Even before we perform surgery, patients often tell us they feel an enormous sense of relief. After all, we are often the first doctors they meet who actually believe them when they say they can hear their hearts beating, their eyes moving or their food digesting.
For years, many of these patients have gone to several different doctors and specialists, most of whom misdiagnose this extremely rare condition or dismiss their symptoms all together.
Because some of these patients also suffer from debilitating dizzy spells and nausea, they’re often diagnosed with migraine headaches and given medication for dizziness.
Others end up seeking psychiatric help, convinced they’re somehow imagining these noises from which there is no escape.
We know, however, that their condition is real, and we’ve developed a minimally invasive surgery to treat them.
Pinpointing the Problem
The condition is known as superior semicircular canal dehiscence, or SSCD. It’s a relatively new diagnosis that was first described in the late 1990s.
The disorder is caused by the formation of tiny holes in the inner ear. That region of the ear contains sealed compartments filled with fluid that help control our balance and the way we process sounds.
When holes develop within the bone between the inner ear and the brain, symptoms often develop quickly, including vertigo, hearing loss and Tullio’s phenomenon, which is sound-induced dizziness.
Patients with SSCD may experience autophony, a condition in which their breathing and voice is amplified internally. In more extreme cases, they’re able to hear their heartbeat, footsteps, chewing, swallowing and even eye movements.
Imagine what these patients experience, tormented by bodily noises that can be both deafening and inescapable.
Surgery to Silence the Sounds
Surgical repair of the bone covering the inner ear is the only cure for this condition, but physically reaching the affected area can be a challenge.
Many patients undergo a middle fossa craniotomy, in which part of the skull is removed to expose the brain, allowing surgeons access to the inner ear. While those procedures are often very effective, there’s considerable scarring, and recovery times can be prolonged.
Hoping to provide another option for these patients, my neurosurgery colleague at Ronald Reagan UCLA Medical Center, Dr. Isaac Yang, and I have spent five years developing a minimally invasive approach to SSCD surgery.
Today, we are performing these operations through a hole the size of a dime.
Inside the OR
We start by pinpointing the area of the skull through which we will enter. The holes that develop in the bone covering the inner ear are only one millimeter in size, so we need to be extremely precise in the placement of our instruments.
To help us determine their exact location, we first map the patient’s brain with a CT scan. Then, we use the BrainLab neuronavigation system, which works like a GPS for the brain. This state-of-the-art technology helps us zero in on the tiny holes in the bones of the inner ear and shows us the precise location through which to access them.
Working in tandem, Dr. Yang first opens the cranium to expose the inner ear. Then I locate the tiny holes in the bone over the inner ear and close them with an artificial filler called bone wax.
The entire procedure lasts roughly 90 minutes, and the results are usually instantaneous.
Getting Back to a Normal Life
It’s estimated that SSCD affects about one person per half million, but because it was only discovered 15 years ago, and because many physicians still aren’t familiar with the symptoms, there may be many more patients who have SSCD and simply don’t know it.
The condition usually affects middle-aged adults, but there have been cases reported in children.
Dr. Yang and I are seeing a growing number of cases, and patients from around the country are coming to see us at UCLA. Today, we see as many as 10 new cases a month.
It may still be relatively rare, but this minimally invasive keyhole approach to surgery makes an enormous difference in the lives of these patients. Not only do they recover and get back to their lives much faster, but they do so with a profound appreciation for a peaceful silence they haven’t experienced in years.
Dr. Quinton Gopen is an ear surgeon at Ronald Reagan UCLA Medical Center.
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A New Treatment for Super Semicircular Canal Dehiscence originally appeared on usnews.com
