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.”
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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.”
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| > One
of the surgeons points to the rupture
in the patient’s ear
cavity. |
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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.
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| > Today,
Richard Christian says he no longer
has to “bump into
walls just to go straight.” |
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| > “We’re closer
to a real understanding of this problem,” says
Lloyd Minor. |
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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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