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GFAP aggregates in the cochlear nerve increase the noise vulnerability of sensory cells in the organ of Corti in the murine model of Alexander disease.
[alexander disease]
Outer
hair
cell
(
OHC
)
loss
in
the
auditory
sensory
epithelium
is
a
primary
cause
of
noise-induced
sensory
-neural
hearing
loss
(
SNHL
)
.
To
clarify
the
participation
of
glial
cells
in
SNHL
,
we
used
an
Alexander
disease
(
AxD
)
mouse
model
.
These
transgenic
mice
harbor
the
AxD
causal
mutant
of
the
human
glial
fibrillary
acidic
protein
(
GFAP
)
under
the
control
of
the
mouse
GFAP
promoter
.
It
is
thought
that
GFAP
aggregates
compromise
the
function
of
astrocytes
.
In
the
auditory
pathway
,
the
formation
of
GFAP
aggregates
was
observed
only
in
GFAP
-
positive
cells
of
the
cochlear
nerve
.
The
presence
of
GFAP
aggregates
did
not
change
auditory
function
at
the
threshold
level
.
To
assess
the
change
in
vulnerability
to
auditory
excitotoxicity
,
both
transgenic
and
control
mice
were
treated
with
intense
noise
exposure
.
Auditory
threshold
shifts
were
assessed
by
auditory
brainstem
responses
(
ABR
)
at
1
and
4
weeks
after
noise
exposure
,
and
OHC
damage
was
analyzed
by
quantitative
histology
at
4
weeks
after
exposure
.
Transgenic
mice
showed
more
severe
ABR
deficits
and
OHC
damage
,
suggesting
that
cochlear
nerve
glial
cells
with
GFAP
aggregates
play
a
role
in
noise
susceptibility
.
Thus
,
we
should
focus
more
on
the
roles
of
cochlear
nerve
glial
cells
in
SNHL
.
Diseases
Validation
Diseases presenting
"hearing loss"
symptom
22q11.2 deletion syndrome
achondroplasia
adrenomyeloneuropathy
alexander disease
benign recurrent intrahepatic cholestasis
canavan disease
cohen syndrome
congenital toxoplasmosis
dentinogenesis imperfecta
fabry disease
familial mediterranean fever
heparin-induced thrombocytopenia
hirschsprung disease
holt-oram syndrome
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
kabuki syndrome
kallmann syndrome
neonatal adrenoleukodystrophy
pendred syndrome
von hippel-lindau disease
wolf-hirschhorn syndrome
This symptom has already been validated