One weekend a little over two years ago, something weird started going on inside my head. The chief symptom of my ailment was vertigo, which came and went spontaneously, in episodes typically lasting less than a minute. Some of these dizzy spells were barely noticeable. Some were accompanied by sudden deafness in my left ear, ringing on the right, and profuse sweating. Some sent me crumpling to the ground in spinning agony.
My only comfort that weekend was in thinking my symptoms were so striking that doctors would pinpoint their cause in no time. Man, was I wrong about that.
In October 2017 I was 54 and divorced, with two kids off in college, and single as a dollar. To improve my chances on the dating front I decided to get serious about losing the few extra pounds around my middle. So, one Monday I hatched a plan to go two solid weeks living the healthiest life possible. I would choose meals with extra care, get lots of sleep, and double my running and hiking routine. I dubbed it my “fitness fortnight.” By Friday, my thrice-weekly run had grown from three miles to six, and on days between running I took long hikes through the woods behind my house at the fastest clip I could muster. It felt great.
On Saturday morning, sitting at my writing desk and looking out at the birds at my feeder, I felt as if something were gently squeezing my brain. It lasted just a few seconds. An hour or so later it happened again, feeling more intense this time but still lasting no more than half a minute. I had one more spell that day, again out of the blue and slightly more intense than the last.
The next morning, I was just waking from a good night’s rest when I heard a whooshing sound I attributed to the dishwasher running in the kitchen downstairs. But then I remembered just emptying it the day before. It must be something outside the house, I figured. When I heard it again later that day I went to the window. There was nothing there. That’s when I realized the whooshes were whooshing at a familiar tempo. I pressed a hand to my neck and realized the whooshing was tracking my pulse perfectly. I was literally hearing my blood as it beat its way through some vessel near my left ear drum.
I decided to see my doctor first thing Monday morning. I also decided, reluctantly, to hold off on running. My fitness fortnight became a fitness four-night.
My regular doctor was out. His replacement was not the least bit alarmed at my symptoms and said he saw two or three new patients a week reporting vertigo. I hadn’t even thought of this as vertigo, as so far I hadn’t experienced any spinning. But apparently there’s such a thing as non-spinning vertigo. In any event, vertigo came with age, the doctor assured me, then prescribed meclizine.
I had had seven spells by then. That evening I had my eighth, while standing in my kitchen making dinner, and this one came with a new feature. When my brain squeeze came on, I held onto the edge of the sink to steady myself. It wasn’t enough. Suddenly I was unable to remain standing, as if my brain had shut off all instructions to the muscles that allow hominids to stand upright. I dropped to the floor.
I remember once playing with a toy doll made of hollow wooden limbs held together by wires. When you pressed a button at its base, the wires went slack, causing the little man to crumple into a pile of body parts. The experience in my kitchen that night was just like that. Something pressed that button. I would later learn these are called drop attacks, and the one that night would be the first of many.
I decided to work from home the next day, fearful of having a severe episode while driving from my home in Carrboro, North Carolina, to my office some 20 miles away. I had another episode that day, and another at night. That one woke me from a deep, dreamy sleep and I had to sit up and steady myself until it passed.
Early the next morning, back at my writing desk, I noticed the birds outside my open screen door went suddenly quiet. Turning to look, I realized my hearing had gone out in my left ear—just as my brain felt the familiar squeeze and, this time, the world began to spin. It was textbook vertigo. I laid both palms on the desk and looked out the window as a great wave of nausea came on. I was on the verge of vomiting, sucking in that one last deep breath as my stomach prepared to spew forth its contents, when the spinning stopped and my hearing came back. As I exhaled, I noticed beads of sweat on the hairs up and down on my forearm and shivered as a light wind blew across my wet skin. I was so drenched I had to change my shirt.
I called my mom, who had had some experience with vertigo, and learned that her doctor had also prescribed meclizine. But she had never experienced episodes quite like mine. She recommended I begin logging the episodes, in order to have solid data to share with my doctors, advice I began following right away.
The meclizine did not appear to be helping. I called my doctor’s office to get an appointment with an ENT, and got one, but would need to wait four weeks. With each episode worse than the last, I was pretty sure I’d be in the ER way before then. Fortunately, the next episode was slightly less intense. And the one after that was even milder. I decided I could wait.
I went two days with only the mildest of spells and so began driving to work again. The whooshing, or pulsatile tinnitus as I learned it is called, also subsided. I was hearing my pulse only once or twice a day, for five or ten minutes at a time. Interestingly, the whooshing spells never coincided with the dizzy spells. They were on their own random cycle.
Ten days after my first episode, sitting at my desk at the bank where I work, I heard a ringing on the right side of my head. Then my hearing went out in my left ear and the world began to spin. It spun for less than a minute, not as badly as it had before, but when it stopped, I again noticed my forearms wet with sweat. I made an appointment to see my regular doctor the next day.
“You need a brain scan right now.” His face had gone slack when I described what was going on. His alarm was oddly reassuring. I’ve been seeing my primary care physician for more than a decade and was glad that he too saw this as being truly weird. Other than an argument over how to pronounce tinnitus (I’d always heard it tin-EYE-tus and he insisted it was TIN-i-tus, which I think sounds silly) my doc and I were on the same page.
While waiting for the results of the emergency MRI of my head, I went online to see what my doctor might be worried about. It didn’t take long to find a condition whose symptoms lined up with mine, a non-cancerous tumor called an acoustic neuroma. This was it, I thought. It was an awful thing to have, but weirdly I took some comfort in knowing what was probably going on. My mind turned to my son and daughter and how I would break the news, explain the condition, and assure them I would be fine once the tumor was removed.
I learned by email that the MRI came back negative. There was no tumor or other sign of anything unusual. My first reaction was disappointment at having to go back to square one. Then I remembered that learning one does not have a brain tumor is actually not a bad thing.
Thus began something of an odyssey, which consisted mostly of waiting for appointments with specialists of various stripes, all the while experiencing random waves of vertigo of varying intensity. It was like a gremlin had taken up quarters in my head, tormenting me at random moments of his choosing. Sometimes he’d sleep quietly for two or three days, then come out 30 times or more in a single day.
My first stop was the otolaryngology or ENT department at Duke Hospital where, over the course of several weeks, a team of ENTs ordered a hearing test, a test of the vestibular functions of my inner ear, additional MRIs, and a CT scan of my brain. Between appointments I began scouring the internet to learn about my hearing and vestibular system, and to see what the hell might be going on.
The space between your earlobes and brainstem is home to a remarkable bit of machinery. Consider just the semi-circular canals, three hollow rings positioned on opposing axes like the rings of a gyroscope and filled with tiny crystals floating in liquid. Moving your head in any direction disrupts the flow of those liquids, disruptions transmitted to your brain by the vestibulocochlear nerve. As its name implies, this nerve, also known as the eighth cranial nerve, also transmits signals produced by air pressing on your cochlea, or eardrum. The mechanisms are magnificent. And as a software engineer I can only marvel at the complexity of the “code” inside the brain that has to interpret the data coming in via this key pipeline of information on either side of our heads. Wrapped all around the hearing and vestibular organs is a web of blood vessels large and small, equally marvelous it its ability to get blood in and out of impossibly compact nooks and crannies.
There are any number of things that can go wrong in this part of the head, all of which would need to be considered in my case. Doctors call this process of elimination differential diagnosis. So far, only the tumor had been struck off my list. That left things like Benign Positional Vertigo (whose cause has to do with those canal crystals not moving around as they should) and Meneire’s Disease (an inner-ear disorder that can cause random vertigo spells like mine). To be considered on the vascular front were things like aneurysms (blood bubbles), stroke (blood clots or bleeding), stenoses (vessel narrowing), fistulas (abnormal connections between arteries and veins), and dissections (blood vessel tears).
When I asked my senior-most ENT about a condition I had run across called vestibular paroxysmia (VP) caused by a blood vessel compressing the vestibulocochlear nerve, and whose symptoms seemed to line up with mine, he responded as if I’d insulted him. “I don’t believe in that,” he snapped. His umbrage made me feel like I’d just suggested voodoo. He hadn’t seen it in 25 years of practice, he continued, and when I pressed him for more he said if such a condition were real it would cause much more severe hearing loss than what I was reporting. He clearly knew what he was talking about. I decided to let the doctors do the diagnosing.
One of the doctors suspected a vestibular migraine, whose typical symptoms include dizzy spells, and suggested I go on a migraine diet for two weeks. But removing nuts, cheeses, processed foods, soy products, and olives from my diet had no apparent effect. Nor did abstaining from alcohol. And thank God for that.
The battery of testing ordered by the ENTs gave no clue as to what was going on. My hearing was fine, except for a slight deafness on my right side, which I’d had since I was a teenager, when a firecracker went off in my right hand after waiting too long to throw it. My vestibular organs all seemed to be functioning properly. I didn’t qualify for Meniere’s, whose spells need to last 20 minutes or more, whereas mine typically lasted 15 or 20 seconds. And none of the brain scans—not the MRI, MRA, MRV, nor CT—showed signs of a dissection or other obvious vascular disorder. One scan did find an aneurism, or blood bubble, deep inside my brain. But its size (thankfully quite small) and location ruled it out as a culprit.
The apparent lack of vascular disorder baffled me for two reasons. One, pulsatile tinnitus by definition is due to blood flowing where it shouldn’t, i.e., where your cochlea can pick it up. Two, my symptoms came on after the most intensive cardiovascular workout of my life, with double-runs or fast hikes every day. Rather stupidly, in hindsight, I had clearly let my heart rate go way into a danger zone. Wasn’t it reasonable to assume I had torn some vessel up there? Perhaps. But the testing showed no sign of it.
The ENTs concluded that a central nervous system disorder should be considered next and referred me to neurology. And the referring doctor told me to expect the neurologists to order a lumbar puncture, or spinal tap, which quickly brought two words to mind: oh, shit. If there was one medical procedure I had feared more than any other it was a spinal tap. (But I did rather enjoy Rob Reiner’s movie). Fortunately, the very first neurologist I went to saw no reasons to indicate a central nervous system disorder. “You don’t need a lumbar puncture,” he told me. I nearly hugged the guy.
He still suspected a vascular issue and referred me to a neurologist who specializes in those. When I told her about another symptom that had come on a few weeks before onset, a stiff neck whenever I ran, she ordered MRIs of my neck to expand the search for a vascular disorder in that area. Nothing.
Meanwhile the episodes continued. In April I crumpled to the ground after drinking one beer at the Ponysaurus Brewing Company in Durham, where the other patrons no doubt assumed I had had way more than one. In May, in baggage claim at the Raleigh-Durham airport, I noticed the loud ringing on my right side, which I had learned by then signaled the onset of a drop attack. I headed for a bench not ten feet away but didn’t make it. I dropped like a rock onto the cold, tile floor. Leaving Duke’s imaging facility one day, passing by the cancer center, I put both hands on its stone exterior when the ringing began, thinking wrongly that I could remain standing. A second later I was on the ground, rehearsing what I would say to the passersby who I knew would soon be coming to assist. But none did. It had been seven months since the onset of this weirdness. By mid-April I had logged 853 episodes, including 28 drop attacks—one per week, on average.
Duke Hospital is a world-class medical facility with a well-deserved reputation for superior medical care, but it appeared my condition had them stumped. I decided to look elsewhere for help and started, of all places, on Facebook. There I found a group of pulsatile tinnitus sufferers and began looking for names of doctors in their posts. Because my PT had begun on nearly the same day as the vertigo, I figured there was a good chance they had the same underlying cause. If I could diagnose the PT, my thinking went, I might simultaneously diagnose the vertigo.
I went to see two doctors who specialize in pulsatile tinnitus, one in New York and one in Baltimore. Looking at my scans, the New York doctor, a neurointerventional radiologist, saw some indication of stenosis but could not say that was causing my symptoms. The Baltimore doctor was equally baffled, until the day after the appointment when he placed a phone call that changed everything.
I was driving to work one morning and saw a call coming in from Baltimore. The ENT had woke up that morning with a new idea, he told me with a hint of pride. I was just thrilled he took the time to call. He suggested my symptoms might be due to something called intracranial hypotension due to a cerebral spinal fluid leak. I thanked him profusely and pulled over for a quick bit of googling. The condition, I learned, would seem to explain every one of my symptoms.
I was ecstatic. After seven months of riding this disturbing roller coaster, finally a doctor had the combination of curiosity and caring to point me to the most promising path I’d yet encountered. Incredibly, one of the three major centers in the US for treating CSF leaks, I learned, is none other than Duke Hospital. Why my Duke ENTs and neurologists did not think of this I can’t say. In any event I went back.
After reading everything I could find on CSF leaks, a plausible story nearly wrote itself. Our brains are suspended in a bath of clear liquid that extends down our spinal column. On one of my intense runs back in October, I had caused a tear that CSF was leaking out of, causing my brain to sag onto my vestibulocochlear nerve, in turn triggering either the pulsatile tinnitus or vertigo, depending on where it sagged. The whooshing was the result of something called the Venturi effect when blood is forced to flow through a constricted space—just as a running water hose hisses when you kink it.
Two things supported my story. First was the fact that from time to time, with no symptoms of a cold, I would find a thin clear fluid dripping from my nose—I read this was a common symptom. I also decided to test the saggy brain theory. One day when I felt the pulsatile tinnitus coming on, I got down on all fours, hunched up my hiny and let my head dangle upside down. The whooshing stopped. When I stood up the whooshing came back. I repeated this test dozens of times with same result.
Waiting three months for testing to confirm a CSF leak (I’d become accustomed to waits of weeks or months between stops on my journey to diagnosis) I read that caffeine was known to reduce symptoms of intracranial hypotension. I happily increased my morning consumption from four cups to six. A lifelong coffee drinker, this was a silver lining, albeit slim, to the gray cloud of dread that hung over me waiting for the test they would use to detect a CSF leak: a spinal tap, while lying on the bed of a CT scanner, to inject dye into my spinal fluid, dye that would show up on the CT scans when it leaked out.
It turned out to be not the least bit painful. Just a bit of pressure against my spine. “Do want to see what I see?” asked the friendly technician who had patiently explained everything beforehand, setting me at complete ease. “Sure!” He positioned the CT screen so I could watch as a needle entered the space between the bones of my spine.
The CT myelogram turned out to be rather cool. But it did not show any signs of a CSF. “I know it’s disappointing to not know what’s going on,” the doctor told me over the phone a few days later. “But trust me, you don’t want to have a CSF leak.”
Damn. I had fallen victim to something called confirmation bias, where you focus on evidence that supports a hypothesis you already believe to be true. It was the same as when I was convinced I had a tumor.
Until this time, my episodes of vertigo came on randomly throughout the day. But now they tended to come on between 2:00 and 3:00pm, when my recently increased morning caffeine infusion was starting to wear off. Might there be a connection? Over the course of ten days I switched to decaf, gradually mixing it into my regular coffee. The result was striking. I went from having 50 or more episodes per week to having just a few. And none were severe. I missed the effect of fully caffeinated coffee but still enjoyed a lesser effect, from the residual caffeine in decaf, but was elated that my symptoms were now easily tolerable. A week went by with almost no episodes. Then two. Then four.
Still wanting an explanation, I went to see a neurologist in Chicago whose name I had seen on dozens of research papers on hearing and dizziness disorders. He examined my growing collection of images and ordered an all new battery of tests of my hearing and vestibular functions. Sitting with me in his office after several hours of testing, the friendly doctor (who looked remarkably like Steve Martin) patiently summarized nearly a dozen different conditions that could potentially explain at least some of my symptoms before choosing for his diagnosis the very condition my Duke ENT said did not exist: vestibular paroxysmia.
The cause of VP, also known as microvascular compression syndrome (MCS), is not known. It may be due to a weakening of the myelin sheath surrounding the vestibulocochlear nerve, which makes it susceptible to interference by a blood vessel running beside it. Such interference triggers sudden episodes, or paroxysms, of hearing loss, vertigo, and imbalance. The impact of caffeine abstention made sense to my Chicago doctor given its well-known effects on both nerves and blood vessels in the head. He suggested that I stay off caffeine as long as that helped, and if the symptoms returned, there were drugs available known to help relieve VP symptoms.
I went back to Duke and found a neurologist who did believe in VP and who in fact was treating other patients for the disorder. But he didn’t think I had it. He told me it did not cause hearing loss, the exact opposite opinion of the Duke ENT who said it would come with severe hearing loss (if it in fact existed).
Three specialists were saying three different things. I decided to just accept the uncertainty of my situation and get on with life. The only thing I knew for sure was that limiting myself to decaf seemed to minimize my symptoms to the point of irrelevance. I brushed off the two or three mild head squeezes per week, enjoyed my decaf, and learned to tune out the occasional whooshing in my ear.
Six months after my trip to Chicago I was sitting at my desk at work when one of the mild dizzy spells came on. It wasn’t bad, but something told me to head to the nurse’s office where I work. As I sat on the examining table explaining my history, the spinning accelerated and suddenly I could no longer sit upright. I rolled over in agony—the spinning had never been this bad—and began vomiting for the first time since this whole thing began.
The spinning and vomiting subsided for ten minutes or so then came back. I texted my friend Elisabeth who rushed over and drove me to the Duke ER. The waves continued, thankfully growing less intense each time. Until that day, no episode had lasted more than a minute. This one lasted nearly four hours. The ER docs ordered a CT scan, reviewed my records, and sent me home at 6:30 the next morning. As expected, they had no idea what was going on.
My Duke neurologist prescribed carbamazepine, known to be effective in treating vestibular paroxysmia, but I decided to try something else first. Learning that “decaffeinated” coffee can contain up to 30% of the caffeine of regular coffee (I think “low caf” is a better name), I went entirely caffeine-free. No decaf, no caffeinated teas, and no chocolate beyond the occasional nibble. That was six months ago. I still hear whooshing from time to time, but not as frequently or intensely as before. And I’ve not had a single episode of vertigo. Not even a mild one.
What’s going on in my head? I still don’t know for sure.
But I do know lots of scary things that it’s not, and for that I’m choosing to be grateful. I miss coffee, sometimes a lot. But whenever I feel the urge to make myself a French Roast pour over, or dash into the nearest coffee shop for a latte, I just think back to that horrible night in the ER. The urge goes away and I get on with life. And the little gremlin inside my head stays asleep.