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Developmental changes of ENaC expression and function in the inner ear of pendrin knock-out mice as a perspective on the development of endolymphatic hydrops.
[pendred syndrome]
Pendrin
mutations
cause
enlarged
vestibular
aqueducts
and
various
degrees
of
sensorineural
hearing
loss
.
The
selective
abolition
of
pendrin
causes
dilation
of
the
membranous
labyrinth
known
as
endolymphatic
hydrops
,
loss
of
the
endocochlear
potential
,
and
consequently
loss
of
hearing
function
.
Because
Na
+
transport
is
one
of
the
most
important
driving
forces
for
fluid
transport
,
the
epithelial
Na
+
channel
(
ENaC
)
is
believed
to
play
an
important
role
in
fluid
volume
regulation
in
the
inner
ear
.
Therefore
,
the
dysfunction
of
Na
+
transport
through
ENaC
by
the
acidification
of
endolymph
in
Pendred
syndrome
is
one
of
the
potential
causes
of
endolymphatic
hydrops
.
We
investigated
the
changes
of
ENaC
expression
and
function
during
the
development
of
the
pendrin
knock-out
mouse
.
In
the
cochlea
,
the
expression
of
β
and
γENaC
was
significantly
increased
at
P
56
in
Pds-
/
-
mice
compared
with
Pds
+
/
+
mice
.
In
the
vestibule
,
the
expression
of
βENaC
was
significantly
increased
at
P
56
,
and
γENaC
expression
significantly
increased
from
P
6
to
P
56
in
Pds-
/
-
mice
.
The
ENaC-dependent
trans-epithelial
current
was
not
significantly
different
between
Pds
+
/
+
and
Pds-
/
-
mice
in
Reissner
's
membrane
or
the
saccular
extramacular
roof
epithelium
at
P
0
,
but
the
current
was
significantly
increased
in
Pds-
/
-
mice
at
P
56
compared
with
Pds
+
/
+
mice
.
These
findings
indicate
that
the
expression
and
function
of
ENaC
were
enhanced
in
Pds-
/
-
mice
after
the
development
of
endolymphatic
hydrops
as
a
compensatory
mechanism
.
This
result
provides
insight
into
the
role
of
Na
+
transport
in
the
development
and
regulation
of
endolymphatic
hydrops
due
to
pendrin
mutations
.
