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Posted by Makala Johnson (@makalajohnson) · Jan 15, 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

Wendy TapperOutgoing 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.”

Related Departments

Balance Dr. Charles Beatty Dr. Daniel Blum ear Hearing otorhinolaryngology Round Window Occlusion Semicircular Canal Dehiscence Syndrome Surgery Wendy Tapper

 

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Rose (@rcook68) · Fri, Mar 21 at 2:40pm CST · #

I was recently diagnosed with SSCD and defect of the tegmen tympani. 54 days ago I began having pulsatile tinnitus in my left ear and after several doctor visits a CT scan from the ER revealed the problem. I am at a lost here in Orlando, Florida, as there are not many physicians aware of this condition. Any help in guiding me to perhaps a doctor in my area? I only have this one symptom and hope and pray it stays that way. I also do not want brain surgery, so any help would be so appreciated. Thank you, Rose

Barb (@barbhuffman) · Thu, Jun 26 at 8:48am CST · #

I also have semicircular canal dehiscence syndrome in both ears. I recently visited Mayo Clinic in Rochester where they were able to diagnose this. After reading your story, I am VERY curious as to whether or not I might be a candidate for this procedure instead of risking one of the other two. I teach music and English as a Second Language, so my hearing is extremely important to my being able to earn a living. Any suggestions on how to explore this option?

Mike Taylor (@bagguy) · Mon, Jun 30 at 8:05am CST · #

Good Morning,

Sounds like we are in the same boat. I was diagnosed about 7 months ago. have an appointment 7-7-14 for pre op check up. surgery the next day. going to do the round window occlusion out patient surgery. hoping good results. Dr Beatty is doing it. after doing probably too much research on the internet(probably like you) this seems like the first thing to try. like it because it is reversible if I am not happy.

Bagguy

Mike Taylor (@bagguy) · Mon, Jun 30 at 8:07am CST · #

would love to hear from Wendy and get an update

Robert (@robnor1) · Wed, Jul 30 at 1:35pm CST · #

Mike,
I too have just been diagnosed with SCDS. How did your round window occlusion go?

stuart (@stulacey) · Wed, Aug 6 at 8:41am CST · #

My wife has been suffering from scsd for about 5 years. Unfortunately it seems to be getting worse. With being a mother to four kids its getting close to being unmanageable.
I was very excited to read about Wendy's story and her recovery from scsd. I was amazed there is a way of fixing the problem without major surgery. We live in Australia and love to hear if this treatment is being performed in this country

Leaj (@leaj) · Mon, Sep 15 at 2:28am CST · #

I have just been diagnosed last Monday after a 12 year battle to find out what was wrong with me , I also live in Australia. I have it in both ears and have had balance issues my entire life , but the symptoms became debilitating about 12 years ago when I bought a property high on a mountain. It is only 889 meters above sea level, but I traveled daily to work and became increasingly ill after the move . I thought something on the property was poisoning me because of the dreadful migraines and upset stomach due to dizziness. I'm now 55 so the doctor diagnosed menopause even though I was 43 . When none of the treatments worked he sent me to a psychologist and then a psychiatrist . I became increasing ill , but no firm diagnosis. When I tried to explain my symptoms had been with me my whole life the doctor thought I was exaggerating and would not listen , he kept discounting them. Finally went to see a respected ENT specialist and he stuck his finger in my ear and proclaimed vestibular migraines , he had contributed to a paper on superior canal dehiscence , I find this astonishing. I live near Mackay in far north Queensland now and thought I would never find a doctor to diagnose my strange symptoms after going to so many in Sydney. I knew what I was experiencing was wrong, the migraines became unbearable and I've now developed 2 aneurysms in my brain. I'm so glad I persisted in my search . I almost canceled the appointment due to the amount of money I have spent so far. Dr David McIntosh in Mackay is my hero at the moment. I'm still trying to come to terms with how quickly he diagnosed me. Many tears have been shed in the last week through sheer relief , and I'm no longer doubting my sanity . I'm certain many people have experienced the same thing. Having grommets next Monday to relieve the pressure in my ears , then not sure where I go from there. All I know is I now get a chance to retrieve my self esteem and sanity.

Kelley Sregor (@sregoryellek2) · Mon, Oct 13 at 2:13pm CST · #

Lea J, can you update any progress you made please. thank you.