Ear-Induced Torture: Maddening Noise, Everywhere
One man's rare condition amplifies all noises, makes eye movement audible.
March 12, 2008 -- Imagine if every sound you heard reverberated right through your brain.
Adrian McLeish could hear a voice in his head. Not just any voice — his own, amplified and distorted, echoing through his skull. But it didn't stop there. Adrian heard every sound in his body. His heart beating in his chest was, at times, deafening. The sound of his own chewing was maddening.
It all began in 1985. British-born McLeish was 35 years old, living in Kassel, Germany and playing French horn in the orchestra there. One day, as he was practicing his horn, he noticed that something felt different.
"I had this strange sensation that I was hearing my horn playing somehow through the inside of my head," McLeish, now 58, said. "The same was true of my voice."
McLeish described the reverberations of his voice as sounding like a cracked loudspeaker or "like somebody humming through a kazoo." In the beginning, they were irritating and constant, but not debilitating.
The Sound Of His Eyes
At first, McLeish, paid little attention to the change, figuring it would eventually just go away. But it didn't. Every day, over a period of months, the sensations grew more intense and more disruptive. He could hear his own footsteps and the sound of his own chewing as he ate. The vibrations of passing cars and trains were nearly intolerable. Even playing his beloved horn became excruciating.
What stopped him in his tracks was the evening he thought he heard a mouse rustling about in the walls. Putting down his book, he rose from bed to inspect for rodents but each time he stopped to listen, the noises stopped as well.
Finally he realized the disturbing truth: There was no mouse. McLeish was hearing the sounds of his own eyes, as they moved back and forth across the pages of his book.
"It was extremely weird," McLeish said. "And unsettling."
McLeish also developed a high-pitched whistling in his ear, a condition known as tinnitus.
But the trouble didn't stop there. Several months after his hearing problems began, McLeish began to be plagued by a new set of symptoms: involuntary eye movements caused by sound.
"I was talking to somebody on the phone," McLeish said. "And when the phone rang on the other end, I noticed that the bookshelves on the other room tipped five degrees in the opposite way."
Any loud noise would cause McLeish's eyes to jerk and throw his balance off. And things only got worse.
"I started to notice that as I spoke, my eyes were jerking with my speech," he said. "If I played a series of notes on the horn, my eyes would jump up and down."
Doctor to McLeish: Drink More Beer
He began to seek out a diagnosis, visiting countless doctors, all seemingly puzzled by his symptoms. One doctor attributed the condition to stress and prescribed tranquilizers. Another told him to drink more beer.
He even consulted a faith healer, who told him that a colleague had placed a demon in his head in order to sabotage his horn playing.
Eventually, McLeish underwent three surgeries, but his condition was once again misdiagnosed and the operations failed. McLeish was beginning to lose hope. He was dealt another blow last year, when the noise finally drove him to stop playing his horn.
"It's difficult when your husband has something like this," his wife Cindy said. "It's difficult to know that he's suffering."
One evening McLeish sat down at his computer and Googled his symptoms. To his shock, the simple Internet search turned up a rare condition that matched his symptoms perfectly: Superior Canal Dehiscence or SCD.
Even more amazing, he learned that Dr. Lloyd Minor, the surgeon at Johns Hopkins Medical Center who originally discovered and described SCD in 1995, had also devised a surgical treatment for it. McLeish immediately made plans to have surgery on his left ear. During June of last year McLeish travelled to Baltimore.
The Cure
The human ear contains three canals filled with fluid. When we move, the fluid in the canals moves with us and signals the eyes to shift subtly. This helps us to maintain our balance, stand upright, and walk and run without falling over.
"They're like a gyroscope in an airplane and every time we move our head, even by a minute amount, these receptors tell our brain how the head is moving," Minor explained.
Superior Canal Dehiscence is a defect in the inner ear caused by the thinning of the bone that separates the superior canal from the brain. In the case of McLeish, the fluid in his ear, separated by only a thin membrane, touched his brain and transmitted all the sounds from his body right to his head.
When McLeish arrived in Baltimore, he endured a battery of tests to confirm the diagnosis, including a CT scan and an exam where copper wired contact lenses were placed on his eyes.
Once Minor and his colleague, Dr. John Carey, were certain that McLeish did in fact have SCD, they scheduled him for the microsurgery that might answer his prayers.
The day before the surgery, the medical team affixed electrodes to McLeish's face, creating a virtual map of his skull that would guide the surgeons through the operation. Once in the operating room, the doctors plugged the hole in McLeish's ear with tissue and bone taken from his skull.
In the post-operative ICU, McLeish's wife waited anxiously for her husband to stir from his anesthetia-induced sleep.
'I Got My Miracle'
When his eyes finally fluttered open, the first words out of his mouth were, "Will I be able to play the piano?" It was the punch line to the couple's favorite medical joke.
McLeish's recovery was surprisingly smooth.
"I said when I came here, if they gave me 50 percent reduction in my symptoms, it would have been good," he said, a week after his surgery. "I said 90 percent would be a miracle. I got 98 percent. It's better than I ever hoped. I got my miracle."
Eight months after he laid down his horn, and only four months after the operation, McLeish was able to return to his orchestra in Germany. When he finished his first concert in more than a year, he knew for sure that life had finally returned to normal.
"The noises are gone," he said. "It took me 23 years to find out what I had. In the future I hope that people can find it much sooner and get the relief I've gotten."
For more information about Superior Canal Dehiscence please visit the Vestibular Disorders Association and the Johns Hopkins Web site.