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an online version of the magazine Winter 2007
Features
A Minor Balancing ActPHOTOS BY CHRIS HARTLOVE
 
  How one otolaryngologist found a way to set the world right for patients with a mysterious form of dizziness.

By RAMSEY FLYNN
 
 
 
 

Richard Christian’s life came unhinged with a belly laugh. In the fall of 2004, the Illinois high school teacher was tailgating with his family before a Notre Dame football game when his youngest son piped up that he was now old enough to drink a beer. The boy’s older brother shot back: “You got about as much chance of getting a beer as of catching a leprechaun in this football game.”

Christian, their athletically built 55-year-old father who had a long record of robust health, doubled over with laughter. And then, he just kept on tilting forward until he collapsed.

“Dad!” said son Sean, “are you stable?”

“I don’t know,” stammered a shaken Christian as his wife and daughter joined the boys in helping him back to his feet. “I just fell over.”

. . .
Minutes before operating, surgeons map out the procedure.  
> Minutes before operating, surgeons map out the procedure.

Worried that he might have suffered a stroke, the family felt reassured when they noted that Christian was speaking clearly and in perfect control of his extremities. Minutes later, though, Sean told another joke. Christian chortled and again lost his balance. This time his sons caught him before he could go down. Once in the stands, every time Christian stood up and cheered with the crowd he’d feel what he later called a “whoops” sensation. The boys flanked him, ready for anything.

In the coming days and weeks, similar episodes unfolded with escalating variations. Even the slightest exertions would give Christian the illusion that he was tumbling. He began hearing the sound of his own heartbeat thrumming in his ear as he tried desperately to sleep, or the strange echoes of his joints moving when he tried to resume his jogging routine; a sudden sense of vertigo would seize him when the organ at church hit a certain note, forcing him to grip the pew.

By now, of course, Christian was making the rounds of local physicians. Some explored cardiac issues. Others probed his cranial vasculature with up-to-date imaging technologies. A Chicago ear, nose and throat expert devised a plan to improve bloodflow through the vertebral arteries. When that proved fruitless, the physician acknowledged: “I’m afraid there’s nothing we can do for you.”

Christian, meanwhile, shielded his condition from colleagues and the 150 young people he taught in his six daily high school classes. But his classroom habits changed drastically. Instead of weaving through the clusters of workgroups, he would stake out locations from which to lecture and remain fixed in place.

Finally, in December 2005—after 13 hellish months in the grip of this mysterious condition—Christian met a Peoria neurologist named Jorge Kattah who had heard similar balance problems described at a conference. One of the national experts on these rare disorders, Kattah had learned, was Lloyd Minor, the new director of the Department of Otolaryngology–Head and Neck Surgery at Johns Hopkins.

 

*****

 

From the moment Lloyd Minor came north from Little Rock, Arkansas, to enter Brown University in 1975, he knew he wanted to be a physician. He also knew he would choose a medical specialty that engaged his appetite for understanding how things work at an ever-deeper level.

By his junior year, Minor had become utterly captivated by a course in biomedical engineering that “used auditory and vestibular systems as models for showing how engineering principles could be used to understand biology.” Finishing Brown Medical School in 1982, Minor sailed into a core surgical residency at Duke and then—during a four-year postdoctoral research fellowship at the University of Chicago—homed in on vestibular physiology. But it was only after his appointment to the Johns Hopkins faculty in 1993 that Minor’s interests converged on the exotic puzzle of human dizziness. “It all came together here,” he says.

At Hopkins, Minor met David Zee, a School of Medicine neurologist who had already developed pathbreaking methods for evaluating patients with balance disorders. One of the areas that both Minor and Zee found fascinating was the long-established connection between balance problems and eye movement. Described a century earlier by Viennese scientist Julius Ewald—who’d opened the ear canals in pigeons to observe telltale motions in their eye movements in response to sound—the phenomenon had inspired the first of “Ewald’s laws”: “If you stimulate the semicircular canal, the eyes will move in the plane of that canal.”

Fascinated by this long-ago observation, Minor began tracking the eye movements in his patients with dizziness problems that couldn’t be easily explained. He wanted to know if the root of their disorder might lie in their semicircular canals. The answers came quickly. A number of patients, Minor found, had tiny holes in the upper arch of their inner ear cavities—right above the spot where the soft outer rim of the superior canal passed. These minute openings were allowing changes in intracranial pressure to cause the balance-sensitive canal to bulge. A sneeze might bring on the bulge. So might a cough, a shout, a deep breath, even a belly laugh. And whenever the bulge occurred, the person’s world would turn on its head. In some the problem was so severe it caused a chronic state of imbalance.

Minor’s team labeled these troublesome openings in the inner ear with the word dehiscence—the term botanists use to describe the holes left behind from a burst seedpod. He and his lab team now began working in the laboratory to try to develop a treatment that could give relief to the most debilitating of these cases by plugging the holes that were causing the dizziness.

By 1996, Minor felt confident that he had defined a little-known medical phenomenon. Dubbing the disorder superior canal dehiscence syndrome, he described it publicly for the first time in 1998 in the Archives of Otolaryngology–Head & Neck Surgery.

By then, Minor also had come up with a surgical solution for closing the dehiscence. “Think of that hole in the ruptured bone area as a hole in a tire,” he would tell patients. “It’s supposed to protect the soft inner tube beneath it, which is our superior canal. What we need to do is to close the hole.”

. . .
One of the surgeons points to the rupture in the patient’s ear cavity.  
> One of the surgeons points to the rupture in the patient’s ear cavity.

But given that this hole-patching would occur several inches into the brain cavity, not all SCD patients were quick to sign on for the procedure. Their decision point, Minor says, is based on whether they can control the problem, or the problem controls them.

Today, Minor and colleagues have collected data on 115 SCD patients. What’s become clear is that the disorder causes more problems than dizziness. Some patients report hearing their own bodily noises (autophony)—their heartbeat, for instance—conveyed through their bones into their hearing center. Some can even hear their own eyeballs moving within their sockets, which sounds a bit like feet sloshing in wet shoes. For a portion of those patients, the disorder had taken over their life, so they were willing to risk even delicate, potentially dangerous surgery to be rid of it.

So far, 39 of Minor’s patients have reached such a point.

 

*****

 

On a thursday afternoon in May, Richard Christian reclines in a motorized examining chair as it whirs and lifts him into position. “I love moving up in the world,” he says, careful not to laugh at his own joke lest it induce a wave of vertigo.

When Christian’s file first came to Lloyd Minor months earlier, it had looked like an obvious SCD case. Detailed physical examinations in March had confirmed those suspicions. Once sure of the diagnosis, Minor had explained to Christian the delicate operation that could give him back his balance. The burden of deciding whether to go ahead with the surgical intervention was then his. Christian returned to Peoria and spent two more months falling and with much-limited movement. Today, he is ready for surgery.

So are Minor and his team. But a final series of confirmatory tests are in order. Minor asks Christian to perform the Valsalva maneuver, a simple gesture in which the patient takes a deep breath and holds it while contracting his stomach muscles, automatically raising his intracranial pressure. Instantly, Minor spots the “torsional” eye movements showing the brain’s reaction to the tumbling sensation. Minor then takes out a tuning fork and asks Christian to expose one ankle. The doctor taps the fork and places it to his patient’s ankle bone, attempting to induce one of the more exotic symptoms that afflict some patients—the tuning fork’s tone would be clearly heard in the patient’s ear. Christian doesn’t hear the tone, but the rest of the symptoms are classically consistent with SCD stemming from a dehiscence in the area of the right ear. The operation will proceed.

“You’ll have some unsteadiness when you get out of bed Saturday morning,” Minor explains while taking Christian through the required permissions signatures and describing the normal surgical risks. Then he adds that, of the patients on whom he has performed the procedure, one experienced postsurgical bleeding. “It is possible that this operation is not going to fix the problem,” Minor adds. But all of his patients so far have experienced relief.

Now, with the looming certainty that his skull will be opened up, Christian is sitting side-saddle in the examining chair, flexing and unflexing his left arm. “This is not a very painful operation,” Minor assures him, “though you’re likely to experience some pain while chewing” in the weeks after the procedure.

 

*****

 

At 9:15 a.m. the next morning, Christian lies in a deep sleep on the operating table as five active screen monitors are lit to aid the 11 members of the surgical team. One monitor shows a copper-colored three-dimensional rendering of Christian’s skull. A superimposed grid with arrows will guide the procedure.

Lloyd Minor contemplates the image with his arms folded while his key associate, John Carey, instructs a pair of residents as he moves into the procedure. “Unlock the bed,” Carey says, shifting his position on a stool and cradling the unconscious patient’s head, turning it just so while adjusting the overhead surgical lights. As the preoperative fine-tuning unfolds, the patient’s head beams like an enshrined celestial object at the center of the room’s attention. Carey asks, “Can I have a marking pen, please?”

At 9:50 a.m., the incision is made with a number 15 blade.

With the craniotomy well under way an hour later, Minor observes the work of Carey and surgical resident Mark Eisen and narrates for the benefit of two first-year residents who are sitting in. “You’re seeing that the dura is nice and healthy,” he says. “It’s well vascularized.”

Within moments Carey’s gloved fingertips gently clasp a piece of the patient’s skull bone roughly one inch square. It will be set aside for the operation’s closing phase.

Meanwhile, Minor quietly begins toiling at a side table, crafting smaller bits of the patient’s excised tissue into material that will form the “patch” to cover the dehiscence. He collects thin strips of fascia, trimming them with surgical scissors and blending them with bone chips. The two residents stand back and watch the unfolding microsurgery unobstructed.

“Nice,” says Minor, alternating his gaze between the actual surgical site and one screen’s grid display, from which he can see the progress of the surgical resident’s probe in a CT scan image. “We’re close,” says Minor. “Go a little anterior.”

John Carey steps back to consult another grid-mapped monitor. The screen shows the precise target. “According to this,” Carey says confidently, “you’re right over it.”

Eisen now gently uses a cotton ball to clear the sliver of space where his probe has pushed the brain sac’s protective dura from an inner layer of bone. In moments it shows a telltale cleft, like a small lengthwise hole at the bottom of a crease left by a paper clip pressed into wet cement. “There’s the dehiscence,” announces Eisen. It’s 11:22, just 82 minutes after the first incision.

. . .
Today, Richard Christian says he no longer has to “bump into walls just to go straight.”  
> Today, Richard Christian says he no longer has to “bump into walls just to go straight.”
“We’re  closer to a real understanding of this problem,” says Lloyd Minor.
> “We’re  closer to a real understanding of this problem,” says Lloyd Minor.

The surgeons study the opening, ensuring that they know the obstacle on the most intimate terms. The light of the microscope soon reveals a trace of a clear membrane through the bone’s tiny opening. It’s the outermost layer of Richard Christian’s superior canal, the soft tissue that has bulged through the opening into his brain cavity for 18 months now, repeatedly toying with his equilibrium and his broader sense of well-being.

Minor looks pleased. “This is going to be a little like that one two weeks ago,” he tells his partners.

Carey signals that it’s time for the prepared patching tissues.

From the microscope’s screen display, the students gaze intently while Eisen maneuvers the patch materials—small chips of bone from Christian’s skull and a glue made of clotting proteins—into place. Neatly positioning a series of the patches onto the cleft area of bone, Eisen carefully tamps them into the canal’s opening. Slowly, the surgeon allows the brain’s dura to start slipping back over the spot. Periodically, he lifts it, adjusting the patch until all three surgeons are satisfied with the seal. 

With the operation’s most delicate stage behind them, Carey offers details about SCD to the observing residents. He describes how his group has been tracking one patient for 10 years, supporting his decision not to undergo the operation. The man finds his condition tolerable so far, Carey says, “but he doesn’t go to concerts, and he doesn’t sing in the shower.”

 

*****

 

“I no longer have to bump into walls just to go straight,” announces Richard Christian just two weeks after his procedure. “In just the last four days, I’ve gotten a whole lot more stability and maneuverability back than I’d ever imagined I’d have again.” What’s more, he’d felt only modest pain during early recovery. Now he can even laugh about trying to “unlearn” the coping strategies he’d developed during his 18-month ordeal. He no longer has to lean on his wife. He needn’t fear slopes and stairs. His sensations of disequilibrium are “virtually all gone.”

Minor says Christian’s was a textbook case, “very straightforward.” Still, his research group is learning more about SCD all the time now. They’ve found, for instance, that some patients suffer autophony problems without the balance disorders, which could mean earlier diagnosis. And in a reference to the apparatus the Hopkins team now uses to confirm the syndrome—an elaborate lab system designed by David Zee—Minor says simpler diagnostic tools are now in the pipeline here. These will be available for physicians across the country to use in their own offices. And this new step, Minor says, would spare these patients from having to make long-distance treks just for a diagnosis. What’s clear, Minor adds, is that “we’re getting much closer to a real understanding of this problem.”*

 
 
 
 
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 The Free-Radical Dilemma
 
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 Circling the Dome
 Medical Rounds
 Annals of Hopkins
 
Class Notes
 The Brain Voyager
 
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 Learning Curve
 Post-Op
 
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