Sharing Mayo Clinic

Stories from patients, family, friends and Mayo Clinic staff

Share this:
MakalaJohnson

Unraveling the Mystery of Semicircular Canal Dehiscence Syndrome

Posted by @MakalaJohnson, Jan 15, 2013

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: balance, Dr. Charles Beatty, Dr. Daniel Blum, ear, Hearing, otorhinolaryngology, Round Window Occlusion, Semicircular Canal Dehiscence Syndrome, surgery, Wendy Tapper

Please login or become a member to post a comment.

rcook68

Posted by @rcook68, Mar 21, 2014

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

barbhuffman

Posted by @barbhuffman, Jun 26, 2014

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?

bagguy

Posted by @bagguy, Jun 30, 2014

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

bagguy

Posted by @bagguy, Jun 30, 2014

would love to hear from Wendy and get an update

robnor1

Posted by @robnor1, Jul 30, 2014

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

stulacey

Posted by @stulacey, Aug 6, 2014

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

Posted by @leaj, Sep 15, 2014

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.

sregoryellek2

Posted by @sregoryellek2, Oct 13, 2014

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

tabkellem

Posted by @tabkellem, Feb 8, 2015

Hello Wendy, I read your story and i know exactly what you have and are going through! I am suffering very bad and i once again am reaching out for help here in Az!! I wish you the best of health and may God bless you!!!

Sincerely,Tab

leftscds

Posted by @leftscds, Wed, Jan 6 at 4:45pm CDT

Would like to hear from Bagguy regarding the newest procedure outcome relative to the partial and reversible surgical option from 7/14. I was just diagnosed today and this is the option that was offered but since so new, cannot yet find but one article from otologist who does. Have seen that John Hopkins does not do yet? and that is where Dr. Minor works....any input on the third procedure outcomes? My main problem is tinnitus (constant) and heart beat in left ear....some autophony and some trouble focusing eyes at times.....thanks.

singergirl

Posted by @singergirl, Wed, Mar 30 at 6:33pm CDT

Hi, I was just diagnosed in September. I'm thinking Mayo or Johns Hopkins. 2 entirely different procedures. May I ask what you have chosen to do? Or did? Thanks.

leftscds

Posted by @leftscds, Wed, Mar 30 at 6:55pm CDT

Hello, I have not chosen anything to date....since this Jan 2016 diagnosis.....still have heartbeat and tinnitus left ear, but hearing has improved and the echoing only for certain low frequency sounds. So, likely unless it gets worse, or if I have balance involved, I will remain at status quo........still have not heard anything about partial closure success/failure of round window occlusion/partial occlusion as current gold standard so still hesitant to decide....hope to remain stable. Good luck...but physician (Otologist) in my area, seemed to recognize and diagnose right after acute otitis media resolved....

singergirl

Posted by @singergirl, Wed, Mar 30 at 7:08pm CDT

Thank you for the quick response. I chose to write to you because your
symptoms mimic mine. Unfortunately I am a music teacher and singer and the
low tones drive me crazy in my head. I have to get it fixed or I can't
continue teaching. If I had any other sort of job it might be tolerable. I
have an appointment in a couple of weeks to decide what I'm going to do.
Take care.

tabkellem

Posted by @tabkellem, Thu, Mar 31 at 2:44am CDT

Hello my name is Tab!!
I want to give you some advice about surgery options! I had Transmastoid,
mini middle fossa, resurfacing and reinforcing the Dura floor. The last two
surgeries were revisions on the left side. Second revision on the left was
just to go in and look at previous plugging procedures! That was performed
December 22 of 2015 and the revision before that was on June 9, 2013. The
surgeries were performed at mayo clinic Hospital in Phoenix Arizona!! The
two surgeons and myself agreed on a middle fossa approach and when I woke
after surgery that was not the case!! My symptoms are so horrific that it
has left me disabled permanently. They amputated that part of the skull and
now nothing can ever be done to repair it. All it did was basically make a
bigger hole!! When the first revision was performed on the left side of
June 9, 2013 the surgeon told me that the plug was missing from the first
surgery in 2010! My mother was with me for the follow up after the revision
surgery and he says he doesn't remember saying that to me but my mom also
heard the same thing come out of his mouth!! The best advice I can give you
is to go to UCLA and have surgery with Dr. Yang and Dr. Gopen!! If your
symptoms are not too terrible I would recommend not having any surgery
because this will completely change your life!! Dr. Gopen went back to
school to learn how to properly perform the resurfacing procedure! He does
not do any trans mastoid that I am aware of because it is not proper that
is why he does resurfacing only!! Instead of having a big scar on your head
they now do a dime size Hole procedure that is less invasive!!

Anything further I will do anything I can help you with!!
I wish you the best of luck keep in mind this is a very important decision
to make!! Take care of yourself!

Tab

--
Sent from Gmail Mobile

singergirl

Posted by @singergirl, Thu, Mar 31 at 8:10pm CDT

​Dear Tab,

I'm so sorry to hear about your situation. Your response really threw me
for a loop . . . I was so excited about the prospect of not having to go to
a strange city and have my ear cut off, and brain pushed back. I'm
thankful that I'm not just hearing the gloss stories that only frame
successes.

I was going to get my work done at the University of Minnesota, however I
had one of their top doctors open the conversation with me by saying, "Yes,
you have Superior Canal Dehiscence, and trust me, you don't want the
cure." This was in November of 2015, and I haven't been to see a doctor
since. I've been just suffering through each day of teaching. My worst
symptoms are autophony and ear fullness. Like I said, I'm a music
teacher, choir director and singer so the symptoms are very debilitating.

I'm at the very beginning of my journey, so I'm not familiar with all of
the lingo. Just to be clear - you had the surgery where they go in through
the hole in your ear?

I was going to see Dr. Yang, until I read about some very unfavorable
post-surgical protocols from UCLA, as well as the monitoring of websites
and removal of negative posts.

Thanks again for your response.

leftscds

Posted by @leftscds, Fri, Apr 1 at 8:11am CDT

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

tabkellem

Posted by @tabkellem, Fri, Apr 1 at 9:19am CDT

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

singergirl

Posted by @singergirl, Fri, Apr 1 at 10:37am CDT

Thank you so much.

leftscds

Posted by @leftscds, Fri, Apr 1 at 11:07am CDT

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.

Contact Us · Privacy Policy