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Congenital cholesteatoma and cochlear implantation: Implications for management.
[pendred syndrome]
Cholesteatoma
is
an
uncommon
condition
that
has
occasionally
been
associated
with
cochlear
implantation
(
CI
)
.
Cases
of
secondary
acquired
cholesteatoma
have
been
described
,
in
which
intra-operative
breech
of
the
posterior
canal
wall
is
thought
to
be
a
contributing
factor
.
Primary
acquired
cholesteatoma
is
not
typically
associated
with
congenital
sensorineural
hearing
loss
(
SNHL
)
or
CI
in
children
.
Congenital
cholesteatoma
is
a
rarer
entity
yet
with
an
incidence
in
the
literature
of
2
â€
4
%
of
all
cholesteatomas
.
We
present
lessons
learned
from
our
experience
of
congenital
cholesteatoma
in
CI
candidates
.
Retrospective
reviews
of
departmental
CI
and
cholesteatoma
databases
in
a
tertiary
/
quaternary
pediatric
center
were
conducted
.
Cases
of
congenital
cholesteatoma
were
identified
.
The
proportion
of
congenital
cholesteatoma
cases
in
CI
candidates
was
compared
with
number
of
acquired
cholesteatoma
.
Optimum
management
of
congenital
cholesteatoma
in
CI
candidates
was
reviewed
.
In
our
pediatric
CI
population
,
2
/
794
patients
(
0
.
25
%
)
were
recognized
as
having
a
congenital
cholesteatoma
during
their
evaluation
for
CI
.
No
cases
of
primary
acquired
cholesteatoma
were
identified
in
this
population
at
presentation
or
at
follow
up
to
18
years
.
The
0
.
25
%
incidence
of
congenital
cholesteatoma
in
our
population
of
CI
patients
is
higher
than
expected
of
this
rare
condition
.
It
is
surprisingly
common
given
the
absence
of
any
cases
of
primary
acquired
cholesteatoma
,
which
is
considerably
more
common
even
in
the
pediatric
population
.
Both
patients
likely
had
an
inherited
form
of
hearing
loss
and
a
genetic
contribution
to
the
presence
of
congenital
cholesteatoma
can
not
be
excluded
.
The
presence
of
congenital
cholesteatoma
has
implications
for
the
algorithm
currently
employed
for
the
assessment
of
CI
.
We
consider
that
surgery
should
be
staged
to
ensure
complete
removal
of
the
cholesteatoma
before
implantation
.
Thus
bilateral
CI
should
be
provided
sequentially
rather
than
simultaneously
in
the
presence
of
unilateral
cholesteatoma
.
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"a genetic contribution to the presence of congenital cholesteatoma cannot be excluded"
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