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INNER EAR ANATOMY

BY DR.KARISHMA MISHRA
ENT PG-1st year,
KIMS,BBSR
EMBRYOLOGY
 Development starts in 3rd week of IUL & is completed by 25th week.

Surface ectoderm of hindbrain(at the level of first occipital somite)


Otic placode
Invaginate into mesenchyme
Otic pit
Otic vesicle/otocyst (membranous labyrinth),mesoderm surrounding it
forms bony labrrinth
Otic vesicle/otocyst

Medial Lateral

Endolymphatic Utriculosaccular
diverticulum chamber
Utrirosaccular chamber
SACCULAR CHAMBER UTRICULAR
CHAMBER
Sacculus AND Cochlear duct Utricules AND
SCC 8th wk

Ductus reuniens
Sup>post>LAT
Dilations of one end of each of
the semicircular ducts become
the ampullae, and the ampullar
ends and the opposite ends of
the ducts remain connected to
the utriculus.
WEEK ADULT FORM

9th HAIR CELLS

14-16th MACULAE

23rd CRISTAE

25th ORGAN OF CORTI

The mesoderm surrounding the membranous labyrinth becomes


the bony otic capsule, or bony labyrinth.
The membranous labyrinth is suspended in fluid (perilymph)
within the bony labyrinth by a loose connective tissue known as
periotic tissue.
ANATOMY

 The inner ear lies inside the petrous temporal bone between
medial wall of the middle ear and internal auditory canal.It
is composed of:
1)Bony labyrinth
2)Membranous labyrinth
 Membranous labyrinth is filled with endolymph.
Between membranous & bony labyrinth perilymph is
present.
BONY LABYRINTH

Parts:-
1.Vestibule
2.Semicircular canal
3.Cochlea
 Its bone has three layers:
an inner, or endosteal layer;
an outer, or periosteal layer;
and a middle layer consisting
of enchondral and
intrachondrial bone.
1.VESTIBULE
It is central part of bony labyrinth with boundries:-
Lateraly- medial wall of middle ear
Medialy- Internal acoustic meatus
Anterior-Cochlea
Posterior- Semicircular canal
a. Lateral wall: It has oval window.
Oval window (fenestra vestibuli): It lies In the lateral wall and closed by
footplate of stapes surrounded by annular ligament.

b. Medial wall: It shows following structures:


i. Spherical recess: It is situated anteriorly and lodges the saccule.
Perforations of maculae cribrosa media provides passage for fibers of inferior
vestibular nerve.

ii. Elliptical recess: It is situated posteriorly and lodges the utricle. The
perforations of maculae cribrosa superior (Mike’s dot) provide passage to
nerve fibers that supply to utricle and ampulla of superior and lateral
semicircular canals (SCC).
iii. Vestibular crest :
The spherical and elliptical recesses are
separated from each other by vestibular
crest.
Inferiorly vestibular crest splits to
enclose cochlear recess for cochlear
nerve fibers.

iv. Opening of aqueduct of vestibule:


Present below the elliptical recess. Through this passes the endolymphatic
duct.

c. Posterosuperior region:-Five openings of semicircular canals


d. Anterior: Cochlea opens into the anterior region of vestibule.
Semicircular Canals:

There are three SCCs:


1.Lateral(horizontal)
2.Posterior(vertical)and
3.Superior (anterior).

Each canal occupies 2/3rd of a circle and has a


diameter of 0.8 mm.
They lie in planes at right angles to one another.
Each canal has two ends: ampullated(2mm in
diameter) and nonampullated(1mm in diameter) .
1. Superior (anterior):-
 15–20 mm long
 Situated transverse to the axis of petrous part of temporal bone.
 Its anterolateral end is ampullated and opens in the superolateral part of
vestibule.

2. Lateral(horizontal):
 12–15 mm long
 It makes an angle of 30° with the horizontal plane.
 Projects as a rounded bulge into the middle ear, aditus and antrum.
 Its anterior end is ampullated and opens into the upper part of vestibule.
 The posterior nonampullated end opens into the lower part of vestibule
below the orifice of crus commune.
3. Posterior SCC:
18–22 mm long
Situated parallel and close to the posterior surface of petrous part of temporal
bone.
Its lower end is ampullated and opens into the lower part of vestibule.
Its upper limb joins the crus commune.
Crus commune: The nonampullated ends of posterior and superior canals join
and form a crus commune (4 mm length), which then opens into the medial part
of vestibule. So, the three SCCs open into the vestibule by five openings.
COCHLEA

 Formed by three parallel canals coiled in a


spiral around a central bony axis i.e.
Modiolus.
 There are 2.75 turns in the human cochlea.
 The cochlear tube
is 30 mm long.
It is 5 mm from
base to apex and
9 mm around its base
1. Modiolus:
 The base of modiolus,is directed
towards internal acoustic meatus.
The apex lies medial to tensor
tympani muscle.

 The axons of the central projections


of the auditory nerves that innervate
the sensory epithelia, and the
vessels of the cochlear blood
supply, the cochlear artery and
cochlear vein, run through the
length of the modiolus
2. Osseous spiral lamina:
A thin plate of bone winds spirally around
the modiolus.
Gives attachment to the basilar membrane
and divides the bony cochlear tube into three
compartments: Scala vestibuli, Scala tympani
and Scala media (membranous
cochlea/cochlear duct).
3. Scala media:-
 Lined by epithelia (part of the membranous labyrinth)
 Filled with endolymph. In cross sections of the cochlea,
 It is bounded by three ‘walls’ and appears approximately
triangular in shape
 Roof-reissners membrane
 Laterally-stria vascularis
Floor-organ of corti
4.Scala vestibuli:
Above Reissner’s membrane which acts as the barrier between
endolymph and perilymph .
This upper most channel is continuous with vestibule and
closed at oval window by the stapes foot plate.
Filled with perilymph.

5. Scala tympani:
Underneath the Basilar membrane
Thislowermost channel is closed by secondary tympanic
membrane of round window (RW).
6. Round window (fenestra cochlea):
At the basal end, the scala tympani terminates here
It is a flexible membrane formed of two epithelial sheets
sandwiching connective tissue, containing collagen and blood
vessels.
The apical surface of the outer epithelium is exposed to air in
the middle ear; that of the inner epithelium is bathed in
perilymph.

The RW is a surgical
landmark to locate the
scala tympani and hence
a convenient access for
the electrode insertion in
cochlear implantations.
7. Oval window:-
Opening over the vestibule, is covered by a membrane and is
filled with the footplate of the stapes.
8. Helicotrema:
The scala vestibuli and
scala tympani,communicate
with each other
at the apex of cochlea
through an opening called
helicotrema.
Filled with perilymph.
9. Aqueduct of cochlea:
The scala tympani is connected
with the subarachnoid space
through the aqueduct of cochlea
It is thought to regulate
perilymph and pressure
in bony labyrinth.
INNER EAR FLUIDS
ENDOLYMPH PERILYMPH
Found in Scala media Found in scala vestibuli, scala tympani

High potassium ; low sodium Low potassium ; high sodium


similar to intracellular fluid similar to extracellular fluid and csf
Maintained by the stria vascularis Communicates with cerebrospinal fluid
Communicates with the endolymphatic via cochlear aqueduct
duct and sac
Disorders include endolymphatic hydrops Likely conduit for bacterial meningitis
(Ménière disease) or wide vestibular into the inner ear
aqueduct
Source: Source:
1. Stria vascularis. 1. Filtrate of blood serum from the
2. Dark cells of utricle and ampullated capillaries of spiral ligament.
ends of semicircular ducts. 2. CSF reaching labyrinth via aqueduct of
Absorbed by endolymphatic sac cochlea.
This two-fluid system within the cochlea, creates an environment crucial to:-
• The mechanical displacement of the basilar membrane from the traveling
wave.
The cellular depolarization and subsequent synaptic activity in the hair cells.

Longitudinal flow(slow)
Radial flow(rapid) Stria vascularis
Planum
semilunatum,dark Scala media
vestibular cells &
Local absroption Ductus reunies

Both processes are Saccular duct


operational
Vestibular labyrinth

Vestibular aqueduct

Endolymphatic sac
Membranous Labyrinth
Membranous labyrinth consists of :-
1.Vestibular labyrinth
2.Semicircular canal
3.Cochlear labyrinth
MEMBRANOUS VESTIBULAR
LABYRINTH
 It consists of:-
1.Saccule
2.Utricle
3.The endolymphatic duct
and sac Types of endolymphatic
compartment epithelium:-
a)Five sensory epithelial
patches or sheets: the maculae
of the utricle and saccule, and
the three cristae, one in each
of the semicircular canals
(SCCs)
b)Ion transporting
c)Unspecialised/non sensory
Utricle:-
 Oblong and irregular, has anteriorly upward slope at an approximate angle
of 30°.
It lies in the posterior part of bony vestibule and receives the five openings
of the three semicircular ducts.
The utricle (4.33 mm2) is bigger than saccule (2.4 mm2) and lies superior to
saccule.
Connected with the saccule through utriculosaccular duct.
Its sensory epithelium, which is called macula, is concerned with linear
acceleration and deceleration and position of head wrt gravity.
Saccule:-
The saccule lies anterior to the utricle opposite the stapes
footplate in the bony vestibule.
Its sensory epithelium, macula responds to linear acceleration
and deceleration.
The saccule is connected to the cochlea through the thin
reunion duct.
Semicircular Ducts:-
The three semicircular ducts, which open in the utricle,
correspond exactly to the three bony canals.
The ampullated end contains a thickened ridge of
neuroepithelium, which is called crista ampullaris.
Endolymphatic Duct and Sac:-
Membranous utricular and
saccular ducts connect their
respective end organ with the
endolymphatic duct.
Endolymphatic duct travels
within the bony vestibular
aqueduct and terminates as the
endolymphatic sac along the
posterior face of the petrous part.
The endolymphatic duct and sac
are thought to be involved in the
reabsorption and regulation of
endolymph.
Situated inferior to donaldson’s
line
ORGAN OF CORTI
 The mature organ of Corti is a ridge of cells resting on the
basilar membrane and overlain by the tectorial membrane.
 The length of the coiled basilar membrane and attendant organ
of Corti is about 35mm long (range, 28–40mm)
 The widths & thickness of the
basilar membranedecreases systematically
from the base to the apex
of the cochlea.
 Medially, seated atop the osseous spiral lamina, is the spiral limbus, a thickened
band of periosteum that serves as the point of medial attachment for the
Reissner membrane and gives rise to the tectorial membrane

Tectorial membrane:-
•Lies over the inner and outer hair cells.
•It is a compliant gelatinous structure composed primarily of collagen II
fibers
•It serves as a mass load that moves similarly to a rubber band.
•Shearing force between hair cells and tectorial membrane produces
stimulus to hair cells.

 Lateral to the spiral limbus is the inner spiral sulcus, which is lined with the
border cells of Held
 One row of inner hair cells is present, the cell bodies of which are surrounded
by supporting cells called phalangeal cells
Spaces within the organ of Corti:-
a)Tunnel of Corti:- Between the inner and outer pillar cells
b)Space of Nuel:- Between the outer pillar cells and first outer hair cells
c)Outer tunnel:- Between the third outer hair cells and Hensen cells
d)Intercellular spaces:- Surrounding hair cells themselves
Reticular lamina:-
 The phalangeal cells, phalangeal processes of the Deiters cells,
and superior surfaces of the hair cells form the reticular
lamina,
 It is a tightly interwoven matrix that supports the apices of the
hair cells.
 It act as an barrier from endolymph, the fluid in the scala
media, which, owing to its ionic composition, is toxic to hair
cells.

Gap junctions in the organ of Corti contain two connexin


isoforms, Cx26 and Cx30.Mutations in the genes for these
connexins have been identified as causes of Hereditary
sensorineural hearing loss.
HAIR CELLS

 Theinner and outer hair cells function as


receptor cells that transduce mechanical
energy into an electrochemical signal to
stimulate the auditory nerve.
 The apical portion of all hair cells includes
a thickened region called the cuticular
plate, which, in conjunction with the
supporting cells, forms the reticular
lamina.
 Stereocilia:-
 Bundles of actin filaments
rooted in the cuticular plate of
each hair cell and projecting
through the reticular lamina
(into endolymph)
 Stiff hair-like structures that
deflect with mechanical
disturbances.
 Adjacent to this is a
noncuticular region that
contains a rudimentary
kinocilium.
 The hair bundle is composed of rows of stereocilia that increase in height in
one particular direction across the apical surface of the hair cell, and a
single kinocilium located behind the row of longest stereocilia.
 Kinocilium is absent from the hair bundles of mature hair cells in the
cochlea.

Proteins of Hair cells:-


Steriocilia:- Actin > Plastin-1 > Fascin,Fimbrin,Epsin
 Loss of Plastin 1 results in progressive thinning of stereocilia d/t which
there is progressive hearing loss and balance dysfunction.
 Mutations in the genes for myosins 6, 7a or 15- deafness and balance
disorders
 Myosin 15 is mutated- Stereocilia are greatly reduced in height
 Mutations in the myosin 7a gene- Usher syndrome type 1B.
 Mutations in the cdh23 gene - Usher syndrome type 1D, Hearing
impairment and vestibular disorders
INNER HAIR CELLS OUTER HAIR CELLS

Flask shaped Cylindrical


Wide at bottom, narrow at the top Narrow at bottom and top
High concentration of metabolic organelles e.g. High concentration of motile structures
Golgi bodies, mitochondria

Approximately 3500 .main function Approximately 12,000,more in number,more


mechanoelectrical transduction of sound. time to develop,more prone to noise induced
damage and ototoxicity. Amplification of sound.
Arranged in one row Arranged in three rows
Modiolar side of tunnel of Corti Strial side of the organ of Corti
Stereocilia are shallow and U shaped Stereocilia are V or W shaped
 (a) Inner hair cell. Flask-shaped
cell closely surrounded by
supporting cells. Terminals of
afferent synapse at the base of the
cell (arrowheads)
 (e)Outer hair cell. Efferent nerve
terminals (eff) around the basal
pole of the outer hair cell. The
lateral surfaces of the outer hair
cell bodies are surrounded by
extracellular space (the space of
Nuel)
 (h) Deiters’ cell: consists of a
main cell body region that at its
upper end encloses the base of an
outer hair cell (ohc
Vestibular Receptors

Peripheral vestibular receptors are of two types: cristae and


maculae.
1.Cristae: They lie in the ampullated ends of the three
semicircular ducts and respond to angular acceleration.
On a crest-like mound of connective tissue lie the sensory
epithelial hair cells, which are covered by cupula.
a. Cupula: The cilia of epithelial hair cells project into
cupula that consists of a gelatinous mass (complex
carbohydrates or glycoproteins and proteoglycans
arranged in filamentous network), which extends
from the surface of crista to the ceiling of the
ampulla.
b. Sensory epithelial hair cells: The sensory hair cells
are of two types: type 1 and type 2. From the upper
surface of each cell projects a kinocilium and multiple
stereocilia.
i.Type 1 cells: These cells are found only in birds and
mammals. They are flask-shaped and correspond to the
IHC of organ of Corti.
ii. Type 2 cells: They are cylindrical and have multiple
nerve terminals at the base. They resemble OHC of
organ of Corti.
2. Maculae:-
They lie in otolith organs (utricle and saccule).
Macula of the utricle is situated in
its floor in a horizontal plane in the
dilated superior portion of the utricle.
Macula of saccule is situated in
its medial wall in a vertical plane.

The macula utriculi (approximately 33,000 hair cells) are larger than saccular macula
(approximately 18,000 hair cells).
The striola, which is a narrow curved line in center, divides the macula into two areas.
They appreciate position of head in response to gravity and linear acceleration.
Saccule-vertical linear acceleration
Utricle-horizontal linear acceleration
 b. Otolithic membrane: The otoconial membrane consists of a gelatinous
mass, a subgelatinous space and the crystals of calcium carbonate called
otoconia or statoconia.

 The otoconia, which are multitude of small cylindrical and hexagonally


shaped bodies with pointed ends, consists of an organic protein matrix
together with crystallized calcium carbonate.
 The otoconia (3–19 μm long) lie on the top of the gelatinous mass. The
cilia of hair cells project into the gelatinous layer. The linear, gravitational
and head tilt movements result into the displacement of otolithic
membrane, which stimulate the hair cells lying in different planes.
BPPV

 Benign paroxysmal positional vertigo (BPPV)


is characterized by brief recurrent episodes of
vertigo triggered by changes in head position.
 BPPV is the most common peripheral etiology
of recurrent vertigo and is caused by abnormal
stimulation of the cupula by free-floating
otoliths (canalolithiasis) or otoliths that have
adhered to the cupula (cupulolithiasis) within
any of the three semicircular canals, most
commonly in posterior scc.
BLOOD SUPPLY TO

VESTIBULAR END ORGANS
The main blood supply to the vestibular end organs is through the
internal auditory (labyrinthine) artery, which arises most often (45%
of the time) from the anterior inferior cerebellar artery(AICA)

 Shortly after entering the inner ear, the labyrinthine artery divides
into two branches:-
 Anterior vestibular artery
 Common cochlear artery
 Posterior scc-posterior ampullary nerve branch of IV nerve
passing through foramen singular and is known as singular
nerve.
Internal Auditory Canal

Internal auditory canal (IAC) is about 1 cm


long and passes into petrous part of temporal
bone in a lateral direction.
It is lined by dura.
At its lateral end (fundus) IAC is closed by
a vertical cribriform plate of bone that
separates it from labyrinth.
A transverse crest divides
this plate into smaller upper
and larger lower parts.
Upper part is further
divided into anterior and
posterior quadrant by a
vertical crest called Bill’s
bar.
 Anterio superior:- Facial
nerve (7th )
 Anterio inferior:- Cochlear
nerve
 Posterio superior:- Superior
vestibular nerve
 Posterio inferior:- Inferior
vestibular nerve
 BILL’S BAR:-
 Named after Dr. William F. House “Father
of Neurotology,”
 Vertical ridge of bone
 Itis a useful landmark in translabyrinthine
surgery of the cerebellopontine angle as it
separates the superior vestibular nerve
from the anteriorly located facial nerve.
Anomalies of the inner ear

 Can be divided as follows:-


1. Cochlear malformations
2. Vestibular malformations
3. Semicircular canal malformations
4. Internal auditory canal (IAC)
malformations
1)Cochlear Malformations

a)Michel Deformity
Complete absence of bony and membranous
labyrinth.
b)Bing siebenmann dysplasia
Complete absence of only memebranous labyrinth
c)Scheibe dysplasia
Most common inner ear anomaly.
Cochleosaccular dysplasia.
c)Common Cavity Deformityan undifferentiated cystic cavity or
otocyst representing the cochlea and vestibule is identified
d)Cochlear HypoplasiaIn this variant, the malformation appears
more differentiated. The cochlea and vestibule are separate
from each other but their dimensions are smaller than normal.
 e)Incomplete Partition Type I (IP-I):-In the IP I, the cochlea lacks the
entire modiolus and cribiform area has a cystic appearance. A large cystic
vestibule is usually identified
f)Incomplete Partition Type II (IP-II) (Mondini Dysplasia)-
consists of 1.5 coils of the cochlea (instead of the normal two-
and-half coils), a flattened cochlea with the development of
the basal coil only, cystic dilatation of the common apical
chamber with absence of the interscalar septum between the
middle and apical coil, and a hypoplastic modiolus.
Due to Absence of osseous spiral lamina.
Associated with syndromic –waardenberg
syndrome,pendred,treacher Collins.
Thank You 

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