makalajohnson (@makala johnson) published a blog post · January 15th, 2013
Unraveling the Mystery of Semicircular Canal Dehiscence Syndrome
Imagine listening in real time to the thump, thump of your own heartbeat, the rush of your blood pulsing through your veins, and even the slightest twitch of your eyes - all in surround sound. Those are but a few of the symptoms that Wendy Tapper was experiencing when she arrived at the Mayo Clinic in May of 2012.
The Journey to Mayo
Outgoing and energetic Wendy, of Kansas City, Mo., enjoyed a career as a producer and publicist. Bringing people and ideas together was second nature to Wendy and aided in her determination to find the answers in her own health care.
For three years prior to coming to Mayo Clinic in spring 2012, Wendy went from doctor to doctor and endured batteries of tests, scans, appointments and misdiagnoses. Her rare condition ultimately revealed by Mayo physicians was masked in part by two distinct illnesses - breast cancer and a stroke.
While those illnesses and the treatments Wendy was receiving are life-altering, they were compounded with the escalation of an underlying third and separate issue. It was the escalation of her symptoms of dizziness, hearing loss and a drastically diminishing quality of life that brought Wendy to Mayo Clinic.
One Simple Question
Wendy met with Dr. Daniel Blum, an otolaryngologist (ear, nose and throat specialist). After a series of scans and balance tests, the scan revealed the absence of bone near her ear superior semicircular canal.
That particular bone provides a protective barrier and helps to insulate the critical centers of hearing and balance from the brain. Dr. Blum immediately reached out to his colleague, Dr. Charles Beatty, an otologic surgeon.
Soon, Wendy was sitting in his office while he reviewed her scans and hearing test results. She was not responding to soft sounds. Her low tone hearing loss could be a symptom of many different conditions. But it was a critical piece of the diagnostic puzzle for her medical team.
After consulting with the audiologist, Dr. Beatty asked an unusual question, “Can you hear your eyes move?” and the mystery began to unfold. Wendy promptly responded, “Yes!”
This rare disease is called Semicircular Canal Dehiscence Syndrome, which means that there is an opening or no separation between the upper inner ear canals and the brain. First documented in 1998, the number of diagnosed cases at Mayo Clinic is less than 75. Wendy’s condition was even rarer, having the condition bilaterally, in both ear canals.
Jamming the Circuits
To better understand the condition, without that particular insulating bone to act as a barrier, Wendy’s brain was receiving many faulty messages regarding balance and hearing. It was this misinformation and information overload that caused her auditory, visual, and cognitive processes to compete for airtime in her brain, essentially jamming her brain signals. This is what had caused an eruption of symptoms such as imbalance, cognitive dissidence, and hypersensitivity to internal sounds over these last three years.
Weighing the Risks
While there are two standard invasive surgical procedures used to treat the disorder, both carried risks of hearing loss or further balance problems.
Dr. Beatty presented a third option — Round Window Occlusion — the less invasive surgical procedure which involves going through the external ear canal instead of the skull. Dr. Beatty was only aware of it being performed once before and it had never been done at Mayo Clinic.
The most attractive part of this option was that it was reversible if there was no improvement.
While still infrequent, the people that have the more invasive surgery have a much longer recovery and risk permanent hearing loss. Dr. Beatty’s minimally-invasive surgery was performed as an outpatient procedure, but as a precaution, Wendy’s medical team monitored her in the hospital for 24 hours.
Wendy and her husband Eddie have a solid partnership. They have taken each health challenge with focus and determination to help Wendy resume her full personal and professional life.
“It Changed My Life”
Like so much of medicine, the answers were tucked in across disciplines. Each person from technician to clinician to surgeon brought a critical piece of information to the diagnostic and treatment puzzle.
Since the creation of that barrier to slow the inner ear messaging to Wendy’s brain, she has been able to resume her active life. She encourages others by sharing her story on blogs and message boards with other patients as far away as Germany.
Wendy tears up when she speaks of Drs. Beatty, Blum and her entire Mayo Clinic experience, “When you say the needs of the patient come first, you really mean it,” she says. “Finally finding the answers at Mayo Clinic changed my life.”