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MR IMAGING IN INNER EAR PATHOLOGY

ModeratorPROF & HOD . DR R.K. GOGOI

Presented by :: Sarbesh Tiwa

Introducti
on

The ear functions both as an organ of hearing and as2 an


organ of equilibrium

Embryology of ear
The ear is the first organ of special
senses to become differentiated in man,
inner ear reaches full adult size by
midterm
The external and middle ear develop
from 1st and 2nd brachial arch
Neural sound perceiving apparatus of
inner ear develops from ectodermal
otocyst
3

External Ear
PINNA (auricle) :
# sixth week of embryonic life,
# six tubercles appear around 1st
branchial cleft.
# They progressively coalesce to
form the auricle
External auditory meatus
# develops from the first
branchial cleft.
# 16th embryonic week,
# cells proliferate and form a
meatal plug.
# Recanalisation of this plug forms
the epithelial lining of the bony meatus.
# External ear canal is fully formed
by the 28th week of gestation
4

MIDDLE EAR
Tympanic membrane:
Develops from all the three
germinal layers. Outer epithelial
layer by ectoderm, Middle fibrous
layer by mesoderm & Inner
mucosal layer by endoderm.
Middle ear cavity :
# Develops from endoderm of
tubotympanic recess which arises
from 1st and partially from 2nd
pharyngeal pouches.
# Head of Malleus and short
process of incus are derived from
mesoderm of 1st arch
# Rest of malleus and incus with
stapes suprastructure develop
nd

Inner ear
Starts by 3rd week of fetal life and completed by 16th wks
The inner ear is derived from the ectoderm in the
region of the hindbrain.
A thickening of the ectoderm, the otic placode
becomes invaginated to form the auditory/otic vesicle.

OTIC PLACODE

OTIC PIT

OTIC VESICLE
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DIFFERENTIATION OF OTIC VESICLE INTO INNER


ELEMENTS
EAR
Each
vesicle divides into
-- the dorsal portion which forms theutricle, semicircular
canalsandendolymphatic duct, and
-- the ventral component, which gives rise to
thesacculeand the cochlear duct (scala media).

Mesoderm around otocyst soon forms a


cartilaginous otic capsule.
Part of the cartilaginous shell undergoes
vacuolization, and twoperilymphatic
spaces(scala vestibuli and scala tympani) are
formed.
Ossifies by 25 weeks

Small group of cells breaks away otic


capsule and along with cells of neural
creast origin forms the statoacoustic
ganglion.
The ganglion subsequently splits into
vestibular and cochlear nerves.

Anatomy of inner ear


It lies in the petrous part of the temporal bone
Inner ear consists of osseous labyrinth that
encloses membranous labyrinth.
Outer bony labyrinth
1. bony cochlea
2. vestibule
3. three bony semicircular canals
4. Vestibular and cochlear
aqueduct

10

Bony labyrinth

1. Vestibule :- Central rounded portion of labyrinth.

Medial wall has two recesses, a spherical recess


for saccule and an elliptical recess for utricle.
Continues antero-inferiorly with cochlea and
posteriorly with SCC & vestibular aqueduct.

11

2. Semicircular Canal:
They are three in number, the
lateral, posterior and
superior, and lie in planes at
right angles to one another.

3. Cochlea :The bony cochlea is a


coiled tube making 2.5 to
2.75 turns round a central
pyramid of bone called
the modiolus.
The bony cochlea
contains three
compartments:
(a) scala vestibuli,
(b) scala tympani,
(c) scala media or the

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4. Vestibular aqueduct
Tubular structure that arises
from vestibule and runs
along posterior inferior
aspect of petrous bone
Contains endolymphatic
duct and sac
Normally measures less
than 1.5mm in diameter or
approximates the size of
post. SCC which runs
anterior and parallel the
aqueduct.

13

Membranous labyrinth
Cochlear duct : Also called the scala media. It
is a blind coiled tube. It appears triangular on
cross-section and its three walls are formed by:
(a) the basilar
membrane, which
supports the organ of
corti,
(b) the Reissner's
membrane which
separates it from the
scala vestibuli,
(c) the stria vascularis,
which contains vascular
epithelium and is

14

Utricle and saccule


The utricle lies in the posterior part of bony
vestibule. It receives five openings of the three
semicircular ducts
The saccule anterior to the utricle and opposite the
stapes footplate
Endolymphatic duct and sac
Endolymphatic duct is
formed by the union of two
ducts, one each from the
saccule and the utricle.
It passes through the
vestibular aqueduct.
Its terminal part is dilated
to form endolymphatic sac
which lies between the two
layers of dura on the
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posterior surface of the

Internal Auditory Canal


A bony conduit that transmits VII & VIII cranial
nerves from pontomedullary junction to inner ear.

Divided by a bony
lamina (falciform
crest) into
A. Smaller superior
part
Superior vestibular
N.
Facial Nerve
B. Larger Inferior part
Inferior vestibular N.
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Blood supply of labyrinth


Arterial supply : Labyrinthine artery

which is a branch of anterior-inferior


cerebellar artery
Venous drainage : through three veins :
internal auditory vein
Inferior petrosal
vein of cochlear aqueduct sinus
vein of vestibular aqueduct
Transverse
sinus.
17

Cross sectional anatomy of inner


ear

Fig.1.-----Axial HRCT of
Inner Ear
White arrowhead : Modiolus with cochlea
White arrow : I A C

18

Cross sectional anatomy of


inner ear

Fig. 2 Axial heavily


T2 WI
Axial images shows basal turn of
cochlea and osseous spiral
lamina

Fig. 3 Axial heavily T2


WI
Middle and apical turns with the
modiolus (arrowhead) and the
spiral lamina (curved arrow)
dividing the cochlea into scala
vestibuli and scala tympani . The
nerves are seen in CP angle. 19

Cross sectional anatomy of


inner ear

Fig. 3 Sagittal T2 MR
Images
Showing the four nerves within IAC : The facial nerve (arrow head),
cochlear nerve (curved arrow) and superior and inferior vestibular
nerves (arrowhead)

20

When and why MRI


MRI essentially compliments CT because of its
excellent soft tissue contrast
MRI is directed toward imaging of
# Fluid containing spaces in temporal bone
# Vascular structure and their pathologies
# Adjacent brain parenchyma
# Evaluation of 7th and 8th nerve complex
Loosely, one can state that conductive hearing loss
is mainly evaluated by CT scan and sensorineural
hearing loss by MRI
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IMAGING PROTOCOL -- MRI


Inner ear Imaging

Tumors and
infection

1.5 or 3 Tesla MRI is preferred


Sedation used in most children
3D volumetric CISS in axial plane with coronal and sagittal
reformation and MIP reconstruction
Slice thickness of 0.4 0.7 mm
Oblique sagittal reformatted images in plane perpendicular to 7 th
and 8th nerve in IAC
Routine axial T2WI of brain to exclude CNS causes of
sensorineural hearing loss
3D MPRAGE may be added.
Precontrast brain with thin section through the CPA- IAC region
MRA/ MRV as required
Post contrast fat sat.

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3D CISS
Three dimensional (3D) constructive interference
in steady state (CISS) is a heavily T2 weighted
fully refocused gradient echo MR sequence.
Being heavily T2 weighted it is better suited for
imaging of structures surrounded by fluid like 7 th
8th nerve complex and membranous labyrinth.
3D sequence , so reconstruction in any plane
possible.
Other uses:1. Evaluation of cranial nerves
2. Diagnosis of NCC
3. Evaluation of CSF rhinorrhea
23
4. Evaluation of ventricular system etc.

The 3 D reconstruction of inner ear done with post-processing by maximum


intensity projection(MIP) and multi-planar reconstruction(MPR) by using 3DCISS sequence

24

CONGENITAL ANOMALIES
25

Congenital malformation of
inner
ear and Jackler et al
Cochlear abnormalities
are numerous
classified them on the basis of arrested development during
organogenesis

26

TIMELINE OF CONGENITAL
MALFORMATION OF INNER EAR

27

COMPLETE LABYRINTHINE APLASIA OR


MICHELE APLASIA
Most severe inner ear deformity
Etiology : Arrested development of otic placode
during 3rd gestational week
Extremely rare only 1% of inner ear
malformation.
Unilateral/ bilateral. Unilateral cases are associated
with contralateral inner ear dysplasia.
HRCT :# Complete absence of inner ear with
hypoplasia of petrous bone and narrow atretic IAC.
# Absence of round and oval window
# Flattening of medial wall of middle ear cavity
MRI : 8th cranial nerve not visualized on MR images28

Axial CT : Flat middle ear cavity


and hypoplastic petrous bone

Coronal CT : Atretic internal


auditory canal

Sagittal MRI : single nerve within


the IAC, suggesting absent 8th

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Common cavity
Defined by absence of normal differentiation
between the cochlea and vestibule
25% of cochlear malformation
Arrest during 4th arrest of gestation
Associated with poor
differentiation of
membranous labyrinth
as well resulting in
severe to profound
hearing loss

30

Confluence of cochlea and


vestibule in a cystic cavity with
no internal architecture

Common cavity

Absence of cochlear nerve

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Cochlear Aplasia

Cochlea fails to form


Due to arrested development in latter part of
5th week
The vestibule and semicircular canal are
often malformed but may be normal.
Imaging features :
1. Absence of cochlea
2. Dilatation of vestibule
3. Deformity of
semicircular canal
4. dense otic bone is
present where cochlea
would be

32

Axial CT images shows dilated


globose vestibule (arrow head) ,
dense sclerotic bone where cochlea
should be (curved arrow) , and a
stunted dilated posterior
semicircular canal (straight arrow).

Coronal CT images shows


malformed dilated lateral
semicircular canal (straight
arrow) with stunted superior
semicircular canal (curved arrow)

33

Cochlear Hypoplasia

Small rudimentary cochlear bud with normal


or malformed vestibule and semicircular canal
6th week of gestation -- 15 % of incidence
CT scan:
# Height around the cochlea is around (3-6)
mm as compared to normal height of 12-13
mm
# Abnormal Small IAC
# Though cochlea is visible it has
one turn or partial turn
Diagram shows : Small cochlear
bud and abnormally small and
deformed vestibule
34

Axial CT & MRI :- Small cochlear bud and abnormally small


and deformed vestibule

Stenotic IAC

35

Incomplete partition or dilatation defect


Due to development around 7th week
Includes the Mondinis defect (most common
malformation 50%)
Here basal turn of the cochlea is formed , however the
middle and apical turns are replaced by a common
cavity.
Imaging : (Triad of )
# Instead of 2.5 turns , only 1.5 turns are present
# enlargedvestibulewith normalsemicircular
canal
# enlarged vestibular aqueductcontaining a
dilatedendolymphatic sac.
MRI reveals : complete or partial absence of the
normal interscalar septum
36

Axial CT shows
absence of the
modiolus with a cystic
cochlear apex
(straight arrow) ,
dilatation of vestibular
aqueduct
(arrowhead) and
vestibule (curved
arrow)

Slightly lower level shows


normal basal turn

Coronal CT images shows the cystic cochlear

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Lateral semicircular canal malformation


Of anomalies of SCC, lateral semicircular canal
malformations is most frequent.
Hypoplasia of semicircular canal can lead to
compensatory enlargement of the vestibule
(lateral semicircular canal- vestibule dysplasia
syndrome)
Aplasia of SCC is associated with CHARGE
syndrome
Axial CT shows a stenotic IAC
(curved arrow) , mildly dilated
vestibule with stunted lateral
semicircular canal ( straight
arrow). The posterior semicircular
canal appears normal.

38

Large vestibular aqueduct


syndrome
Thelarge vestibular aqueduct
syndrome(LVAS)refers to the presence of
congenital sensorineural hearing loss with an
enlargedvestibular aqueduct
C/F : Sensorineural hearing loss starts in
childhood and is progressive
Associated with Pendred syndrome, vestibular
and cochlear anomalies
Imaging :
# Vestibular aqueduct of 1.5 mmis
considered the upper limit of normal.
# Usually less than posterior semicircular
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canal.

40

IAC and cochlear nerve anomalies


IAC normal diameter range from 2- 8 mm,
average 4mm
Diameter less than 2mm, described as Stenotic
Sagittal oblique images obtained in a plane
perpendicular to the long axis of IAC provides
best depiction of the four major nerves of IAC
Types of cochlear anomalies
Type I A Stenotic IAC with absent 8 th nerve
Type 2 A common vestibulocochlear nerve with
hypoplasia or aplasia of cochlear branch
2a associated with other inner ear anomaly
2b No associations.
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COMMON VASCULAR ANOMALIES

Aberrant (intratympanic) Internal


Carotid Artery
Due to abnormal regression of cervical ICA
during embryogenesis
Aberrant ICA has a sharp angled
posterolateral course where it tracks through
the middle ear over the cochlear
promontory.
Importance :
# Produces pulsatile tinnitus in few pt.
# May be confused with Glomus
tympanicum Paraganglioma
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CT:
1. Posterior carotid plate is
absent
2. Horizontal part of carotid
canal appears to merge with
lateral cochlear promontory
MRI :
1. Routine MR sequence are
not helpful.
2. MRA shows unusual
posterolateral course of ICA

44

Other arterial anomalies :


1. Persistant stapedial artery
2. Persistant trigeminal Artery
3. Anomalous artery in the stria vascularis
of the cochlea
Ectatic vascular loops of AICA/PICA or
tortous course of vertebral artery may
present with tinnitus due to compression of
VIII nerve complex.

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Venous variants
1. High riding Jugular bulb : Large jugular
bulb reaching above the internal auditory
canal with intact sigmoid plate
2. Dehiscent Jugular Bulb : The sigmoid plate
is deficient, the bulb protrudes into the
middle ear cavity. It is a common cause of
aretro-tympanic vascular mass.
3. Jugular bulb diverticulum

47

Dehiscent jugular bulb

High riding jugular bulb

48

INFLAMMATORY
CONDITIONS

LABYRINTHITIS
Inflammation of
membranous labyrinth.
Viruses are the most
common etiologic agents,
but can be bacterial or
autoimmune .
Types:
1. Tympanic labyrinthitis : Infection spreads from
middle ear via oval or round window or labyrinthine
fistula
2. Meningogenic : Infection spreads along CSF spaces
via IAC or cochlear aqueduct. Usually bilateral.
3. Hematogenic labyrinthitis : spread of infection by
blood-stream. Virus e.g, measles and mumps,
syphilis etc
50
4. Post traumatic labyrinthitis.

Imaging features:
CT : # Usually normal in acute stage
# Ossification of membranous labyrinth in late
chronic phase
MRI : contrast enhanced MR is the method of choice
# T1- CEMR shows moderate to intense
enhancement within normal fluid filled structure
of inner ear
# Usually viral conditions causes subtle
enhancement and bacterial causes intense
enhancement.
Complication : Labyrinthitis ossificans is a Sequela
of chronic labyrinthitis, usually Pyogenic in origin.
51

Axial MRI in patient with


suppurative
labyrinthitis.Enhancement of
internal auditory canal,
cochlea, and vestibule.

52

Labyrinthitis ossificans

Labyrinthitis ossificans (LO) is the pathologic


formation of new bone within the lumen of the otic
capsule.
Etiology :1. Sequela of inflammation of the inner ear, e.g
bacterial meningitisor purulent labyrinthitis
2. Vascular obstruction of the labyrinthine artery
3. Autoimmune labyrinthitis etc.
Imaging :
CT scan :- Osseous deposition within the
membranous labyrinth
MR Imaging :- Loss of the normal high signal on T2weighted images from displacement of the
endolymphatic fluid is suggestive of this diagnosis.

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CHOLESTEATOMA WITH
COMPLICATION
MRI features of cholesteatoma ::-Hypointense on T1WI & Hyperintense on T2 WI
No enhancement or faint peripheral rim
enhancement
Delayed Contrast scan (after 45min)
continued enhancement of inflammatory or
granulation tissue and not in cholesteatoma.
DWI Cholesteatoma shows restricted
diffusion and are hyperintense on b= 1000/m2.

55

CHOLESTEATOMA WITH
COMPLICATION
A . Labyrinthine fistula -- Most frequent
complication with middle ear cholesteatoma
(prevalence of 5% 10%).
C/F : Episodic vertigo, sensorineural hearing loss,
tinnitus
CT Findings :1. Dehiscent lateral semicircular canal support
the diagnosis
2. Uncommonly , dehiscence of cochlear
promontory or fistula in oval window.
MRI Findings :- a labyrinthine fistula causing
labyrinthitis, shows enhancement of the
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Other complications B. Perineural extension of cholesteatoma


along facial nerve.
C. Erosion of the sigmoid sinus plate and
consecutive thrombosis, tympanic tegmen
erosion and subsequent intracranial
invasion, recurrent\bacterial meningitis, and
intracranial abscess are rare complications,
which, nevertheless, require an urgent
CT/MR imaging examination
58

Petrous apex granuloma


Cholesterol granuloma is an inflammatory granulation
appearing in response to the deposits of cholesterol
crystals
Etiology: Middle ear disorderscausing mucosal edema
and deposition of cholesterol crystal
Site : Middle ear cavity followed by mastoid process and
petrous apex
C/F : Hearing loss, vertigo, headaches, tinnitus
Any focal neurological deficit, especially of cranial
nerves VVIII
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Petrous apicitis
Petrous apicitis is infection with involvement of
bone at the very apex of thepetrous temporal
bone.
Pathology : Osteitis developing from infected
and obstructed air cells in a pneumatised
petrous apex
C/F: Presents with Gradenigos syndrome

1. petrous apicitis, with


2. 6th nervepalsy, and
3. Retro-orbital pain, or pain in the cutaneous
distribution of thetrigeminal nerve, due to
extension of inflammation intoMeckel's cave.61

CT scan :
1. Erosive lysis with ill-defined
irregular edges of petrous
apex
2. Peripheral enhancement of
petrous apex with dural
enhancement and thickening
MRI:
1. Fluid signal intensity in
petrous apex often with
peripheral enhancement
2. More sensitive in detecting
dural thickening and
enhancement as well
asleptomeningitis,
cerebritisandcerebral abscess
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FACIAL NERVE LESIONS

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65

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BELLS PALSY
Bell's palsyis characterized by rapid onsetlower motor
neuron facial nerveparalysis, often with resolution in 6 8 weeks.
Etiology :
1. Idiopathic
2. Reactivation of Herpes Simplex Virus infection
in geniculate ganglion.
Pathogenesis : Secondary to swelling and edema of the
7th nerve within the facial nerve canal
Indication for imaging : MRI not done routinely . Indicated
if :# Decompressive surgery is being planned
# Atypical: No recovery in 6 wks, recurrent
palsy, multiple cranial nerve involvement.
67

On MRI imaging enhancement of the facial nerve


within the tympanic portion of the facial nerve canal
supports the diagnosis.
Reported enhancement rates vary from 57% to
100%.
Typically long segments of the facial nerve enhance
in a uniformly linear fashion

68

Facial Schwannomas
Facial nerve Schwannoma are uncommon tumors
arising from the Schwann cell sheath
Site : Geniculate ganglion, followed by labyrinthine and
tympanic segment.
Pathology : Originate from surface of the nerve, and
splay the nerve fibres over their eccentric growth
C/F : 1. Persistent and gradually facial paresis.
2. Conductive hearing if tympanic segment
involved causing ossicular compression
3. In CP angle or IAC : Presents with sensorineural
deafness with facial paresis being rare in these cases.
4. Other like, tinnitus, hemifacial spasm, and
otalgia
69

Imaging features
HRCT :
Enhancing soft tissue density lesion along facial nerve
Intracanalicular or CP angle tumor can cause bony
erosion of anterosuperior portion of IAC
MRI:
T1: Iso- to hypo intenserelative to gray matter
T2: Hyperintense ;large lesion may show
heterogeneous signal
T1 C+ (GAD): Homogeneous enhancement with larger
lesionsshowing cystic degeneration as focal
intramurallow signal intensity
70

CT SCAN : focal enlargement of


the labyrinthine segment of the
facial nerve
MRI: homogeneously enhancing
mass filling the internal auditory
canal with extension into the CP
angle and labyrinthine segment
Diagnosis : Facial Nerve

71

The axial T1-weighted post-contrast MR image (left) shows


homogeneous enhancement of the mass (between
arrows).
The bone algorithm CT (right)at the same level shows focal
enlargement of the descending segment with extension
toward the external auditory canal.
Diagnosis : Schwannoma of the mastoid segment of facial

72

FACIAL NERVE
HEMANGIOMA
Rare tumor of vascular origin (0.7% of all intratemporal tumors)
This along with other vascular malformations are
termed as Intra-temporal Benign Vascular Lesions

Age : 3rd to 6th decade without sex predilection


Site : Geniculate fossa followed by IAC
C/F: Facial nerve paralysis progressing over weeks.
Sensorineural hearing loss and pulsatile tinnitus
may occur if there is erosion of otic capsule.
73

IMAGING FINDINGS
MRI :
# Intratemporal hemangiomas characteristically have
variable signal intensity on T1-weighted images
increased signal intensity on T2-weighted images
avid contrast enhancement.
# Low-signal-intensity foci may be seen on T1- and
T2-weighted images, corresponding to the ossific
matrix of the lesion
CT Scan : Enables exquisite visualization of associated
bone changes
Tumor causes erosion which are irregular with
indistinct margins giving a Honeycomb pattern of
eroded bone.
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75

Tumors in association with


Inner ear

Anatomy: Cerebellopontine
angle
CRANIAL NERVE -

V , VII & VIII


ARTERIES :
SUP. CEREBELLAR
A.
AICA
VEINS :
TRIBUTARIES OF
SUP PETROSAL V.
Boundaries :pons & Cerebellum

- Anterolateral surface of
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Imaging signs of extra-axial CPA


cistern masses
1. Enlarged ipsilateral CPA cistern
2. CSF/ Vascular Cleft between mass
and cerebellum
3. Displaced gray-white interface
around mass
4. Brainstem rotated
5. Fourth ventricle compressed.

78

Cerebellopontine angle cistern


masses
Common tumor

Acoustic Schwannoma
[75%]
Meningioma [10%]
Epidermoid [5%]
Non acoustic
Schwannoma [4%]
Aneurysm
Metastasis
Paraganglioma
Ependymoma
Choroid plexus
papilloma

Uncommon tumor

Arachnoid cyst
Lipoma
Dermoid
Brain stem glioma
Osteocartilagenous
tumor

79

Vestibular Schwannoma
Benign tumor arising from Schwann cells
that wrap vestibulocochlear nerve
70- 80 % of CPA lesions
Age 5th -6th decade.
B/L acoustic Schwannoma pathognomonic for NF-2.
Origin : Most from Inferior Vestibular Nerve, at glialschwann cell interface
Morphology :
# Entirely intracanalicular
# Intracanalicular with cisternal
component Ice-cream cone appearance.
# Rarely purely intracisternal.
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Pathology : Compact Antoni Type A


tissue or loose textured , often
cystic Antoni type B tissue
C/F : SNHL or tinnitus,
Hydrocephalus, CN palsies (If large)
Imaging findings : CT SCAN:# NECT :- Extra axial hypodense to
isodense mass with its base on post
aspect of petrous temp in region of
IAM.
# CECT :- Marked enhancement,
large lesion m/b heterogeneous.
# Erosion & widening of Internal
Acoustic canal.
# Small lesions c/b missed d/t beam
hardening artifact.

81

MR IMAGING FEATURES
T1WI: 2/3rd are hypointense and 1/3 rd are isointense.
may contain hypo intense cystic areas
T2WI :
# Heterogeneously hyperintense
# Small leson : "Filling defect" in high signal CSF of CPA-lAC
cistern
# may have associated peri-tumoural Arachnoid cysts
T1 C+ (Gd) :
# contrast enhancement is vivid
# Heterogeneous in larger tumors
# Occasionally, may show extension into the cochlea and dural
tail of enhancement.

82

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MENINGIOMA
Extra-axial neoplastic lesions arising from
Arachnoid cap cells.
2nd most common CPA tumor (10%)
Site : arises from the meninges covering posterior petrous bone.
Female > Male (2-3:1) , peak age = 60yrs.
C/F : Small Meningioma -entirely asymptomatic
Large tumors headache, paresis or neurological deficit.
Morphology :
# "Mushroom cap" (hemispherical) with broad base towards
posterior petrous wall (75%)
# Plaque-like : +/- bone invasion with hyperostosis (20%)
# Ovoid mass : mimicking Acoustic Schwannoma
84

Imaging features

NECT :
# Frequently hyperdense with focal areas of
calcification
# Bony hyperostosis of petrous bone
CECT : Presence of broad dural base with dural tail
and intense enhancement is typical.
MRI :
# Isointense to brain parenchyma in T1 & T2WI
# Blooming s/o calcification in GRE
# Dural tail with other features of extra-axial lesion
# May rarely extends into IAC and presents with
diagnostic dilemma.
ANGIOGRAPHY : Homogenous blush which lasts till
late venous phase (Mother In Law sign)

85

86

Lipoma of internal auditory


canal
Rare congenital lesion often

associated with CP angle lipoma.


CT Scan: Fat attenuating non
enhancing lesion
Presents with unilateral sensorineural
hearing loss
MRI : Non enhancing lesion which is
hyperintense on T1WI & T2WI with
suppression of SI on fat saturated
images.

87

GLOMUS TUMOR (PARAGANGLIOMA)


Slow growing, locally aggressive
vascular tumor arising from
chemoreceptor cells
Arising from the 9th & 10th nerve,
most common tumor of middle
ear
2nd most common tumor of
temporal bone
Unique names associated with
location: Jugular bulb Glomus
jugulare
Middle ear
-Glomus

88

Clinical feature:
Pulsating tinnitus with conductive hearing loss
Invasion into cochlea leads to sensorineural hearing loss
Otoscopy : Tympanic membrane appears blue
Glomus tympanicum : Isolated to
middle ear cavity
Soft tissue density protruding from
cochlear promontory .
Homogenous enhancement on
post contrast scan.
Glomus Jugulare :
Confined to jugular fossa and
large at presentation
Invades the hypo-tympanum and
infralabryrinth compartment
Tumor can follow the Jugular
venous system upto lower cervical
jugular vein.

89

MRI features :
T1WI Low signal intensity
T2WI High signal Intensity
T1C+ -- Marked intense enhancement
Salt pepper appearance (T1 + T2) : Salt
represents areas of hemorrhages and pepper
represents areas of flow void.

90

Perineural Spread of tumors


Common with head & neck cancer.
Nasopharyngeal CA followed by adenoid cystic
carcinoma and lymphoma.
Trigeminal and facial nerve are the commonest
nerves involvement.
Features of Perineural spread :1. Enlargement and enhancement along the
course of nerve
2. Obliteration of the fat surrounding neural
foramina
3. Denervated muscles becomes atrophic with time
and replaced with fat.
4. Tumor in lateral aspect of pons should raise a
suspicion.
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92

LESION OF ENDOLYMPHATIC SAC &


DUCT

Endolymphatic hydrops ( Menieres )


Refers to increased hydraulic pressure within
inner ear endolymphatic system.
Etiology:
# Idiopathic (Menieres disease)
# Association with autoimmune disease ,
hormonal and metabolic condition noted
(Menieres syndrome)
C/F : 1. Fluctuating hearing loss
2. Episodic vertigo
3. Tinnitus
4. Aural fullness
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Role of MRI in Menieres disease


Bilateral intratympanic GBCA is being used for semi
quantitative evaluation of Menieres.
1- 1.5 ml of diluted gadolinium is injected into middle
ear through a small myringotomy and evaluated after
24 hours.
3D FLAIR is used to evaluate inner ear
3Tesla is better.
The gadolinium successfully penetrated the round
window membrane, entering the perilymphatic space
and delineating the contrast-enhanced perilymphatic
and contrast-negative endolymphatic spaces of the
inner ear
If the non-enhancing endolymphatic area exceed the
95
perilymphatic area, it is considered significant.

Normal : Inner ear ; No hydrops

3D FLAIR : Significant
enlargement [3350%] of the
endolymphatic compartment
in the cochlea;
in the vestibule and
semicircular
canal endolymphatic hydrops96
[>50%] has displaced almost

Endolymphatic sac tumor


Papillary adenomatous tumors
originating form endolymphatic sac.
Described by Heffner in 1989.
Occurs sporadically or with VHL
disease
Age : Presents early if associated
with VHL
C/F : Sensorineural hearing loss +
Tinnitus +Vertigo

Imaging : CECT
a. Heterogeneous lesion with motheaten retrolabyrinth petrous bone
b. Intratumoral spiculated bone seen
c. Intense enhancement in seen
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MRI Features :
T1 /T2WI : Mixed signal intensity lesion
where focal high signal intensity d/t
subacute hemorrhage and low signal
intensity d/t calcification or hemosiderin.
Blood filled cysts and protein cyst, both
appearing hyperintense on T1/T2WI
suggests the diagnosis
Masses larger than 2 cm shows flow voids
T1C+ : Heterogeneous enhancement

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ISSUES WITH POST COCHLEAR


IMPLANT CASES
Cochlear implants are not safe at 1.5T
MR compatible CI are now available
External component should be removed in
all cases
Role of MRI is in preimplant evaluation to
exclude cochlear aplasia which is
contraindication for surgery. Absent
Cochlear nerve is relative contraindication.
CT scan is better for post-operative
evaluation of these patients.
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Conclusion
MR provides accurate anatomical delineation
of complex soft tissue of inner ear
3D reconstruction improves preimplant
evaluation
Detailed delineation of 7th & 8th nerve
complex in temporal bone as well as
membranous labyrinth
Depiction of tumor size and extension into
CP angle determines the approach to surgical
removal.
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References
1. Diagnostic Radiology- Neuroradiology AIIMS
MAMC- PGI Course series . 3rd edition.
2. CT and MRI of whole body John R Haaga 5 th edition
3. Joshi VM,Navlekar SK et.al -Ct and MRI imaging of
the inner ear and brain in children with
sensorineural hearing loss. Radiographics.2012
May-Jun;32(3):683-98
4. Jeremy Hornibrook, Mark Coates, Tony Goh, Philip
Bird et.al MRI imaging of the inner ear for Menieres
disease. Journal of the New Zealand Medical
Association. 27 August 2010, Vol 123 No 1321
5.

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THANK

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