Internal cacophony

These dramatic scars represent blessed relief to these patients. Until their successful surgeries (see photos posted by Mindy Haines, Maryland, USA, 2nd image), they suffered from a condition I was unaware of until the other day. Superior Canal Dehiscence Syndrome (SCDS) is characterized by extreme sensitivity to sound (autophony, hyperacusis), dizziness (disequilibrium, sound-induced vertigo), pulse-synchronous movements and noises (nystagmus, tinnitus), and brain fog.

I thought M.S. had some strange symptoms, for instance the month or so that an 8" patch of skin on my side - which appeared no different than the rest - felt like it was rippling whenever fabric touched it. That's nothing compared to what those with SCDS put up with. They hear the noises of movements and processes occurring within their bodies - and it goes far beyond borborygmus. Consider these descriptions (and watch this video):

"I was sitting quietly alone in the house one evening, and I suddenly heard this quite loud scratchy noise, like sandpaper being rubbed on wood. I was quite alarmed and looked around, wondering what it was. Then I noticed the noise came every time I moved my eyes. I started to think I was going mad....I found if I raised my voice, I'd get a vibration in my head. If I was eating a bag of crisps, the crunching noise drowned out people speaking. Then I found I would be hearing my heartbeat."~Stephen Mabbutt, 57, Oxfordshire, UK

"I know some of you are thinking, 'But wait, didn’t you get ear surgery because of hearing problems?' Well, yes I did, but the problem was that I was hearing too much. My voice, my heartbeat, and other sounds in my head and body were way too loud in my right ear."~Todd Bradley, Colorado, USA (3rd image, watch his video "Inside Todd's Head," which demonstrates his symptoms)

"Over the past 5 to 10 years I began to experience hearing loss in my left ear. I first noticed this when I realized I was not hearing my clock radio in the morning and it was more than willfulness to play hooky. This condition caused me a lot of frustration as a musician as I began to have trouble not only hearing external sounds but it was becoming hard to hear my own pitches internally....On top of that I began having other strange symptoms like if I heard a loud sound or sang very loud myself my left ear would 'reverberate' or kind of jangle and echo, but not my right ear. Loud sounds would also make my left ear ache very badly and radiate out around the side of my face. Besides these hearing issues I had other odd things that would go on….for example if I called someone on the telephone, as the phone rang in my left ear my eyes would vibrate and whatever I was looking at would shake in synch with the telephone ring!"~Chris Hartzog, Washington, USA (1st image, photos here)

"The surgery went well but it is difficult to tell how successful it was as the symptoms go away gradually as her skull/brain/ear heal. The surgeons used a composite of ground bone and wax compound to plug the dehiscence and a type of cement wall used between the canal and brain. The extreme vertigo and pain she is experiencing is typical for this type of surgery. One of the first things she said when she awoke was 'I don't hear my eyeballs swish anymore.'"~Mother of Holly Abbuhl, Michigan, USA

SCDS was discovered by American surgeon Lloyd B. Minor, who described it in 1998 and put forth a surgical solution to this defect of a bone in the inner ear. A colleague says, “Reports of new disorders don’t come along very often. It’s especially rare for the first paper describing a new syndrome to also include the science that explains what’s causing it, plus an operation that fixes it completely.”