Cindy Hirsch was at a professional conference for audiologists in 1997 when suddenly she just didn’t feel right. It was an unusually noisy conference for audiologists, with entertainment that included loud music and dancers dressed in silver lame at a venue in Fort Lauderdale. At the same time that she started to feel queasy, she looked up at the dancers on stage and was amazed to see them break out into the can-can, bouncing up and down perfectly in sync.
"I thought, 'Oh my god, how can they dance like that all exactly timed together?'"
In reality, the dancers were not doing the cancan at all. It was Hirsch's eyes that were bouncing up and down -- she just didn't know that yet. Thus began Hirsch's seven-year odyssey with a nightmarish constellation of mysterious and strange symptoms. Each day was like living through a rock-concert of overwhelming sounds. The sound of her feet touching carpet pounded in her brain. The turn of her neck creaked loudly. She could even hear her eyeballs move.
I recently interviewed Hirsch for a story in Stanford Medicine magazine about a mystery illness that went undiagnosed for years and left patients complaining of such strange symptoms that many doctors told them they were crazy. As I wrote in the article:
Symptoms described by these patients ranged from relatively mundane (though unpleasant) nausea and dizziness to superhero-like abilities to hear the inner workings of their own bodies — their pulse, their chewing, their digestive systems. They got misdiagnosed, underwent unnecessary surgeries, fell into depression, withdrew from the world.
No one had any idea what was wrong until one of these patients made their way to the office of Lloyd Minor, MD, an expert in the inner ear at Johns Hopkins. All it took was for Minor, now dean of the Stanford School of Medicine, to play a specific tone in his patient’s ear and look into his eyes to figure out what was wrong.
Upon seeing the direction of the eye movement in response to this particular sound, Minor suspected that the source of the problem was a hole in the superior semicircular canal — one of the three tiny canals hidden deep within the inner ear. And he went on to prove it.
Minor discovered a disorder that he named superior canal dehiscence syndrome — and then developed a surgery to treat it. Just how Minor was able to do this when so many others had failed is a long tale involving his years of research into the workings of the inner ear and the vestibular system along with his training as both a scientist and a surgeon, which together made him uniquely qualified to solve the mystery.
As for Hirsch, she'll tell you that Minor quite simply saved her life. In 2006, at Johns Hopkins, she became the 34th patient with superior canal dehiscence syndrome to have the canal-plugging surgery engineered by Minor and colleagues. As I wrote in my piece:
After waking up from surgery, the surgeons asked if she was OK. She motioned with her hand for them to wait, then closed her eyes and moved them back and forth. There was no sound. Her eyeballs had been silenced.
Previously: The power and limits of zeroing in: Stanford Medicine magazine on diagnostics, Stanford's Lloyd Minor featured in piece on rare inner ear disorder and A look at one woman's long journey to diagnosis and treatment of rare disorder
Photo by Timothy Archibald