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The Challenge: Can you solve a medical mystery involving a middle-aged woman with a pulsing noise in her ear and a stabbing pain in her head?
The Diagnosis column of The New York Times Magazine regularly asks Well readers to sift through a difficult case and solve a diagnostic riddle. Below you will find a summary of a case that left a previously healthy, active woman nearly crippled by pain. The lab results, consultation notes and images obtained over the two-and-a-half-year course of the illness will give you the same information available to the doctor who made the diagnosis. As it happens, I was that doctor and found it to be a tough diagnosis. Will you?
The first reader to figure out this case will get a signed copy of my book “Every Patient Tells a Story,” along with the satisfaction of knowing you could outdiagnose the Diagnosis columnist. Good luck.
The Presenting Problem:
A healthy and active 53-year-old woman complains of severe pain on the left side of her head and a “swooshing” sound in her left ear.
The Patient’s Story:
“It all started at the chiropractor’s,” the patient told me at her first appointment. It was a hot August day, and she had brought to my office a friend, a huge stack of old medical records and a complicated story.
Her story began two and a half years earlier. She was at the chiropractor, getting a chiropractic treatment on her neck when, suddenly, a loud whooshing noise filled her left ear. The noise pounded against her eardrum like some sound-effect machine gone mad. It scared her, and she told the chiropractor about it right away. The chiropractor stopped immediately, but the noise didn’t. Indeed, the patient told me, it hadn’t stopped for even a moment ever since.
The noise seemed to get worse when she lay down or turned her head a certain way. Usually it was just annoying, but sometimes it was so loud she had trouble hearing voices over the “whoosh, whoosh, whoosh.” She was a social worker, and listening was a big part of her job. Still, it seemed manageable, at least at first.
The headaches started six weeks after the noise. She was talking with a client when suddenly she felt as if she had been hit on the left side of the head with a brick. The pain was blinding, excruciating. She had to leave work and go home to lie in a dark room.
The next morning her doctor, suspecting a migraine, gave her a drug called Zomig and ordered an M.R.I. and then a CT scan of her brain. The medicine didn’t help, and the scans didn’t reveal any useful information. The headache lasted two more days. After that, the left-side headaches came every few weeks, requiring her to take off work; the whooshing noise remained her constant companion.
You can see the M.R.I. report here, part of the patient’s thick stack of medical records she brought to me that first visit.
She saw a neurologist, who ordered an M.R.A. scan, which is an M.R.I. that focuses on the arteries of the brain. It was normal, and the doctor told her she had tinnitus and migraine headaches. An ear, nose and throat specialist ordered a hearing test and an M.R.V., which is an M.R.I. that focuses on the anatomy of the venous system of the brain. A second E.N.T. reviewed the studies that had already been done and suggested a diagnosis of pulsatile tinnitus, mild hearing loss and eustachian tube dysfunction. None of the doctors had advice on how to get rid of the noise or the headaches.
You can see the M.R.A. and M.R.V. reports in her chart.
The Pain Gets Worse:
A few months later, the patient read an article about a disorder that she thought might explain the constant noise in her head and the occasional migraine that still trapped her in a dark room for a couple of days at a time. The disease was called fibromuscular dysplasia, or FMD, an unusual disorder in which the walls of arteries become narrow and restrict blood flow to essential organs, most commonly the kidneys and brain.
She made an appointment with Dr. Jeffrey Olin, director of vascular medicine at the Mount Sinai Medical Center in New York, who was quoted in the article. Dr. Olin thought that the woman’s symptoms, while not classic, could very well be a result of FMD, so he arranged for detailed imaging of the carotid arteries, the vessels that carry blood from the heart to the brain.
As the technician was injecting contrast dye into her veins, the patient felt an ice pick of pain shoot from the top of her skull past her ears and down to her clavicle. It was all she could do not to scream, the pain was so intense. Dr. Olin was immediately concerned. This kind of pain can be seen when arteries tear. Could the slightly increased pressure caused by the injection of dye into the bloodstream have torn a fragile segment of the patient’s carotid artery? Dr. Olin examined the CT scan closely for any evidence of a torn or dissected artery. He didn’t see that, but as he looked at the images of her carotids, it was clear that they were not normal.
Usually the carotid arteries make a straight line from where they take off from the aorta, the large vessel that leaves the heart, to where they deliver blood deep into the brain. This patient’s carotids seemed to meander up the neck, wending their way to the brain like some lazy river. On the left side, where the pain and noise were, the vessel made a 360 degree loop-de-loop on the way.
Here are images from the CT (left) and M.R.I. (right) of the patient’s neck.
Looking at the images, Dr. Olin could tell that the patient didn’t have FMD, but he was fascinated by the baroque configuration of her carotid arteries. Although the situation was rare, Dr. Olin had seen other patients with similarly twisted blood vessels. Many had complained of a pulsating tinnitus like hers, as well as migrainelike headaches. But no one else had complained of the sharp ice-pick pain that now dominated her daily life.
You can read more of Dr. Olin’s reports here.
Treatment Efforts:
Muscle relaxants prescribed by a neurologist didn’t help. Although the patient had been treated for invasive thyroid cancer several years earlier, an oncologist’s evaluation revealed no evidence of recurrent tumor. A vascular surgeon gave a diagnosis of temporal arteritis, an inflammatory disease of the arteries of the head, eyes and face that can cause blindness and stroke. The patient was started on high-dose steroids, but an ultrasound and a biopsy of the artery were normal, and the patient was weaned off the potent drugs.
On top of all this, the patient had made several trips to the emergency room when the pain in her head became too intense to tolerate. She was given a variety of painkillers and had several imaging studies done on her neck, brain and chest. None revealed anything new. At several of those visits her blood pressure, normally low, had been quite elevated. The E.R. docs thought it might have been a response to pain.
The patient used to be an athlete. Now just walking up a flight of stairs could trigger the stabbing pains on top of the steady daily pain. Simply trying to maintain the appearance of a normal life with this constant pain was exhausting. The pain, she told me, was sapping all the pleasure from her life
The Doctor’s Exam:
Start to finish, it took over half an hour for this patient to tell me her story. Then she handed me her thick sheaf of medical records. On top was a seven-page summary of all the doctors she’d seen and tests she’d had done. I looked through it quickly and put it aside. It was far too much to review in the time I had left.
The examination was mostly normal. The patient did have some tenderness over the carotid artery on her left side. And there was a soft tissue mass at the base of the neck that measured 10 centimeters in diameter and was raised a couple of centimeters. The patient told me that this had been biopsied and PET scanned, because of concern about a possible recurrence of her thyroid cancer. No cancer had been found, and the biopsy suggested it was a lipoma, a benign fatty tumor. The records are all there, the patient assured me, waving to the pile of papers she’d given me.
I knew I couldn’t make a diagnosis based on the information I had so far. I thought it was possible that some part of her carotid artery had been injured by the chiropractic manipulation, but I was baffled as to why she would develop such terrible pain when contrast dye was injected for imaging. I would need to review the records she had brought in and try to figure out what her problem might be. I asked her to return in a week or two and told her I would have a plan of action at that point.
You can read my summary notes here, as well as the extensive patient chart below. (Click on the lower left for a full-screen version.)
Solving the Medical Mystery:
Now I turn the question over to you, my readers. What do you think the patient has? Or if a diagnosis cannot be made based on the information you have here, what additional studies would you want to get? How can you help this middle-aged woman get back to a normal life without the pain and pulsations that plague her?
Rules and Regulations: Post your diagnosis and questions for Dr. Sanders in the Comments section below. The correct answer will appear tomorrow on the Well blog. The winner will be contacted. Comments may also appear in a coming issue of The New York Times Magazine.
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