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Robert Wood Johnson
daughter pairs with the condition.
Repair of the third window depends on precisely where the
hole is, as there are at least six separate places in the inner ear
where it can occur. "But the basic concept is to go from three
windows to two," Dr. Wackym says. "If we can access the hole,
during a craniotomy we plug it with the patient's own tissue
and repair any other defects that are there." Some of the
holes--or windows--are in places that can't be accessed. In the
cases where the location of the third window is in a place where
surgery could cause additional hearing loss or facial paralysis--
for example, between the cochlea and carotid artery or the
cochlea and facial nerve--other surgical strategies are used. In
these cases, Dr. Wackym closes a different window to change
the biomechanical properties of the inner ear.
Helping Joey
here are certain obvious external signs of third window
syndrome. "Patients typically look ashen; their faces look
older. They have circles under their eyes," Dr. Wackym says.
Those were visible indications that Joey Zarello was suffering
from the condition. To learn signs, during Joey's consultation
at Robert Wood Johnson Medical School, Dr. Wackym began
by asking questions and letting him talk. "As is typical of these
patients, Joey had trouble stringing words together," Dr.
Wackym says. During a structured interview and examination,
he explained the balance issues, applied ear pressure, and
watched eye movements. "When there is a hole in the inner ear,
there are predictable responses," Dr. Wackym says. With a spe-
cial high-resolution temporal bone CT and other electrophysi-
ological tests, many of which are available only here, the diag-
nosis was confirmed as superior semicircular canal dehiscence.
It was a relief to finally have an answer.
Dr. Wackym performed the craniotomy--making an opening
in Joey's skull, so he could surgically close the boy's third win-
dow--on April 11 at The Bristol-Myers Squibb Children's
Hospital at Robert Wood Johnson University Hospital (RWJ).
An incision was made in front of the ear and through the large,
fan-shaped temporalis, or temporal muscle. Then an opening in
the skull was made, and the brain was lifted to access the base of
the skull. Using an endoscope, Dr. Wackym identified the third
window and then plugged it with Joey's own tissue. A covering
was fabricated with bone cement, and everything was put back
in place and his skull was repaired with titanium mesh and bone
cement, which, over time, his living bone will integrate into.
Patients like Joey typically stay in the hospital two days.
During the first three days of recovery, a different kind of ver-
tigo is experienced. It takes approximately six weeks for the
brain to adjust to the new mechanics of the inner ear. When the
adjustment takes place, patients start to regain their lives.
After surgery, it took some time for Joey to get his energy
back, and then he began to notice that things were different.
The vertigo stopped. Headaches became less frequent and less
painful. Now he's looking forward to learning to ride a bike
and going on a roller coaster--things he couldn't have dreamed
of doing before the surgery. And this summer he worked at his
first job, working on a boardwalk at the Jersey Shore.
Joey talks about how much this surgery has changed his life:
"I'm thinking, it's finally over. I'm looking back at all the
headaches and vertigo. I'm thinking it was so annoying I would
have this. After seven years of having this, I feel amazing. I feel
like a new person."
"Joey is totally back to normal and healthy," Dr. Wackym
says. Joey is the first child to have this operation at The Bristol-
Myers Squibb Children's Hospital at RWJ. In fact, this type of
surgery was unavailable for anyone at the Robert Wood
Johnson University Hospital or The Bristol Myers-Squibb
Children's Hospital prior to Dr. Wackym's arrival. Dr. Wackym
is among the top three surgeons in the country by volume for
this procedure and patients regularly travel here for surgery
from across the United States and occasionally from Europe.
Building a Department
n internationally renowned neurotologist, Dr. Wackym is
one of the most experienced surgeons for superior semi-
circular canal dehiscence, cochlear implants, and skull base
tumors in the United States. He was the first neurotologist in
the world to perform Gamma Knife radiosurgery. He comes to
the medical school from Legacy Health in Portland, Oregon,
where for seven years he served as vice president of research
and built a thriving clinical practice. Previously, Dr. Wackym
served for more than a decade as the John C. Koss Professor
and Chair, Department of Otolaryngology and Communication
Sciences, at the Medical College of Wisconsin, Milwaukee.
Chair of the American Neurotology Society's Stereotactic
Radiosurgery Study Group, Dr. Wackym has been a senior and
neurotology examiner for the American Board of Otolaryn-
gology. He has published more than 150 peer-reviewed manu-
scripts and is senior editor of Ballenger's Otorhinolaryngology
Head and Neck Surgery
--a book that was first published more
than 100 years ago--as well as an editor of many other works in
the field. He has also received more than two decades of research
support from the National Institutes of Health (NIH) and other
agencies and foundations.
As chair of the Department of Otolaryngology­Head and
Neck Surgery, Dr. Wackym is bringing his expertise in ear and
skull base tumor surgery to the medical school. Other proce-