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January 15, 2013

Unraveling the Mystery of Semicircular Canal Dehiscence Syndrome

By MakalaArce

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

Tags: 5267, 5335, balance, Dr. Charles Beatty, Dr. Daniel Blum, ear, ENT/Audiology, hearing, otorhinolaryngology, Round Window Occlusion, Semicircular Canal Dehiscence Syndrome, Surgery, Wendy Tapper

Try contacting The Silverstein Institute in Sarasota, FL for alternative approach to surgery to alleviate symptoms of SCDS! Look at website.

COMMENT

I am currently seeing the same doc you saw at the U of MN. He said the exact same words to me! I am not terribly impressed with him. He is very knowledgable I think but he has an attitude that I’m stupid and he’s the only smart one in the room. Might have to see someone at Mayo. I have the same symptoms as everyone else. I don’t really think this is as rare as they say. It was only given a name a few years ago and many physicians aren’t real familiar with the symptoms so it is being misdiagnosed. I have it in both ears but my left ear is the worst. I don’t have a lot of dizziness but some. Who would be a good dr to see at mayo in Rochester,MN.?

COMMENT

Hello,

I’m so sorry about the U of M. I know how I felt after that horrible experience. One just feels lost.

I’m a poor one to be giving advice because I’m in a quandary myself as to the direction I want to take. But I do have a couple of suggestions. . .

First, (if you already haven’t) join the Superior Canal Dehiscence Syndrome Support Group (SCDS) on Facebook. It is a closed group. There I have found compassion mixed with great information. The leader of the group monitors the postings very closely. She also has a good section of information on the page under, “Files.” I have found that there is no, “Stupid question.” I also have found that no two cases of SCDS are the same.

Second, my insurance company declined my request to be evaluated and treated at Johns Hopkins. I wanted to see Dr. Carey. My insurance will cover Mayo, but as you can see by this site, not all Mayo doctors have the expertise to treat this syndrome. However, I did read this on a site: . . . . had my surgery at Mayo Rochester and I am extremely happy with my results. Dr Neff does quite a few of these each year and really seemed to know what he was doing. I had the Dr Neff/Dr Link combo.

Again, so sorry about your experience at the U. Reading your e-mail just brought back all of the negative, confusing feelings.

If you do go to Mayo, keep me posted. I’m strongly considering it myself.

All the best.

COMMENT

Hi there, I am new to this site and I am very curious about your situation. I had transmastoid surgery at Mayo Phoenix this year for a cholesteatoma in my ear…..but they discovered I have SCCD when doing scans for the other issue. I think the first surgery did some more damage, because I did not have symptoms before but I do have some now. Overall, I have not been happy with Mayo and if I have to have surgery for SCCD I definitely do not want to have it there. Can you tell me who your doctors where that performed your surgery? I am sorry that you have had such a hard time 🙁

COMMENT

I went to Mayo and ended up seeing someone who doesn’t do that surgery. Really irritated me that I told them exactly what my diagnosis was and I wanted to see someone very familiar with it. Instead they give me a novice. I’m back at square one. Very frustrating. I’m done with mayo in Rochester.

COMMENT

I was just diagnosed and am starting my journey..was curious how your treatment has been going

COMMENT

I have no where to turn to fix this SCDS problem because of what they did to me by amputating that part of the skull!! I went

completely crazy after the first surgery because of all the new symptoms and loss of hearing that I was dealing with.. The doctor said on the first revision on the left side that the plug was missing in action. He told me this in front of my mother and myself and then he said he did not remember saying that. At this point since they will do nothing to help me I am going to speak with my attorney and proceed with litigation!! My life is completely ruined. I cannot go to restaurants or to the grocery store or anywhere where there is a lot of noise!! I have the worst migraines and clusters that is beyond anyone’s imagination! The pain is so awful that I start vomiting! I have had these last over four days continuously!! I take several medications just to try to cope with what I experience every single day every single minute!! I do not think that they were properly trained to do this type of surgery! I asked both surgeons how many surgeries that they had performed and they told me six or seven!! My vision is terrible along with vertigo, wooshers, nystagmus basically every single symptom I have and it has destroyed my life!! I see an outside neurologist and a neurosurgeon and he told me that I was SOL and he was very angry with mayo hospital and the doctors! At the same time I was experiencing trigeminal neuralgia so he advised me to have the mayo hospital decompress the nerve away from the artery since they were going to be right there while they were in my head performing the trans mastoid surgery and they said that they could not do that!! The second revision surgery was performed December 22 of 2015 and he said he had to pack an air cell and everything else looked fine from the previous revision surgery! If they would have performed the middle fossa approach they would have seen everything much better. I tried to perform The tests before the revision I failed horribly and got terribly sick and had to leave! My CT scan and radiology report still show a dehiscence on both sides! I am not writing all of this to scare anyone or give negative feedback I just want everyone to know that this could change your life and you will be handicapped for the rest of your life as I am now and have been since 2009!! I wish all of you the best and if you have anymore questions please feel free to ask..

Respectfully, Tab

COMMENT

Thank you so much.

COMMENT

This is documented in multiple site study that you can find on website….very well known in Sarasota area….where I went but not severe enough to try any surgery…..good luck….nice facility…study as much as you can but it might be a solution for day surgery only….I believe for partial closure of round window and the hole in semicircular canal then supposedly takes over the function of the round window…..releasing the traveling wave. look into it as alternative…..I got mine at age 65 and am mostly retired and can live with it…..I would also guess that having balance problems might make one move on surgery also which thank you, God, I do not have and hopefully will not get. But being musician could be a problem. During the height of my experience with it, I was at two holiday concerts and I had echoing of singers and also could not sing myself. This was before diagnosis….and I did not know why and I thought I was nutsy……again good luck.

COMMENT

Thanks for the encouragement. I will join the facebook group. It’s always good to have info and support from those that understand. My family is great but I don’t think they understand.

COMMENT

After 5 years and visits to many doctors, I’m finally on my way — surgery is scheduled for April (taking the conservative approach w/ tympanotomy & repair of round window). Dr. Haverkamp at the Cleveland Clinic is amazing; he and his team zeroed in on the SCD diagnosis right away, despite at least 4 other ENTs hearing me describe the exact same symptoms and diagnosing me with everything ranging from otosclerosis to basically “having the vapors”!

Everyone I’ve worked with at Cleveland Clinic, from ENT to audiology to vestibular testing to CT imaging, has been just amazing. I know this is a Mayo Clinic site, and I’ve seen references to other hospitals like Johns Hopkins here too, but I just wanted to let people know that there are other places out there that have the expertise to help you.

It was nice to cry tears of happiness instead of frustration after a doctor’s visit.

COMMENT

Hi I was recently diagnosed with Superior Semicircle Canal Dehiscence. I am a hairdresser and a mother of 2 little ones. I am waiting to get into a another specialist but have to wait 2 months to get into. I have the heartbeat in my right ear, chronic headaches. and balance problems. I was reading through some of these and now am a little overwhelmed about my future options. Also is there anything you have tried that helps with symptoms? I find that sitting and laying down make the heartbeat and pressure in my ear and head worse. any advice good and bad stories would be appreciative as I start my new journey.

COMMENT
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