“Sorry But You Are Inoperable!”
“You have the disease I suspected. It is very rare. I believe that less than 2 percent of the population suffers from this disorder.”
My oto-neurologist hesitated for a moment. “You have Semicircular Superior Canal Dehiscence, and it seems your right ear is worse than the left. The only way to correct this situation and give you somewhat of a normal life is to undergo a craniotomy. We would have to drill a hole into your skull…lift your brain…drill to the dehiscence in the superior canal and fill the hole,” he continued.
My eyes shot open wide, I thought craniotomies were for people with head problems and not for people like me with an inner ear disorder. I bit my lower lip harder; I could feel the color drain from my face.
“You have the dehiscence in both ears. You have holes in both of your superior canals…opening in your temporal lobes. I’ve not seen many people with this. I believe that less than one thousand people are diagnosed with this disorder.”
Never in my wildest dreams had I ever imagined such a disease existed. My surgeon continued talking but his words were gibberish. He reached over and touched my hand. His words were soft but strong, “I am sorry, I am so sorry, but you are not a candidate for surgery. I have spoken with several other surgeons. You have other vestibular problems that would not benefit from the surgery. It seems as if you also have Meniere’s Disease and it, combined with the Superior Canal Dehiscence, makes your condition even more rare…”
“But what can I do, I cannot live like this,” I began to nervously twirl my hair. Breathe, I told myself, just breathe.
“There is medicine that might help you, and of course over time maybe new technology, and of course with research…you will just have to learn to deal with it one way or the other,” his soft and compassionate voice began to fade. I cradled my head with my hands, and began to think back to the night that started this whole nightmare.
Three years ago, I woke in the middle of the night to a strange sound, and suddenly the bedroom was spinning, a literal vertigo experience. I was laying still but the whole room, including the bed, was wildly spinning around me. My stomach had started to convulse, and I had this horrible buzzing and ringing sound in my ears. I had tried to sit up but any movement caused another vertigo episode.
“Help me,” I reached for my daughter’s hand. I found myself almost paralyzed; even the smallest and slightest movement was a gigantic effort. “Help me, please,” I managed to grasp one of her long delicate fingers. “Please help me,” I cried out in the dark. My voice petered out; trying to speak was overwhelming. My daughter turned on the night light near the bed. The immediate brightness and the clicking sound of the lamp’s knob made the vertigo worse. “No,” I uttered, “it hurts.” I closed my eyes and forced myself not to wretch.
That night began the four-month journey to find an answer. Every night I petitioned Jesus for an answer and for healing. I also petitioned God to let me live.
A possible brain tumor or stroke was the first suspected cause of my sudden illness. Head trauma, Alzheimer’s, or maybe Multiple Sclerosis where other thoughts.
A MRI ruled out any immediate dangers or threats of tumors, but every time I presented my symptoms to a new and yet another specialist, they would look at me as if I was crazy. “You see doctor,” I would nervously begin to voice my string of symptoms, “I am nauseated all of the time. I sometimes get vertigo that drops me to my knees. I have a ringing and buzzing sound constantly in my ears. Any pressure change causes me to get disorientated, my memory is fading, and my hearing is radically fluctuating.”
“I also hear noises and sounds that cause me to become confused, and sometimes the vertigo gets so bad that I actually drop to the ground,” timidly I would look at the doctor for support. By this time the doctor would be scribbling notes and nodding his head up and down and sometimes to the side. But the last symptoms always threw him for a loop. “I get lost between time and space. It’s like I am in a slow-motion movie, and no one is moving except me or objects that I know are stationary. It’s almost like I’m watching the world around me, and I can see people’s lips moving but cannot understand.” This symptom would always get a raised eyebrow or a scowl.
I would take a deep breath and force myself to continue, even though I could tell by the doctor’s expression that he was now beginning to classify me as a mental case. “And I can hear my own pulse, my own heart beat, and other internal and external noises are magnified to the point that they cause pain…and vertigo.”
By now the doctor would be scribbling on his prescription pad. He would usually end the counseling session at that point and say that he wanted me to see the psychiatrist, then hand me several anti-psychotic or anti-depressant drug prescriptions.
“No,” I would shake my head, “I am not crazy. You don’t understand. I have several psychology degrees and I know I am not a mental case.” But I guess the six or seven degrees the doctor held meant more than mine.
“I am not saying you are crazy. You definitely have vestibular problems and head trauma,” would be his usual consensus, “but I have never heard of such symptoms and…” For months this is what I heard several doctors say, or something similar.
One evening I was working on my laptop when a strong urge came over me to search for another doctor. “Dear Lord,” I began to silently pray, “I am lost, please help me.”
Several minutes later I found the Washington University - Barnes Jewish Hospital website. There was a picture of a middle-aged man. He had a kind face and was very distinguished looking. He resume was impressive and his credentials were remarkable. I decided to have someone call and make an appointment for me. I was so tired of this disease or whatever I had. Most days were hard but some days were unbearable.
Three long weeks later I found myself in Dr. Chole’s office. When I met him, I was pleased with his soft voice and kindness. Instead of verbally telling him my symptoms, I handed him a typed list. Stuttering and memory problems had sometimes plagued me since the onset of this disease. He sat quietly and read my two sheets of symptoms. When he finished, he turned the paper over and asked me to tell him in my own words what was going on. I gave him the reader’s digest version. I wasn’t going to tell him about the really weird symptoms, but something inside made me blurt them out. “I get lost between time and space and can hear my own pulse,” wide eyed I looked at him. “But I am not crazy and I don’t need a psychiatrist, shrink, or medication…”
Doctor Chole held up his hand to quiet me. “I know you are not crazy,” his voice was filled with concern and compassion. “Let’s run some tests and see what is going on,” he picked up his pen and began to write in my chart. “I think you have vestibular problems and possibly Meniere’s Disease.”
He motioned for his nurse, “Let’s get started right away.”
“Hand me your MRI films,” he reached for the films that were lying across my lap. “Let’s have a look.” He flicked on a light switch of the x-ray machine and motioned for me to look with him. Dr. Chole noticed my eyes jumping. He handed me a funny looking pair of goggles. “Put them on,” he said. He positioned my head forward and then clapped his hands. He held my head in his hands and watched my eyes for several moments. “You have nystagmus. Your eyes jump up and down, but they also jump upward and away from the sound…going in the wrong direction…causing the illusion of movement.”
I was shocked he was including me. I was more stunned that he actually looked at every film and every slide. He didn’t rush through them as other doctors had done. “No tumors, no MS,” he began to rule out possible diseases. He reached for the last film. “You have head trauma but I don’t see anything too terrible.” “Wait,” he pushed on his glasses, “right there,” he pointed to the last slide on the last film, “I think we might have found something.”
“Right here,” he traced the film with his finger. “This area should be bone but it’s not,” he flicked the light off. “What I think you have is a very rare disorder where you are lacking bone, a dehiscence, a hole over your inner ear balance canals. This disease is very rare but I have seen it before,” he assured me.
“What happens is that your ears send the wrong signal to your brain and thus the illusion of movement. It plays havoc with your brain, balance, equilibrium, and other cognitive skills. We need to do a high resolution cat scan and run vestibular and other tests.”
“Thank you, God,” I said quietly. “God bless you, Dr. Chole.”
A month later, and after numerous vestibular tests and a very sophisticated cat scan, I was diagnosed with a very rare disease — Semicircular Superior Canal Dehiscence - SCD. It is believed that less than one thousand people suffer with it. Dr. Lloyd Minor of John Hopkins University is the man credited for discovering this rare disease.
I also have Meniere’s Disease, which is another rare and disabling inner-ear/vestibular disorder/disease. There are very few ENTs, surgeons, doctors, or hospitals that have seen or dealt with these diseases. I was fortunate enough to find a doctor who had actually seen two other cases before me, and who knew Dr. Minor and was very familiar with his research.
There is not a fairy-tale ending to this story. I am not a candidate for surgery and my symptoms are still the same. Life with this disease and other vestibular problems can be devastating, and the only prayers I can utter are for mercy. Even though I have not been healed physically, I have done a tremendous amount of spiritual healing. I am drawn to Jesus every day and know that I will never survive these disorders without him and his love. I am so very blessed though. I have wonderful children and fabulous friends who are very supportive and nurturing. I love my children very much, and they are such a blessing even though the change they see in their mother is so very difficult for them to deal with.
These diseases are very humbling, but if they keep me closer to my Jesus, then that is where I need to be. I cannot fall with Jesus; I can only stumble to my knees in prayer.
Hugs and Prayers