Mindy's Otosclerosis and Superior Canal Dehiscence Syndrome (SCDS)

In the fall of 2002, I began experiencing strange and very annoying sounds in my left ear. I thought that I had some fluid trapped in the ear and after waiting for several months and allowing the problem to progress to a point where I could no longer ignore it, I made an appointment with my doctor. He could not see any fluid and said that it could be deep within the ear. He prescribed some decongestants and told me to give him a call in two weeks if they did not help. They didn't, so I called him back. He referred me to an ENT (Ear Nose Throat specialist). I went in for my ENT appointment expecting to get a different prescription. Unfortunately, that's not what happened.

After examining me, the doctor asked me to move to the audiology lab and have a full hearing screening. Once that was complete, we knew that I had a 50% hearing loss in my left ear and a 25% hearing loss in my right. YIKES! I had been aware for a few years that I was missing some conversation and about a year before this I had started watching TV with the closed captioning on. I attributed the need for closed captioning to the noise of my children playing, not to hearing loss. Now I was being told that I was wrong and that I had been hiding the fact that I was losing my hearing. The diagnosis was Otosclerosis. (Otosclerosis is a fusing of the inner ear bones. This causes it to not vibrate and transmit sound.) It was recommended that I have a stapedectomy on my left ear and wait for my hearing to degenerate in the right before taking action. This surgery would replace my fused stapes bone with a prosthetic bone. I agree to go ahead with the surgery and was scheduled for November of 2002.

While the stapedectomy was a success, I had lingering symptoms from the surgery that were of grave concern. I had no improvement in my hearing. Sounds irritated me as if someone were walking around behind me with a chalkboard to my ear and scrapping their fingernails down it. Noise input actually made me dizzy and nauseous. I couldn't sit in church while the music was playing. I had a hard time staying in the room my children were in. Even going to the mall was far to noisy for me. I was actually to the point of not being able to function. My ENT referred me to the University of Maryland Medical System. After an initial visit, a cat scan and a VEMPS test, I was diagnosed with SCDS (Superior Canal Dehiscence Syndrome). This is a disorder in which the bones in the superior canal develop thinner than normal. When a trauma occurs (a trauma can be anything from hitting your head to a cough fit or large sneeze), the bone is to thin to handle it and it breaks away. In my case, the stapedectomy procedure caused the trauma to my already thin bone and the bone broke away. You have three canals in your inner ear. They control your balance. Each is at a slightly different angle and has spinal fluid moving through it. The canal sends signals to the brain based on the movement of the fluid as to how to balance you. Because of the hole in my superior canal, the brain was getting the wrong messages.

Dr. Staecker recommended surgery. The surgery would be cranial and in it he would attempt to "patch" with a bone graph the area of missing bone. I had the surgery preformed and for the first 4 weeks I was symptom free. But then the symptoms returned. Apparently, the bone graph slipped and I was back to square one. After much research and conversation, Dr. Staecker recommended that I undergo a second cranial surgery. This time he would fill the superior canal with bone wax and make it completely disabled. I had this second surgery preformed in June 2003 and the recovery was tough. I was in the ICU for 2 days and during that time I could not even move my eyes without getting sick. I couldn't lift my head up off the pillow even a fraction of an inch. I stayed in the same position without moving a single muscle in my body because any movement caused me to get sick. Phil had to feed me because I couldn't look down at my plate or follow the fork to my mouth. I spent a couple of months slowly improving and today I'm glad I had the surgery. My hearing loss has not improved, but I can tolerate life again. There are many side effects of SCDS, even post-op, that I will have to tolerate for the rest of my life, but I'm a lot better off now. Today, barometric pressure changes, looking up or bending down, lifting heavy items, and certain motions cause me nausea and virtego. I have problems in what I call "clutter" conditions such as super Walmarts with large open spaces and lots of activity. Most regular noises are now fine for me, but I can't tolerate loud vibrating sounds (i.e.: motorcycles, certain engines, loud bass in cars, airplanes, etc.) and high pitched noises are bad. While I may look perfectly healthy, I'm probably feeling very ill in those situations and these post-op symptoms do cause me to modify what I'm able to do. However, I am able to do 90% of the things I could not do pre-op. I've posted pictures from my first cranial surgery below. They are graphic.

When all of this first started happening to me, I looked all over the web for other people who have experienced what I was experiencing and I couldn't find it. So I decided to create a SCDS support. If you have been diagnosed, think you might have SCDS or know someone who does, please feel free to join our group.

WARNING GRAPHIC PICTURES BELOW!!!!

Mindy's skull. The square area is the skull bone that was lifted out to perform the procedure.

Above the tools is Mindy's brain being pushed out of the way, below the yellowish area is the superior canal. The line you see on the canal is the actual dehiscence.

This is the inner ear as seen by the back of the ear being cut and pulled up. The silver and white piece is the prosthetic stapes bone.

The actual incision, three days post-op.