I need this like a hole in the head.

A musician reflects on living with Semi-circular/Superior Canal Dehiscence Syndrome (SCDS)
(updated Feb 2022)

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 actually the sound of my own eyeballs. At gigs 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. In 2019 I was diagnosed with 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). I just have the hole on my right side, although scans showed the bone is very thin on the left too. 

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 or even a change in the weather can mean these symptoms ramp up considerably.

Here are 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.
Certain sounds make me feel like I’m falling over or about to blackout. Common culprits are higher frequency sounds like dogs barking, kids shouting, police sirens, electric guitars and my even own voice, especially when singing.
3) Hyperacusis - sound sensitivity
Everyday sounds will set me on edge. They become intolerable. Something like putting plates away in a cupboard, or typing on a keyboard can grate on my nerves or put my teeth on edge. It's like nails scraping down a blackboard but it might be a chair scraping on a floor. Just sitting inside a noisy bar/restaurant becomes a feat of endurance. 
4) Balance problems
Some days I can’t walk straight. I walk into doors and veer into walls. It’s like having had 8 pints. The weather can affect it - when there are extremes of barometric pressure. At worse I just fall over for no reason.
5) Nausea
This is like getting sea sickness on dry land. I just feel sick a lot of the time.
6) Cognitive disfunction - Brain Fog / Memory Loss
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. This is perhaps the worst of all the symptoms, as I cannot function as fast as I used to. Another consequence is that 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
The brain recognises something is wrong, but it's not sure what it is and that can manifest as Anxiety. Especially if my brain is visually overloaded with information (like a busy visual environment such as a supermarket or driving on a motorway) it can lead to feelings of dread or unease - at worse bring on a panic attack. With SCDS anxiety is also generalised. I just feel on edge most of the time and I hardly ever feel relaxed.
8) Tinnitus.
Another side effect. I get both regular tinnitus (very high pitched constant ringing mostly in my right ear) 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. So I need subtitles when I watch TV and I can't have a conversation over dinner, as all i can hear is food.
10) Headaches and pain
It hurts. Earache, headache, jaw pain, neck pain. As my body is never balanced correctly, over time it affects posture and so I have muscle and joint pain on one side. I also get vestibular migraines and headaches which can be caused by the weather pressure.

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
–  If I'm singing then I can’t hear my guitar playing at all, so I don’t know for sure if I’m hitting the right notes with my fingers
– 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
- singing can be physically painful and uncomfortable

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? That bit of doubt can destroy a performance and so live gigs have become very challenging.

How am I adapting to it?
Here are some of the ways I have tried to adapt
1. I record myself singing and playing guitar regularly so I can listen back and check that I am sounding OK and in tune. It helps reassure me.
2. When I was performing gigs I paid close attention to my posture. If your feet are planted wide you're less likely to fall. It helped reassure me that my core is strong, should I get a tullios attack when I’m singing.
3. I wear ear plugs. 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.

Since the 2020 lockdown I have stopped live performances altogether. When I do perform such as livestreaming or during a workshop I will sit down.

Above all the one adaptation that has worked best has been to be open and honest about the condition with people I work with. Although it’s awkward sometimes, I always tell people how it might affect the work I’m doing and what if anything they can do to help. (that's partly why I wrote this blog) In all cases people have been supportive and willing to help.

What caused it?
We don't know for sure but one theory is that it’s most likely congenital – an early child development issue. The damage is thought to be 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 presents to people in their forties. Pressure can cause it to crumble away so symptoms can seem to appear suddenly 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 involve a craniotomy – where the skull is sawn open – 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. I'm on a waiting list. Surgery will put me out of action for a few months and that’s a scary prospect for someone who is self-employed. 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 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.

UPDATE Feb 2022

Since I wrote this blog in December 2019, things have deteriorated. Day to day my symptoms are worse. I find driving really hard, I was confused at first but I've since found out it is because of something called vestibular overload, my brain is struggling to process all the incoming information. I'm the same in supermarkets. So many of these things are very hard to explain - they are weird and sometimes the only symptoms is a sense of unease or feeling uncomfortable.

When lockdown happened in March 2020, most of my work (gigs, song workshops) stopped overnight. From a financial point of view it was a disaster but from an SCDS point of view, I breathed a sigh of relief. I took to livestreaming from home which was much more comfortable. But as the months went by, any sort of singing became harder for me. I used to play guitar and sing every single day. These days I might do it once a week. I can't play standing up anymore, I have to sit down.

So this has led to some awful decisions. I had to cancel a tour I was about to do with a project called The Low Drift. I felt terrible letting my friends down. I've stopped taking any more bookings for live gigs. It's just not possible anymore -  the driving is exhausting, the stress of performance - it's just not possible for me.  I am about to begin a series of songwriting workshops - I feel like I can manage these and I still need to make some money - so I am giving it a go.

I have been waiting for surgery now for 2 years. So I am hoping I can get the surgery soon, this year. It can't come soon enough. I miss my music.