Bioengineering,
Journal Year:
2024,
Volume and Issue:
11(5), P. 425 - 425
Published: April 26, 2024
Sensorineural
hearing
loss
(SNHL)
is
a
prevalent
and
growing
global
health
concern,
especially
within
operational
medicine,
with
limited
therapeutic
options
available.
This
review
article
explores
the
emerging
field
of
in
vitro
otic
organoids
as
promising
platform
for
modeling
developing
novel
strategies.
SNHL
primarily
results
from
irreversible
or
dysfunction
cochlear
mechanosensory
hair
cells
(HCs)
spiral
ganglion
neurons
(SGNs),
emphasizing
need
innovative
solutions.
Current
interventions
offer
symptomatic
relief
but
do
not
address
root
causes.
Otic
organoids,
three-dimensional
multicellular
constructs
that
mimic
inner
ear’s
architecture,
have
shown
immense
potential
several
critical
areas.
They
enable
testing
gene
therapies,
drug
discovery
sensory
cell
regeneration,
study
ear
development
pathology.
Unlike
traditional
animal
models,
closely
replicate
human
pathophysiology,
making
them
invaluable
translational
research.
discusses
methodological
advances
organoid
generation,
use
pluripotent
stem
(hPSCs)
to
development.
Cellular
molecular
characterization
efforts
identified
key
markers
pathways
essential
development,
shedding
light
on
their
disorders.
Technological
innovations,
such
3D
bioprinting
microfluidics,
further
enhanced
fidelity
these
models.
Despite
challenges
limitations,
including
standardized
protocols
ethical
considerations,
transformative
approach
understanding
treating
auditory
dysfunctions.
As
this
matures,
it
holds
revolutionize
treatment
landscape
balance
disorders,
moving
us
closer
personalized
medicine
conditions.
Cell Proliferation,
Journal Year:
2023,
Volume and Issue:
56(5)
Published: Feb. 24, 2023
Abstract
Otic
neurons,
also
known
as
spiral
ganglion
neurons
(SGNs)
in
mammalian
cochlea,
transmit
electrical
signals
from
sensory
hair
cells
to
cochlear
nuclei
of
the
auditory
system.
SGNs
are
sensitive
toxic
insults,
vulnerable
get
irreversible
damaged
and
hardly
regenerate
after
damage,
causing
persistent
sensorineural
hearing
loss.
Yet,
authentic
for
research
or
therapeutic
purpose
remains
challenging.
Here
we
developed
a
protocol
generate
human
otic
neuronal
organoids
(hONOs)
pluripotent
stem
(hESCs),
which
hESCs
were
step‐wisely
induced
corresponding
stages
according
their
developmental
trajectory.
The
hONOs
enriched
SGN‐like
at
early
stage,
both
astrocytes,
Schwann
supporting
thereafter.
In
these
hONOs,
determined
existence
typical
Type
I
II
SGNs.
Mature
(at
differentiation
Day
60)
formed
neural
network,
featured
by
giant
depolarizing
potential
(GDP)‐like
events
rosette‐organized
regions‐elicited
calcium
traces.
Electrophysiological
analysis
confirmed
glutamate‐responsive
hONOs.
generated
this
study
provide
an
ideal
model
related
disorders,
facilitating
development
Fundamental Research,
Journal Year:
2023,
Volume and Issue:
unknown
Published: Dec. 1, 2023
The
inner
ear
is
one
of
the
most
complicated
structures
that
harbor
organs
for
perception
sound
and
balance,
which
deep
in
temporal
bone
challenging
to
operate.
Organoids
serve
as
promising
platforms
understanding
developmental
processes
pathological
dysfunctions
discovering
therapeutic
drugs
gene
therapy
strategies
disorders
ear.
To
better
understand
origin
application
value
organoids,
we
reviewed
history
advancement
organoid
research.
We
summarized
cell
sources
organoids
matrices
supporting
their
formation.
research
on
derived
from
pluripotent
stem
cells
(PSCs)
primary
progenitor
has
been
clarified
detail.
elaborated
applications
development,
hereditary
deafness
modeling,
hair
(HC)
regeneration
strategy
formulation.
Finally,
mentioned
limitations
current
culture
methods
described
several
prospects
optimizing
next-generation
potential
translational
applications.
Neuroscience Bulletin,
Journal Year:
2023,
Volume and Issue:
40(7), P. 963 - 980
Published: Nov. 28, 2023
Hearing
loss
and
deafness,
as
a
worldwide
disability
disease,
have
been
troubling
human
beings.
However,
the
auditory
organ
of
inner
ear
is
highly
heterogeneous
has
very
limited
number
cells,
which
are
largely
uncharacterized
in
depth.
Recently,
with
development
utilization
single-cell
RNA
sequencing
(scRNA-seq),
researchers
able
to
unveil
complex
sophisticated
biological
mechanisms
various
types
cells
at
level
address
challenges
cellular
heterogeneity
that
not
resolved
through
by
conventional
bulk
(bulk
RNA-seq).
Herein,
we
reviewed
application
scRNA-seq
technology
research,
aim
providing
reference
for
organs,
pathogenesis
hearing
loss,
regenerative
therapy.
Prospects
about
spatial
transcriptomic
scRNA-seq,
based
genome,
Live-seq
will
also
be
discussed.
Cell Proliferation,
Journal Year:
2024,
Volume and Issue:
unknown
Published: Dec. 15, 2024
G
protein-coupled
receptor-associated
sorting
protein
2
(GPRASP2)
has
been
identified
as
the
causative
gene
for
X-linked
recessive
syndromic
hearing
loss
(SHL)
in
our
previous
study.
However,
role
of
GPRASP2
auditory
function
remains
unclear.
The
present
study
demonstrated
that
Gprasp2
overexpression
mouse
organoids
promoted
proliferation
supporting
cells
(SCs),
which
was
mainly
mediated
by
Hedgehog
signalling
pathway.
Meanwhile,
hair
cell
(HC)
formation
from
SCs
via
β-catenin
signalling.
In
addition,
deficiency
resulted
increased
lysosomal
degradation
SMO
protein,
leading
to
decreased
expression
and
pathway
transcription
factor
GLI1.
neomycin-treated
cochlear
explant,
smoothened
agonist
(SAG)
recured
HC
further
facilitated
AAV-ie-Gprasp2
promote
HCs.
Our
results
suggested
could
be
a
potential
candidate
therapy
regeneration
Materials Today Bio,
Journal Year:
2023,
Volume and Issue:
24, P. 100937 - 100937
Published: Dec. 28, 2023
Spiral
ganglion
neurons
(SGNs)
in
the
mammalian
cochleae
are
essential
for
delivery
of
acoustic
information,
and
damage
to
SGNs
can
lead
permanent
sensorineural
hearing
loss
as
not
capable
regeneration.
Cochlear
glial
cells
(GCs)
might
be
a
potential
source
SGN
regeneration,
but
neuronal
differentiation
ability
GCs
is
limited
its
properties
clear
yet.
Here,
we
characterized
cochlear
Sox10-positive
(Sox10+)
neural
progenitor
population
developed
basement
membrane
extract-based
three-dimensional
(BME-3D)
culture
system
promote
generation
capacity
vitro.
Firstly,
purified
Sox10+
GCs,
isolated
from
Sox10-creER/tdTomato
mice
via
flow
cytometry,
were
able
form
neurospheres
after
being
cultured
traditional
suspension
system,
while
significantly
more
found
expression
stem
cell-related
genes
was
upregulated
BME-3D
group.
Next,
promoted
evidenced
by
increased
number,
neurite
outgrowth,
area
growth
cones,
synapse
density
well
excitability
newly
induced
neurons.
Notably,
also
intensified
reinnervation
generated
with
HCs
protected
derived-neurons
against
cisplatin-induced
damage.
Finally,
transcriptome
sequencing
analysis
performed
identify
characteristics
differentiated
These
findings
suggest
that
considerably
promotes
proliferation
efficiency
vitro,
thus
providing
possible
strategy
regeneration
study.
Bioengineering,
Journal Year:
2024,
Volume and Issue:
11(5), P. 425 - 425
Published: April 26, 2024
Sensorineural
hearing
loss
(SNHL)
is
a
prevalent
and
growing
global
health
concern,
especially
within
operational
medicine,
with
limited
therapeutic
options
available.
This
review
article
explores
the
emerging
field
of
in
vitro
otic
organoids
as
promising
platform
for
modeling
developing
novel
strategies.
SNHL
primarily
results
from
irreversible
or
dysfunction
cochlear
mechanosensory
hair
cells
(HCs)
spiral
ganglion
neurons
(SGNs),
emphasizing
need
innovative
solutions.
Current
interventions
offer
symptomatic
relief
but
do
not
address
root
causes.
Otic
organoids,
three-dimensional
multicellular
constructs
that
mimic
inner
ear’s
architecture,
have
shown
immense
potential
several
critical
areas.
They
enable
testing
gene
therapies,
drug
discovery
sensory
cell
regeneration,
study
ear
development
pathology.
Unlike
traditional
animal
models,
closely
replicate
human
pathophysiology,
making
them
invaluable
translational
research.
discusses
methodological
advances
organoid
generation,
use
pluripotent
stem
(hPSCs)
to
development.
Cellular
molecular
characterization
efforts
identified
key
markers
pathways
essential
development,
shedding
light
on
their
disorders.
Technological
innovations,
such
3D
bioprinting
microfluidics,
further
enhanced
fidelity
these
models.
Despite
challenges
limitations,
including
standardized
protocols
ethical
considerations,
transformative
approach
understanding
treating
auditory
dysfunctions.
As
this
matures,
it
holds
revolutionize
treatment
landscape
balance
disorders,
moving
us
closer
personalized
medicine
conditions.
