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18 Robert WoodJohnson
offers parents and children complete evaluation, treat-
ment, and rehabilitation services related to cochlear
implants. By the end of the program's first year, the team
had completed 10 cochlear implants in children ranging in age
from 13 months to 5 years old.
A Shocking Discovery
hen Annabella was born on June 10, 2013, Florio
never imagined her daughter would need these types
of services; Annabella had passed her newborn hearing
screening with no indications of any issues. But just three
months later, Florio was noticing that there might be a
"She wasn't responding to any noises around the house.
You could bang pots and pans right next to her, and there
would be no reaction," Florio recalls. "When I brought her
to the pediatrician for her 4-month-old check-up, they did
another hearing test, but they got no reading off of it."
A hearing test the following week yielded the same
result. A subsequent auditory brainstem response test--
which measures the way the hearing nerve responds to dif-
ferent sounds--confirmed that Annabella had sensorineur-
al hearing loss.
In sensorineural hearing loss, damage to the inner ear
prevents the process by which sound travels to the brain,
whether due to missing or damaged hair cells, or damage to
the nerve pathway itself. A cochlear implant bypasses the
process, transmitting sound from an external speech
processor and transmitter to an internal receiver that gen-
erates electrical pulses directly to the auditory nerve itself.
Finding Some Answers
ne of Annabella's teachers at the Midland Park Schools'
early intervention program for deaf and hard-of-
hearing children first mentioned the Robert Wood Johnson
Pediatric Cochlear Implant Team to Florio, prompting her to
do some in-depth online research about the team and cochlear
implants in general. What she discovered had her reaching
out to learn more, she says.
Months of tests followed, including MRIs, CT scans, and
genetic testing, which revealed that Annabella has connex-
in 26--a protein found on the GJB2 gene that disrupts
potassium flow in the ear and is the most common cause of
congenital sensorineural hearing loss.
Annabella was 16 months old when the first cochlear
device was implanted in her left ear in October 2014, and
it was activated a month later; the remaining device was
implanted in her right ear at the end of April 2015 and
activated in June.
"With the first implant, we noticed that, yes, she could
hear something. But as soon as she got that second implant,
it was like a switch was flipped and everything started to go
really quickly," Florio says. "She's trying to say her ABCs,
she knows about eight different colors and the noises of dif-
ferent animals--says `ooo' for `moo'--and has really start-
ed catching up a lot quicker than what was expected. It's
wonderful. She's babbling, making noises, and starting to
sing. She knows the difference between when people are
speaking and when they are singing. Every time she hears
music, she stands up and dances."
Hard Work ... and Teamwork
lorio is well aware that the process is just beginning.
Annabella is involved in classes and therapy four days a
week, including sessions with a speech therapist, as well as
twice-weekly classes where she interacts with other children
who have hearing loss. She sees a member of the cochlear
implant team every few weeks for "mapping," or adjustments
to the sound programming of the device. Once mapping on
both sides is complete, Annabella will return annually for any
necessary tweaks to the programming, Florio says.
"It can take approximately a year to stabilize the sound
mapping so the implant can be customized to each child's
individual hearing loss," explains Dr. Kwong.
"While the surgery itself takes only a few hours, and
most children are home the same day, it's not just a simple
procedure and then you're done," he adds. "The process is
very labor-intensive after the surgery, and a committed
team is needed to help lay the groundwork for success."
It takes dedication from the family and everyone else
involved, agrees Dr. Chee. "It's very intensive, but fortu-
nately there is a lot of support from the team. They are not
in it alone," he says.
Clinical developmental psychologists with the Institute
for the Study of Child Development in the medical school's
Department of Pediatrics work with the program to help
families understand the developmental and cognitive poten-
tial of their young child, consider potential obstacles, and
help them cope with the stresses and anxieties associated
with the procedure. After surgery, they also are available to
assist as children learn to understand new sounds and
adjust to life with their implant.
In addition, the Pediatric Cochlear Implant Team has
developed partnerships with other organizations--for