A friend forwarded this article on a man who had a devastating brain injury and has recovered tremendously due to cognitive rehabilitation therapy, something I’m doing right now, too. I think it would be particularly useful to Superior Canal Dehiscence patients with brain fog and disorientation symptoms.
I received a tweet today from a fellow Superior Canal Dehiscence Syndrome sufferer, @ElzElz, which included this link to an amazing journal article on how vestibular disorders affect cognitive function. I nearly wept. The article details the whole picture, and when I sent it to my wife, she wrote — “Crazy! It totally describes you and your symptoms.”
I’ve had a good day. Clearly, I’m blogging twice! I also drove 36 miles to Chicago, had a meeting, taught a three-hour class, took a call from brilliant producers and a director who gave me great notes on a script, and I will now drive back home in the snow. It hasn’t always been this good, and believe me, my head hurts and I’m tired. Not to mention, I forgot the names of students, actors, movie titles, and even mixed up a plot line or two in class (but my lecture kicked ass, if I do say so myself.) In my meeting, I felt like maybe I asked for clarification on several points I would normally have processed the first time. Those are small things. Workable things. And hopefully, fixable things. I’m grateful to be as functional as I am, but I’m thrilled that someone is researching the links between vestibular problems and cognitive function, as these are the long-terms symptoms I fear the most.
Perhaps plugging up this hole in my head will help keep the ideas and memories inside where they belong.
As I have been made to understand it, there is a clear equation, or proof, required for diagnosing a Superior Canal Dehiscence. The first step seems to be the hardest of them all:
You need 1 doctor who can put together the puzzle of your symptoms into a clearly assembled picture and send you for testing; or, you need to somehow put that puzzle together yourself and find the 1 doctor who understands this problem and will take a shot at investigating it. It’s still new, it’s very rare, but I don’t buy that the modern ENT worth his or her otoscope shouldn’t have heard about this disorder by now. Google lists hundreds of pages about SCDS. It was on The Doctors TV show, famous people have suffered from it and brought it into the news, and there are dozens of journal articles regarding the problem. And yet, it remains so “mysterious.”
If you manage to solve the first part of the equation and get a referral to a specialist, there are many tests that are performed on dizzy people. Dizziness is my primary symptom, and it is what leads to my other complaints of brain fog and fatigue. In some ways, people who have stronger audiology-related SCDS symptoms should be a tad bit easier to diagnose. Autophony and Tullio are very rare, and strongly indicate a dehiscence to a doctor who is current on the research in his/her field. There are many causes of dizziness, however, and most docs are under the inclination that “it’ll just go away over time.” If you’ve somehow passed through the first stage and found a SCDS-suspicious ally, you will need to have at least 2 tests. First, a hearing test. Most SCDS sufferers have some degree of low-frequency hearing loss. But again, lots of people have that symptom for a variety of reasons, so, in and of itself hearing loss cannot be the smoking gun. That gun is usually the VEMP TEST (Vestibular Evoked Myogenic Potential). If you have a strong VEMP response (which is perfectly comfortable, but very strange to endure), you are allowed to continue your progress to the final proof and solve for X.
It’s all well and good to have lots of symptoms, low-frequency hearing loss, and a strong VEMP, but they have to see the dehiscence in order to fully diagnose the problem. So, step 3 is a high-resolution, thinly-sliced CT of the temporal bone. It was strongly suggested to me that I have the images taken at the location of my doctor’s choosing because that facility is quite familiar with SCDS, understands how to get the best images, and would forward those images to a radiologist with experience diagnosing the disorder. Proof is in the picture. I understand that sometimes they can’t see it. Unfortunately, as I’ve been told, if they can’t see it, they don’t like to operate, so getting a great image and experienced radiologist are crucial in this final step.
And yet, even with a clear image and a reading from a trustworthy radiologist who has seen SCDS before, there can be wild differences of opinion. My radiologist called my dehiscence “slight.” Dr. Hain, the specialist who diagnosed me, thought I should opt for a more conservative approach and let my condition ride for a while longer, while Dr. Wiet, my surgeon, feels a 3mm hole is quite significant, and that my situation is clearly one in need of repair sooner than later. So, I then sought a second and now third opinion just to be sure.
With clear proof and X solved, there comes some relief — mentally. You know you’re not crazy, you’re validated in that someone believed you, and now you have a solid answer. But what you do with that answer is ultimately up to you, whose opinion you trust, and the degree to which you are suffering. I see that big X in front of me every day, and still debate what to do with it.
My body is constantly fatigued. Like Sandra Bullock at the end of Gravity fatigued. My concentration and short-term memory are fried. It feels as if my brain is floating loosely in my skull and cannot keep up with the movement of my eyes. I turn my head right, and my brain locks in two seconds later. I have the balance of a drunken toddler. My head pressurizes like a hot air balloon with the slightest bit of physical activity. Throbbing pulsatile tinnitus keeps me awake at night. Loud noises stab through my skull and make me vibrate like a human gong. The slightest bit of emotional stress instantly sends me over the top. I’m the fractured shell of a man who one month ago set a personal best in a 5K and was routinely running over thirty miles a week at a nine minute pace, but now is stuck in bed at noon. I have no answers as to why I feel this way, and I won’t have even the slightest clue for another seven months.
July 10, 2013: the last entry in my iPhone training log. I ran 6.5 miles that day, and entered this note: “Muggy. Too early.” I didn’t feel very good as I cooled down, and must have rationalized that an earlier than usual start (to avoid the blazing heat) had caught my body off guard. Two days earlier, on my 41st birthday, I had cut a run short in the rain because my quads were burning – I found this unusual, but chalked it up to overtraining as I was trying to quickly ramp up for a 10K race. Since May 1st, right after the Kentucky Derby (and a hangover that lasted a week), I had lost close to thirty pounds in about two months through a strict regimen of diet and exercise. I was finally getting healthy after two years of severe sinus and lung trouble. I looked and felt like the guy I wanted to be. I was writing well on a screenplay that I cared a lot about. It was summer. It was all good. Or so it seemed. I haven’t gone for a run since.
I took a few days off but didn’t recover. It couldn’t be dehydration after a week. I began to notice my heart constantly pounding in my right ear. A veteran of a thousand ear infections, this was nothing new to me, but my ears were ventilated with tubes and my sinuses were as healthy as they’d ever been. High blood pressure? No. I was 110 over 60 with a pulse in the low 50s. Within a day or two, I began to feel twinges of swimminess and disorientation. My balance felt off. A bit of trouble with our rental house in LA sent me over the edge with stress. And then the fog descended: a deep gray mist of dense confusion. My brain simply would not concentrate. Forget writing, I barely had enough focus to work the phone and make doctor appointments. I had to quit my three-cup-a-day coffee habit cold-turkey, as even a decaf latte would set the room spinning and my anxiety soaring. Alcohol was impossible, too. One drink made me feel like I’d downed the whole damn bottle, and then I felt hungover for two days. A gorgeous Outer Banks vacation was squandered lying in bed due to what had become full-on vertigo. In the course of about three weeks, I had gone from 60 to zero. Dead stop.
The previous November, I had my third sinus surgery to clear polyps and ease chronic infections. I’d spent the better part of eighteen months, as we moved from LA to upstate New York and then to Chicago, living on antibiotics and steroids. The surgery was supposed to help, but it didn’t, and I became even more sick. My ENT put me on a regimen of gentamicin rinses to kill the highly resistant infections in my sinuses (I had more than one bug at a time). Essentially, I would water board myself with liquid antibiotic. I’d hang upside down off the side of my bed and fill my sinuses to the brim with this stuff twice a day for six months. It worked. The treatment killed everything and allowed me to recover and regain my fitness – until July, when I began to think this treatment was the cause of my new problem. Gentamicin is ototoxic. It kills the little hairs in your inner ear that are essential to the operation of your balance system. In fact, they use it to destroy the vestibular function of people with such severe vertigo that it cannot be cured or mitigated. It is ear poison – the nuclear option – for people who have no other way to stop the world from spinning. Given that my ears were ventilated with tubes (negating the natural reverse pressure of the Eustachian tubes), and that I was filling my head with liquid while upside down, there was a strong chance that the ototoxic gentamicin had reached my inner ear and permanently damaged my vestibular system. My ENT assured me that if this were the case, my brain would eventually figure out how to cope with the situation. It’s called “neural plasticity,” he said, and it could take up to two years. I wasn’t settling for that.
My primary care physician cautioned that I was just getting older and these things happen. She suggested that maybe it would all just go away. An ER doctor took a CT of my abdomen and determined that I simply had a bad case of gastritis. I was referred for a psychiatric consult, as perhaps this was a chemical disorder of the brain. It wasn’t. A Chinese Medicine herbalist told me that I was just allergic to absolutely everything. The acupuncturist said he could fix me in one visit. I let him try twice. The dentist thought it might be my wisdom teeth perforating my sinuses. I was sent to an endocrinologist who informed me that all of this trouble might be the result of “low-T.” She referred me to a urologist who didn’t think my T was too low, but was willing to give me the testosterone anyway…just because. My physical therapist believed that I was suffering from Benign Paroxysmal Positional Vertigo (BPPV), and we began a series of exercises and Canalith Repositioning Maneuvers to clear crystals from my vestibular canals. That didn’t work either, but she became a great gift to me, and was able to reduce my symptoms through massage, manual manipulation, and balance exercises that ultimately restored enough function so that I could return to work and appear to most people as relatively normal. Finally, and you would think obviously, I was referred to a neurologist who ordered an MRI of the brain. I waited a week. MS? CF? Brain tumor? I finally called her. “Oh, sorry! Yeah, it came back clean. Good for you!” While I knew it was utterly ridiculous, morbid, and disgusting, I was actually hoping they would find something. Anything. An answer.
I begged my ENT for more options. Surely there must be a true specialist I could see. I’m not sure why it took him six months, but he finally referred me to a specialist in Chicago at the Northwestern Medical School: Dr. Timothy Hain, known as “The Dizzy Doc.” Dr. Hain is a world-renown dizziness expert, and, not surprisingly, it took me six weeks to see him. I spent an entire day undergoing tests and evaluations the likes of which were once done in secret cold war labs far below ground in the 1950s. Spinning chairs, metal probes, sound booths, cold and hot air injected into my ears…. After five or six tests, it became clear to everyone in the office that, “yes, you have a problem! But….sorry, we still don’t know what it is.” I was losing hope again just before the last procedure of the day, called “The VEMP Test.” When it was performed on my left ear nothing happened. When we did it on the right side, the walls of the room started jerking up and down as if I were pinned to a trampoline being bounced on by a gymnastic giant.
The tech ran to get the doctor, they conversed, and then he asked: “Brad, can you hear your eyeballs moving in your head?” Indeed I could. It was a grating, sand-papery swishing sound. They put a tuning fork on my head – I heard it clearly in my right ear and not my left. They put the fork way down on my left ankle – again, clear as a winter church bell, but only in my right ear. The answer finally came: “You, sir, have a hole in your head. Probably.”
The suspicion was that my particular demon was named Superior Canal Dehiscence, which essentially means there is an opening inside my skull, below the brain and over my ear canal, which creates a “third window” in my head causing sound vibrations to bounce around inside my brain. It also throws the balance system for a loop, so to speak, which makes the brain work incredibly hard to maintain basic equilibrium, thus the leg weakness, brain fog, memory loss, and fatigue. It had begun to also affect my spine, as the strain of remaining upright was contorting every muscle in my neck. Autophony, or hearing the internal sounds of one’s own body – pulsatile tinnitus, eyeball movement, even creaking joints, jaw and vertebrae – is another major symptom, as is the tullio affect, or sound-induced vertigo. Loud noises rattle the brain and create dizziness as a result of this condition. It all made sense after seven months. But, in order for it to be real, it had to be confirmed by CT Scans and MRIs, which took a good long while and a lot of deliberation, as my dehiscence was only 3mm wide. So small, and yet so damn disruptive.
I met with two Chicago surgeons who have experience with Superior Canal Dehiscence and discovered that there are two surgical options. The first is the less invasive “transmastoid approach,” which is an outpatient surgery with few complications, but it does not allow the surgeon to actually visualize the hole, and has a lower likelihood for success. The “middle fossa approach,” which involves a craniotomy, allows for better visualization and a greater chance for success, but comes with significantly more risk. Because the second surgeon I met with believes there is need for a more extensive repair to my tegmen (the boney shelf inside the skull that holds the brain in place), I’ve opted for the craniotomy. So, essentially, you fix a small hole in the head by creating a much larger one!
Why does this happen? They believe it’s congenital. Some people are born with weak spots over the inner ear, and that at some point, usually in a person’s 40s, the bone just pops open. You could blow your nose too hard, get hit in the head, or maybe it’s even the result of chronic sinus and ear infections. Perhaps my bones have been weakened by Celiac disease. Who knows? Superior Canal Dehiscence was not first discovered until 1998. It’s so new and so rare, in relative terms, that the two surgeons I spoke with have only performed thirty of these repairs between them – and yet they do hundreds of more complicated procedures each year. The effects of the “syndrome,” or the symptoms a person feels while suffering from the dehiscence, vary in type and intensity from one individual to another. How many people are dealing with this and have no idea what it is, or are getting the wrong answers? How many people don’t live in a large city with top-notch medical resources, or don’t have the insurance I do, or don’t have the time and perseverance to push through the walls of this medical corn maze to find the exit? It’s sad to think about. I’ve let my (now former) ENT know exactly what my diagnosis is, in hopes that should he run into it again, he’ll be able to help people faster.
Here’s how it goes: they shave a strip of hair behind my right ear, make a four inch incision, then push aside the skin and muscle to expose the skull. They cut a hole in my skull, move my brain out of the way, visualize the bone over the ear canal, and using a mixture of bone paste and my own bone fragments, they putty the dehiscence like you would a hole in the wall. Using that same paste, they will fill and reshape the tegmen (brain shelf), then resurface the skull, reinstall the bone flap they cut loose, insert titanium plates to hold it together, and close me up. It is estimated I’ll spend three days in the hospital, be recovered enough to return to work within two weeks, and be fully recovered within eight. The results, it seems, have been good over time. I’ve read about people who have returned to rock climbing and playing instruments in orchestras – two things I can’t fathom doing right now given how I feel. But there are risks. Some people lose their hearing entirely on the side of the procedure. There are also risks to the brain, so a neurosurgeon, Dr. Kazan, will be assisting Dr. Wiet in the ER. In the meantime, I’ve been taking a 2mg dose of Valium before bed, which has dampened the vertigo and other symptoms significantly. But, on the flip side, it also further depletes my sense of balance, making me more prone to falls. It’s a temporary remedy, but not a long-term solution.
I feel now that surgery is the only option for me. I have a young family, a creative and academic career to sustain, and a lot of running left to do. It’s time to get fixed.
We’ll keep you updated.