mixed up about things.
6) Memory loss
Another consequence of the brain having to work harder. My short term memory is terrible. I often have no memory at all of things I have done just a day or two earlier.
7) Anxiety
Anxiety disorder is a commonly reported effect of this illness. I’ve a long history with anxiety which pre-dates this condition, and I work hard to manage that, so it’s sometimes hard to tell to what extent this is due to SSDS. But in the past my anxiety was specific to certain situations, with this I just feel on edge most of the time and I never feel relaxed.
8) Tinnitus.
Another side effect. I get both regular tinnitus (very high pitched ringing) and pulsatile tinnitus which is connected to the pulse.
9) Ear fullness/hearing problems
My ear feels like it’s been stuffed with cotton wool. Although I’m not technically deaf I struggle to hear anything else when I’m eating, walking, talking or singing because my internal noises are so loud.
10) Headaches and pain
It hurts. Earache, headache, jaw pain, neck pain. As my body is not balancing, over time it seems to affect my posture and I have muscle and joint pain on one side.

As a musician that must be hard?
I think it’s hard whatever your profession. But as a singer it drives me mad. My voice is so loud and distorted and that means
–  it’s hard tell if I am singing at the right pitch
–  I can’t hear my guitar playing at all, so I don’t know for sure if I’m hitting the right notes
– if I’m playing or singing with other people it’s hard to hear what they are doing, making it tough to sing harmony or keep time.
Losing my sense of judgement over what I’m performing has been one of the hardest things to adapt to. It really knocks my confidence. Am I on pitch? Am I too loud or too quiet? Should I be closer to the microphone or further away? Those little seeds of doubt can destroy a performance.

How am I adapting to it?
Here are some of the ways I am trying to adapt
1. I record myself singing and playing guitar regularly so I can listen back and check that I am sounding OK. It helps reassure me that what is coming out bears no resemblance to the mess I am hearing in my head.
2. Before gigs I pay attention to my mental state and spend time reassuring myself that I’m going to be ok.
3. When performing I pay close attention to my posture. If my feet are planted wide I’m less likely to fall. It helps reassure that my core is strong, should I get a tullios attack when I’m singing.
4. I’ve started to use an onstage amplifier as a monitor to boost my guitar volume – just for me and not the audience.
5. I sometimes wear an ear plug in the affected ear. It seems to dull the vibration/frequency of my voice and reduce the tullios/vertigo that is triggered by the sound of my own voice.
6. I communicate. Although it’s awkward sometimes, I tell people I am working with, about how it might affect the work I’m doing and what if anything they can do to help.

What caused it?
It’s widely thought to be congenital – an early child development issue. The damage being caused in the first 3 years of life as the skeleton grows. Most people have 0.8mm of bone over their superior canal but I was born with a lot less and slowly over the years it erodes until a hole is made.  It often happens to people in their forties. Pressure can cause it to crumble away so symptoms can seem to appear after things like flying, a bad head cold or a head trauma.

What lies ahead?
Once I got my diagnosis I joined an online support group for people with the condition. There I learned how devastating it can be. Everyone is different and so far I think I’ve been lucky. I’ve read about people who have complete disability to the point they are unable to work. Some people are unable to stand up, even when lying down are constantly spinning, have constant sea sickness, chronic anxiety and cognitive impairment. I’ve seen one of the expert surgeons in the UK and he assures me it isn’t necessarily going to be like that for me. It just depends and there’s no way to tell.

Can it be treated? Can it be fixed?
There are some options to help people manage it. Anti-nausea drugs can stop sea sickness. A hearing aid can feed the sound of your external voice back into your ear. But the best chance of a better life is to have surgery.

Surgery is still developing. It’s rare and so are the specialists who know how to treat it. Only a handful in the UK, but luckily we have one in Manchester who has done it 22 times. Surgery can be quite invasive and sometimes involves a craniotomy – where the skull is sawn open and the brain moved – so the surgeon can get to the hole and plug it up.  So it’s not a light undertaking and the risks are deafness and failure, but those who have had it say it can be life changing.

So that’s the next step for me. Surgery will put me out of action for a few months and that’s a scary prospect for someone who is self-employed. Like most sole traders I have no savings and you can’t get insurance for pre-existing conditions. You can’t pick your date either and having to take months off at short notice could mean cancelling gigs, projects and plans. But I will cross that bridge when I come to it.

There is hope and that hope means a lot when living with an illness. I count myself lucky that it’s not worse but I do wish it didn’t affect the one thing that I live to do – play music.  Like I said, I need it like a hole in the head.