Our son, Nicholas, was treated by Dr. Gianoli for superior canal dehiscence syndrome in 2011 when he was 8 years old.
We first noticed Nick’s extreme sensitivity to noise when he was just a few months old. He would cry when there were loud sounds, and if it was really loud, he would even break out in hives from the stress. Since it isn’t uncommon for babies to be upset by loud noises, we hoped that he would outgrow it, but we knew deep down that something wasn’t right.
By the time he was two, he was having a hard time even being in situations that would cause him to anticipate noise. We couldn’t spend time with other kids, because the unpredictable and loud nature of toddler sounds were too much for Nick to handle. We turned to an occupational therapist who diagnosed him with sensory processing disorder and started treatment for that. OT helped him cope better with noise, and it addressed the secondary issues that were unknowingly being caused by SCDS, like low muscle tone; he didn’t move around as much as other kids for fear of dizziness. It was still hard for Nick to be around noise and other kids, though. Preschool drop-off was usually really tough for him. We still didn’t really have an answer as to why noise was so hard for him to tolerate.
In kindergarten, school started going better, because all of the kids were getting a little older and quieter. Things seemed to be improving, but in the winter, Nick started being bothered by noise again. He started resisting going to school and was under a lot of distress.
The following year, Nick started doing “astronaut training” at OT, which is a vestibular exercise that involves spinning on a board. He started complaining of lasting dizziness when he would lie down in bed. The therapist told us this might happen. When I asked him when it started, though, he told me he had always gotten dizzy when lying down.
An audiologist suggested that he be evaluated by an ENT. When I brought him to the appointment, I remember thinking that, as usual, we probably wouldn’t get any more answers, but we should at least have him checked out. To my surprise, after the doctor examined Nick and asked him some interesting questions, like whether he could hear his eyeballs move or his heart beating, she said to me “I think he has a condition called superior canal dehiscence syndrome.” She explained some further testing that was needed to confirm the diagnosis and suggested that we didn’t need to do anything right now since he was functioning well.
We did the additional testing, which all pointed to SCDS, but we held off on the CT scan, because Nick was symptom-free at that time. The next winter, his symptoms came back with a vengeance. He could no longer go to school, and noise was becoming unbearable. We had the CT scan, which confirmed SCDS.
I started researching the condition online. The ENT had mentioned an online support group, which I found. It was there that I first heard about Dr. Gianoli. We live up in Massachusetts, but the patient stories about Dr. G were so compelling that we knew we needed to meet him. We scheduled an evaluation down in Louisiana and were very impressed with Dr. Gianoli’s knowledge, approach and compassion. We returned about a month later for Nick’s first surgery and then three months later for the second.
Nick has truly gotten his life back thanks to Dr. Gianoli. He’s a happy, healthy, active 11-year-old who does well in school, plays sports and guitar and is surrounded by nice friends.
When you have a condition like SCDS, even after surgery, there are some precautions you must take and some limitations you may have to accept. Dr. Gianoli explained to us what life would be like after surgery. He has always been there to answer any questions by email or phone since the operations. I feel like he is a partner to us as we continue to watch Nick grow and develop, and things do come up occasionally that we need to ask him about. We feel like we have a lasting relationship with him, and, as a parent, that is priceless.
Dr. Gianoli helped give me my life back! I say this, because, during my fourth pregnancy, I started experiencing symptoms, such as dizziness, extreme light sensitivity, fullness and pressure in the ears. After the birth of my child, I started hearing my eyes moving in my head, my heartbeat in my head, footsteps in my head, autophony, and tulio’s phenomenon, along with many other symptoms. My quality of life quickly diminished. Not only was I not able to work anymore, but I was also unable to take care of my newborn child and my three other children. This threw me into a deep depression and extreme anxiety from the symptoms I was experiencing. After many hospitalizations during my pregnancy and many doctors visits afterwards, I was told that it was ͞all in my head,͟ and it would go away; at one point, they were even suggesting that I commit myself into a behavioral unit (they thought I was crazy). I had seen a total of 21 doctors who couldn’t diagnose me. I refused to give up on myself, but with the support of my family and friends, I continued to research. I finally came across SCDS. After my seventh ENT doctor who listened to me and gave me a special CT scan, I was actually diagnosed with SCDS.
I was then directed to the University of Michigan Otolaryngology Department. I had my first surgery there on January 3, 2011. I had a mid-fossa craniotomy with plugging surgery done on my left side. Unfortunately, the surgery failed. In the process they had accidentally compressed my other canals and left me with very little balance to no balance. I could hardly walk or see straight. My brain was unable to compensate for the loss of balance without the other side being repaired. The plugging technique was not an option for me on the other side. I needed to preserve my other canal’s balance function in order to live a somewhat normal life. I felt at a loss. I knew of no other doctor that did this specific type of surgery. With much prayer and more research, I found Dr. Gianoli. From what I researched, he had the MOST experience and knowledge with this specific condition. He also only resurfaces, which keeps the canals functioning intact. I did not hesitate to schedule with him. I was desperate to have my life back!
My surgery with Dr. Gianoli was on April 28, 2011. I traveled with my husband by car to have testing and surgery. Dr. Gianoli is the kindest, most compassionate doctor I have ever met! He took a lot of time with me as a patient and was very thorough, going through everything and answering all of my questions and making me feel like I was not a crazy person! His testing was very accurate and unique. His staff was very professional and they seemed to really want the best for me, not only as a patient, but as a person.
After my surgery with Dr. Gianoli, my recovery went much quicker and smoother than the first side. It wasn’t instant, but with time and patience, my brain compensated and I was living life again! Three months later, I was camping at the beach with my children! I still am symptomatic on my first time that has the significant loss of balance and still has a dehiscence. I am planning on a revision surgery with Dr. Gianoli in the future. There is no one else I would go to. I completely trust his skills and admire his genuine care for his patients.
In February 2011, our son, Úlfur (11 years old), was flown (at the cost of the Norwegian state) across the Atlantic from Oslo to Dr. Gianoli in Baton Rouge for a resurfacing of the left semicircular canal and reparation of a perilymph fistula. At that point, Úlfur had been unable to lift his head even a few inches off the bed for four months due to vertigo and consequent panic attacks. Eventually, he developed extreme sound sensitivity and started hearing his own body in a strange way, just to mention a few of his symptoms.
Úlfur woke up after surgery in a sitting position. Eight days later, all the symptoms from the inner ear had disappeared, and the family went on a pleasure journey to New Orleans, which included enjoying a river cruise on the Mississippi with a Dixieland band! Today, Úlfur is perfectly normal and healthy. If we believed in white magic, we would think Dr. Gianoli was the supreme craftsman of it.
Arna Mathiesen and Eyjólfur Emilsson
(the parents of Úlfur)
Norway family finds relief for son’s condition in Baton Rouge
By CAROL ANNE BLITZER
Advocate staff writer Published: Mar 22, 2011 – Page: 1D
On the evening of Nov. 3, 2010, 11-year-old Ulfur K. Eyjolfsson had the most frightening experience of his young life. Ulfur, whose nickname is Wolfie, suddenly became so dizzy that he could not stand by himself. What followed was a medical odyssey that led him from his home in Oslo, Norway, to a Baton Rouge physician with an international reputation for treating a rare condition of the inner ear. The morning after Wolfie’s dizzy spell, he was no better, so his parents took him to a doctor in Oslo. Two days later, he was hospitalized for a battery of medical tests. “Of course we were terrified,” said Wolfie’s father, Eyjolfur K. Emilsson, a professor of philosophy in Norway. “We thought of all kinds of horrifying things.”
The first diagnosis was a virus on the balance nerve, Emilsson said. The doctors said Wolfie would be better within a couple of weeks or so. Two weeks later, he was even worse. Then one evening after a bath, he got considerably worse. And he was beginning to have vision problems. ͞They put him back in the hospital for the same tests all over and new ones,” Emilsson said. The doctors even began to wonder if perhaps Wolfie’s condition was mental. His parents, who had been searching the Internet for answers, insisted that the doctors do a CT scan of his skull. Before they got the results of the scan, doctors decided that Wolfie might be having a leakage of cerebrospinal fluid. So around Christmas, they drilled a hole in his head to release (measure) the pressure. ͞That came to nothing in the end,” Emilsson said. In the meantime, Wolfie was suffering terribly. He was so dizzy that he could not lift his head one inch. “It was like being in a tiny boat on rough seas, even when lying down,” said Arna Mathiesen, Wolfie’s mother and an architect in Norway. He couldn’t sit up to eat, brush his teeth, take a sip of water or read. He couldn’t even use a pillow. Light and sound bothered him greatly. And he was disengaged. “When he was still in a room, he would be staring and not answering,” Mathiesen said. Finally around Jan. 20, an otolaryngologist examined the CT scan of Wolfie’s skull and saw a problem. He surmised that Wolfie had a rare medical condition of the inner ear called superior canal dehiscence syndrome, or SCDS, which was first described in medical literature in 1998 by Dr. Lloyd B. Minor at Johns Hopkins.
“The inner ear contains three semi-circular canals for balance. Each one is in a different plane,” said Dr. Gerard Gianoli. who with Dr. James S. Soileau operates the Ear and Balance Institute in Baton Rouge. “The canal at the top goes to the skull base,” Gianoli said. ͞The very top of the arch is at the top of the bone that forms the base of the skull.͟ In about 1 percent of the population, he said, the bone over the top of the arch does not form and the person is left with a hole in the canal. In most people, this does not cause a problem, but in a small number of people usually after the person has strained, pressure goes to the inner ear. ͞This leads to a whole confluence of very interesting symptoms,” Gianoli said. The condition was causing Wolfie’s extreme dizziness. Doctors in Oslo first recommended surgery in Scandinavia, but the procedure was not available.
Norway has a centralized government health-care system. ͞The system is that if you cannot get a service in Scandinavia that the doctor recommends, that you can have treatment somewhere else,” Emilsson
said. A search of the Internet and support sites for patients with SCDS led Wolfie’s family to Gianoli, who has operated on more than 150 patients with SCDS. ͞We asked for advice and read stories of other patients,͟ Mathiesen said. They talked with several of his patients in Europe and the United States. “For us, he was the obvious choice,” Emilsson said. ͞But it took some effort to convince the doctors in Norway. They had never heard of the doctor or of Baton Rouge. “Even without waiting for an answer from the Norwegian health system, Mathiesen e-mailed Gianoli. ͞I got a wonderful letter back in two hours,” she said. The family had decided that even if the health system did not pay for Wolfie to see Gianoli, the family would pay. They made an appointment and booked a flight. “We had to pay for 11 seats or three rows so Wolfie could lie flat on his back in the same direction as the plane was moving,” Emilsson said. Fortunately, the health system approved the family’s request and scheduled a medical jet ambulance to take Wolfie and his parents to Baton Rouge. They arrived on Feb. 26.
Emilsson has a lifelong friend teaching mathematics at LSU. He and Gestur Olafsson had grown up together in Iceland. Olafsson and his wife welcomed Wolfie’s family to their St. Gabriel home. On March 1, Wolfie saw Gianoli, who confirmed the diagnosis of SCDS at the Ear and Balance Institute. “We have one of the most complete testing labs including some really unique equipment,” Gianoli said. He also found that Wolfie had other conditions that accompany SCDS. One unusual condition is called “autophony,” whereby a patient may hear all sorts of noises, Gianoli said. Body sounds like heartbeats, chewing, creaking joints and a person’s own voice will seem unusually loud in the affected ear. “Some patients even report that they hear their eyes move,” Gianoli said. On the other hand, some patients develop hearing loss. “Sometimes a patient can get very position sensitive,” Gianoli said. Others may develop panic attacks. “Wolfie had all of this, but (the autophony was) not so extreme,” Mathiesen said.
On March 3, Gianoli performed a craniotomy on Wolfie. “We lifted up the temporal lobe of the brain to get to the skull base and put a bone graft over the hole and cemented it in place,” Gianoli said. “The cement eventually turns to bone.”The recovery was miraculous. ͞The day after the surgery, he sat up and he stood up and he walked. It was almost biblical,” said his mother with tears in her eyes. Wolfie stayed in the hospital for two days and then returned to Olafsson’s home. Each day following his surgery, he was less dizzy. Within two weeks, he was symptom-free. Wolfie has a few months of recuperation ahead of him. He can’t play soccer for three months. No heavy lifting for six weeks. No swimming for a while.
“In a few months, he should be able to do anything,” said Emilsson. Three weeks from their arrival, Wolfie and his parents returned to Norway. “On a regular flight,” Mathiesen said.
Six doctors, including a major research hospital with an international reputation, were unable to diagnose the cause of my debilitating dizziness, brain fog and fatigue. If it wasn’t for Dr. Gianoli, I would be on total disability and housebound from a superior semicircular canal dehiscence (SCD) in my right ear and perilymphatic fistulas in my left. His diagnostic tests and knowledge were more thorough than any I had encountered.
He has an incredible drive to keep learning and improving to provide the best care and treatment for his patients. I chose Dr. Gianoli to perform the surgery because he has pioneered and perfected the only technique (resurfacing) that preserves the function of the semicircular canals. To my knowledge, he is also the only surgeon who has recognized that stretched oval and round windows in the ear contribute significantly to dizziness and SCD symptoms, and they have reinforced them as part of the procedure. He is also kind, caring, easy to talk to, and spends time with his patients. An hour-long appointment is not uncommon. I am delighted to say that after Dr. Gianoli repaired both my ears, I had my life back!
Anne Chaka, Ph.D.
After five unsuccessful attempts by other surgeons across the country to patch my cerebral spinal fluid leak, Dr. Gianoli saved the day….and saved my life. I was able to finally go back to work and move on with my life, which had come to a screeching halt. He went above and beyond on every level and is simply a genius…I consider myself blessed to have found him.
Los Angeles, California
My daughter Mary is now 23 years old. Her problems began when she went on a family trip with us to Colorado in June of 2011. We drove from Louisiana, and she did not have any problems until we had a large elevation change. We have made many family trips, and she had never been car sick before. She was very nauseated and off balance. When we arrived she had difficulty walking and experienced headaches. We thought it was due to the altitude and thought it would pass with rest and fluids. She did improve, but this was the beginning of a long ordeal.
Following the trip to Colorado, Mary began a new job. She had two episodes of fainting, and we were not sure what caused this. Occasionally, she fell and had some nausea. She was seen by a neurologist, her primary physician, ENT and a cardiologist. They did not find any problem. Mary has a slight learning disability and a rare thyroid problem, but these weren’t new problems.
Symptoms progressed over the next two years, and she became more sensitive to sound and had more frequent problems with balance and nausea, but the doctors still didn’t have any answers. We waited months to see a neurotologist, and she had extensive balance and hearing tests and scans and was given new medications but still no diagnosis. Two years after the trip to Colorado, Mary was in a car accident that caused her symptoms to become much worse. Mary’s employer was very patient, but she was sent home many times and was eventually asked not to return to work due to the frequency of her falls, nausea and inability to tolerate noise. She often had to be assisted to walk, she was not able to tolerate riding in the car for even a short distance without nausea, and she was so sensitive to sound that even the sound of a fork on a plate caused severe ear pain and nausea. She started to use ear plugs daily to tolerate noise. Three rounds of physical therapy did not seem to improve her balance.
In Sept 2013, a church friend told me about his experience, and he described the same symptoms that Mary had. He saw Dr Gianoli and recommended him. We did not have to wait long for an appointment, and after testing and examining her, we finally had a diagnosis –semicircular canal dehiscence. She had surgery in November 2013, two and a half years after her problems began, and she is now doing very well. She had a problem with both sides, but surgery was done on the side that was more of a problem. She improved so much that she does not need to have the surgery on the other side at this time.
When we first arrived to see Dr Gianoli, Mary was not able to go anywhere in public ….she couldn’t go to a store, restaurant, church or even have dinner with the family. She took medication for nausea daily and severe ear pain, and we had to help her walk. Now almost 6 months after surgery, she is working full time and doing well. She still has occasional problems with her balance or nausea, particularly when she is tired or not feeling well. However, her quality of life has improved dramatically since her surgery. She will have to continue to be cautious about things like bending over and loud noises, but she is doing well and continues to make slow but steady progress. She can now enjoy life, and we are very grateful for the care and treatment we had from Dr Gianoli and his staff.
I think it is safe to say that most of us are truly inspired when we hear the story of someone who has persevered against all odds in life. So, when we heard about a special young lady named Camryn Detrick from our client friend and her dad, Norm Detrick, we knew it was a story our readers would enjoy and benefit from reading. After interviewing both Norm and Camryn, I thought long and hard about how to best share their story with you. If you are like me and things in life have come relatively easy, it is hard to have any idea of how ongoing health challenges affect every aspect of living. We look to other’s successes in overcoming obstacles to inspire us today and maybe prepare us if we one day face the same. If you or someone you love has been tested by something similar, then Camryn’s story will reaffirm what you have already learned and inspire you to continue to persevere.
The First Hurdle
Camyrn was always a creative, energetic and very intelligent little girl, so, when her parents received a call from her second-grade teacher, it was a bit of a surprise. The teacher explained that she suspected dyslexia, and learning to read would be an uphill battle for Camryn. For the next four years she was in a special classroom and worked tirelessly learning to adjust, adapt, and finally conquer the challenge of learning to read on the same level with her peers with another six years of special tutoring. Getting the words to make sense was her first opportunity to persevere, but it certainly was not her last.
We asked Camryn how she battled feeling different from her peers in school and she responded,
Honestly, I always felt different in class; however, knowing that I was smart in other areas gave me more confidence when it came to school. I was always in the IEP (Individual Educational Program) classes, therefore it was impossible to escape the notion I was different, but knowing that it was only the fact that I could not read that was keeping me “down” from my academic ability allowed me to focus on that task in order for me to succeed in the other areas. I was not going to let reading hold me back from math and science.
My dad has always told me I was born a fighter. I could not tell you a specific moment that I made a personal choice to own my fight; to me that was just the only option that came to mind. Conquering the battle has always been the only option that seemed viable to me. Taking the easy way out just to fit in was not an option, I refused to let a small difference in how my brain worked dictate my life and education.
Perseverance and Grit
About the time Camryn conquered the challenge of dyslexia and was moved to a regular classroom, she experienced a severe and unexplained case of vertigo. Vertigo is an inner-ear condition that causes sensations of spinning, dizziness and nausea. Searching to find answers, Camryn and her parents began a journey that would take them several years and to more than 30 doctors in Northeast Ohio and across the country, through a barrage of grueling and unimaginable tests and ultimately brain surgery that insurance wouldn’t pay for because it was leading-edge medicine.
Now, it is easy to imagine parents with perseverance to find a health solution for their child but a little harder to see a young person, now a teenager, with the grit and vision to know she not only would get past this, but she would keep up with her school work in the process!
Perseverance is a very important character trait that everyone needs to some degree to be successful in life. Some people, like Camryn, are faced with challenges so great they require more perseverance than a group of us combined. Perseverance means having the determination to work hard regardless of any odds or obstacles that may exist. It means to insist on improvement and to have a firm resolve to get something done without giving up. Perseverance is the steadfast pursuit of a task, mission, or journey in spite of obstacles, discouragement, or distraction. In contrast, grit is argued to be a trait of perseverance. Grit enables an individual to persevere in accomplishing a goal despite obstacles over an extended period. Camryn has both!
School has always been very important to me. From a young age, I realized the importance of education. I have always had to work harder than everyone else because of my dyslexia, so working harder than everyone else because of my illness was almost second nature for me. Graduating on time was always something that was non-negotiable for me. Frankly, graduating on time with honors was non-negotiable as well. I refused to let my circumstance dictate if I would perform at my best. I have always wanted to go to a good college, and graduating with honors was how that was accomplished; therefore that’s what I did.
Against the Odds!
After three inner-ear surgeries and two craniotomies (brain surgeries), Camryn had a miracle recovery from her inner ear problems, thanks to her surgeon, Dr. Gianoli with the Ear and Balance Institute. Camryn’s health challenges continued, unfortunately, as she later suffered chronic allergies in her senior year of high school and missed 75% of her classes. Unable to accept this as a excuse to finish less than her best, she drove herself from home and finished on time with her classmates.
There was no time to feel sorry for myself. There was always something new that was popping up that needed my full attention, whether it was a different diagnosis or school work. However, in the rare times I had a “breather” it was really hard. I am not going to act like it wasn’t. It was hard, painful, and really lonely. But whenever I started feeling bad about myself, normally something new would pop up that would distract me.
I tried to take each day as it came. I was not able to plan in advance because I did not know how I was going to feel and if I was going to have to go to the ER or not. I took one day at a time because that was all I had. All I could do was faithfully handle each day the way it was presented to me. I had to trust that what I was going through was going to be used for God’s glory one day and frankly my suffering was not about me. It was always about how God would use my life to help others.
Camryn graduated with honors and received a full-ride scholarship to college. Today she is a freshman at Moody Bible College and is working toward a degree in theology with the intent to be a professor. When asked what other personal insight she might want to add for our readers, Camryn’s response made me realize why her story is so important to share. Through this amazing young woman, we can learn not only about grit and perseverance but, more importantly, about the interest, compassion and understanding we can offer others as they suffer.
People should be more aware of other’s suffering. Small things make such a big difference. You may not know “the right thing” to say, but just try. Making an effort to let someone know you care about them goes a long way. Visiting someone in the hospital can change their entire mindset on their situation. You do not have to be able to cure someone, but at least make them feel like they have someone. When I talk to other people who have chronic illnesses, the most common thing I hear is that people just don’t want to be feel invisible. With chronic illnesses, you normally cannot even tell the person is sick. So, make some sort of an effort to let a person know they are not going through this alone.
Everyone who is suffering, no matter what they are going through, matters and can make a difference to others through the experience, as well. Make the most out of what you are going through, joke with nurses or talk to the person in the room next to yours. You don’t have to feel sorry for yourself. You are in the situation you are in for a reason.
What About You and Me?
Though the struggles could have been overwhelming, Camryn stayed focused and determined to do her very best against all odds. Unlike her example, we might feel sorry for ourselves or even find ourselves in a great place and still be waiting for or expecting an opportunity to feel like a victim, providing us with an excuse to fall short of achieving great things. Rather than moving forward and welcoming distraction from our troubles, we can quickly identify something that gives us a free pass from putting in the effort that is needed. As we have learned in Camryn’s story of overcoming obstacles, it takes true, committed effort to beat the odds, and I hope just reading her thoughts and considering her example will help you and me choose to make the effort needed to make ourselves better, to make a real contribution to our company, our family or our community, and to set an example that makes a difference in the lives of others.
– Beth Paluch