Story taken from the March 23, 2011 eiditon of The Advocate.
|PATRICK DENNIS/The Advocate
Ulfur K. (Wolfie) Eyjolfsson, 11, has stitches removed by Dr. Gerard Gianoli after surgery to correct an inner ear condition that caused severe vertigo. To get the procedure, Wolfie flew to Baton Rouge from Norway with his parents, Eyjolfur K. Emilsson, standing left, and Arna Mathiesen.
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 arch 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.
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
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, including 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)
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, tulio’s phenomenon, along with many other symptoms. My quality of life quickly diminished. Not only was I not able to work anymore, 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 that it would go away; and 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 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 and intact. I did not hesitate to schedule with him. I was esperate to have my life back!
My surgery with Dr. Gianoli was 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 side 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.
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 incredible drive to keep learning and improving the state of the art 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 reinforces 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 has repaired both my ears, I have my life back!
Anne Chaka, Ph.D.
Over a period of six years, I sought the help of seven 'specialists', each with a different diagnosis for my deteriorating balance, and with one doctor asking if I knew how rare it would be for me, having had a rare brain tumor (acoustic neuroma), to now have something as rare as a semi-circular canal dehiscence--saying it would be next to impossible! With an inner knowledge knowing something was wrong, I sought the help of Dr. Gianoli (the 8th doctor) who was determined and optimistic he could solve my problem. I had a successful surgery for posterior semi-circular canal dehiscence and on the road, finally, to a full recovery. He was up to the task of a challenge and gave back my life. He is a kind, caring, intelligent and skillful surgeon/doctor who will go the distance for his patients.
Coral Springs, Florida
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 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 she 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 were new problems.
Symptoms progressed over the next two years and she became more sensitive to sound, 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 eventually asked to not 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, 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 dehiscience. She had surgery in November 2013, 2 1/2 years after her problems began, and 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, 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.
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 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 was 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 because 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 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.