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Cochlear implantation in Pendred syndrome.
[pendred syndrome]
The
objective
of
this
study
was
to
examine
the
anatomical
,
audiological
,
and
surgical
factors
that
can
affect
the
hearing
outcome
in
Pendred
syndrome
cochlear
implant
recipients
.
Patients
with
Pendred
syndrome
represent
challenging
cochlear
implant
candidates
,
combining
goiter
,
severe
-
to
-
profound
hearing
loss
,
and
inner-
ear
dysplasias
.
The
hearing
outcome
and
further
relevant
parameters
in
such
patients
have
not
been
adequately
evaluated
yet
.
Retrospective
study
in
a
tertiary
referral
cochlear
implant
centre
.
Data
of
patients
with
Pendred
syndrome
,
who
had
undergone
cochlear
implantation
in
the
10
-
year
period
1999
-
2008
,
were
evaluated
.
Five
cochlear
implant
recipients
with
Pendred
syndrome
were
identified
,
four
unilateral
and
one
bilateral
.
The
average
postoperative
follow-up
duration
for
these
patients
was
4
.
8
years
.
Enlarged
vestibular
aqueduct
and
Mondini
dysplasia
were
the
most
common
anatomical
findings
.
Although
these
anatomical
variances
did
not
affect
the
electrode
insertion
,
they
prolonged
the
duration
of
the
surgery
.
Cerebrospinal
fluid
gusher
was
the
most
common
intraoperative
complication
.
All
patients
enjoyed
satisfactory
hearing
rehabilitation
.
Cochlear
implantation
is
the
proper
method
for
optimal
hearing
rehabilitation
in
patients
with
Pendred
syndrome
.
Although
the
precise
pathogenetic
mechanism
and
the
genetic
background
of
deafness
in
Pendred
syndrome
have
not
been
completely
understood
,
the
preexisting
hearing
experience
represents
a
positive
factor
for
satisfying
hearing
outcome
.
The
inner-
ear
malformations
can
cause
mild
surgical
difficulties
and
extended
surgery
duration
.
The
cochlear
implant
surgeon
should
be
aware
of
these
difficulties
to
avoid
complications
.
Diseases
Validation
Diseases presenting
"further relevant parameters in such patients have not been adequately evaluated yet"
symptom
pendred syndrome
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