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    IMAGING APPROACH TO &

    INTERPRETATION OF FINDINGS INLESIONS IN THE CEREBELLOPONTINE ANGLE & INTERNAL

    AUDITORY CANALBYDR (MRS) YANJU AKINOLAMBChB, FWACS, FMCR, FICS

    ASSOCIATE PROFESSOR OFRADIOLOGY / HONORARYCONSULTANT LASUTH / LASUCOM

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    Objectives

    1. To review the process of imaging patientssuspected of harbouring CPA-IAC lesions.

    2. To review the anatomy of the CPA-IAC.

    3. To identify imaging findings of the commonlesions found in the CPA-IAC.

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    Introduction

    CPA tumours are the most common neoplasm inthe posterior fossa, constituting 5-10% ofintracranial tumors.

    Mostly benign and extra-axial

    Vestibular schwannomas (acoustic neuromas),

    85%Lipomas,

    vascular malformations,

    hemangiomas.

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    Introduction (Contd.)

    The most frequent non-acoustic CPA tumoursinclude,

    meningiomas

    epidermoids (primary cholesteatomas),

    facial or lower cranial nerve schwanomas.

    Primary malignancies or metastatic lesionsaccount for less than 2% of neoplasm in the CPA

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    DD of CPA LESIONS Vestibular schwannoma (85%)

    Meningiomas (3-13%)

    Epidermoids (2-6%) Facial and lower cranial nerve

    schwannomas (1-2%)

    Arachnoid cysts (1%)

    Rare tumors

    Lipomas Dermoid tumors

    Neuroepithelial cysts

    Trigeminal schwannoma

    Endolymphatic sac carcinomas

    Ependymoma

    choroid plexus papillomas,

    metastatic adenocarcinomas,

    metastatic neuroblastoma, ependymoma,

    lymphoma,

    cholesterol cyst,

    angioleiomyoma,

    venous hemangioma, cavernous angioma, and

    pontine glioma.

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    OTHER DD. Brainstem glioma

    Astrocytoma

    Medulloblastoma

    Choroid plexus papilloma Large or giant aneurysms of

    anterior inferior cerebellar artery(AICA), superior cerebellar artery(SCA), or the midbasilar artery

    Brainstem arteriovenousmalformation (AVM)

    Brainstem cavernousmalformation originating in thebrain stem (may reach a pialsurface along the CPA)

    Petroclival chondrosarcoma

    Clivus chordoma (usually arise inmidline but may extend laterallyinto the CPA)

    Osteosarcoma of the lateral skullbase

    Metastatic tumors (can reach CPAby hematogenous spread or directextension)

    Granulomatous inflammatorymasses

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    Anatomy of the CPA & IAC

    CPA is the anatomic space between thecerebellum and the pons.

    A common site for the growth of acousticneuromas or schwannomas.

    Neurologic syndrome / deficits occur due to the

    proximity of the CPA to specific cranial nerves

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    Anatomy of the CPA & IAC (Contd.) 8th cranial nerve enters the cranial

    cavity through the internal auditorymeatus, which is found in thetemporal bone

    also entering through this canal arethe facial nerve and thelabyrinthine artery(which providesthe blood supply to the inner ear)

    the close relationship between the VIIand VIII cranial nerves can explainsome of the complications of an

    ACOUSTIC NEUROMA 8th nerve fibres enter the

    ventrolateral surface of the medulla atthe cerebellopontine angle, anterior tothe inferior cerebellar peduncle (25 onthe brainstem) and lateral to the facialnerve

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    Anatomy of the CPA & IAC (Contd.)

    Lateral view of thebrainstem:

    1. medial geniculate body2. brachium of the inferiorcolliculus

    4. inferior colliculus

    25. Cerebellar Peduncle

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    Anatomy of the CPA & IAC (Contd.)

    The internal acoustic meatus (also internalauditory meatus, IAM) is a canal within the temporal

    bone that transmits nerves and vessels from within theposterior cranial fossa to the auditory apparatus.Contents of the IAM include:Facial nerve, V11Vestibulocochlear nerve, V111

    Vestibular ganglionLabyrinthine artery (usually a branch of the Anterior

    Inferior Cerebellar Artery or basilar artery) The opening of the IAM is located within the cranial

    cavity, near the posterior surface of the temporal bone.

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    Anatomy of the CPA & IAC (Contd.)

    IACNormal anatomy of the temporal bone.MPR/CT

    EAC =External auditory canal,

    Carotid canal = CC Jugular bulb =JB,

    Malleus = M,

    Facial nerve =FN,

    Cochlea = C,

    Semicircular canals = SCC,

    Internal auditory canal = IAC,

    Incus = I,

    Vestibule = V,

    Vestibular aqueduct = VA,

    Mastoid air cells = MAC.

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    Anatomy of the CPA & IAC (Contd.)

    1. Ear drum

    2. Malleus

    3. Incus4. Stapes

    5. Semicircular canals

    6. Auditory nerve

    7. Facial nerve

    8. Vestibular nerve9. Cochlea

    10. Eustacian tube

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    Anatomy of the CPA & IAC (Contd.)

    The margins of the opening are smooth androunded and the canal is short (1 cm), running

    laterally to the bone.

    At the distal end of the canal, it splits into threedistinct openings, one of which is the facial

    canal.

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    Anatomy of the CPA & IAC (Contd.)Nerves There are five nerves that run through the IAC:

    1. nervus intermedius (sensory component of CN V11)2. facial motor root (motor component of CN V11)3. cochlear nerve (component of CN V111)4. inferior vestibular nerve (component of CN V111)5. superior vestibular nerve (component of CN V111)

    Their position is most constant in the lateral portion ofthe meatus which is anatomically divided by thefalciform crescent.

    This horizontal ridge divides the canal into superiorand inferior portions.

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    Anatomy of the CPA & IAC (Contd.)

    Superior: facial nerve and superior vestibularnerve (SVN); the facial nerve is anterior to the SVN and

    is separated from it laterally byBills bar, a verticalridge of bone Inferior: cochlear nerve and inferior vestibular

    nerve (IVN); the cochlear nerve is situated anteriorlyGanglion

    In addition to the three nerves which enter it, it alsocontains the vestibular ganglion (Ganglion of Scarpa)From here three bundles emerge (superior and inferiordivision of the vestibular nerve and the nerve from theposterior semicircular canal

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    Imaging The IAC is housed by the petrous temporal bone which

    is a complex structure with many tiny bones (crura of

    stapes, vestibular acqueduct,

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    Imaging (Contd.) Plain radiography, now obsolete except for postop.

    assessment of cochlear implants Views include, Stenvers view (Oblique PA)

    Periorbital view best for IAM, if tomography isnot available

    CT shows intracranial lesions & its extensions

    Contrast enhancement required except if lesion isconfined to petrous pyramid

    Thin slices with wide window setting, 3000-4000HU Starts with lateral scout film, then axial, coronal &

    reformatted images

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    Imaging (Contd.)MRI

    Bone Negligible signal. Appears same as air

    Bone details better seen by CT

    Soft tissue details on MRI. Cranial Nerves well seen

    TIW intense signal with yellow bone marrow

    (high fat content especially in petrous apex) CSF & Labyrinthe fluid low signal intensity on

    T1W, Spin echo & high signal on T2W

    Flowing blood gives no signal blood vessels are

    normally black

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    Imaging (Contd.)

    Standard MRI studies using TI & T2 Protocol

    T1W - better spatial resolution

    T2W - poorer spatial resolution & take longer toacquire images.

    T2 gives higher signal from water containing

    structures (as in tumours) With Gadolinium DTPA, there is significant

    enhancement

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    Imaging (Contd.)

    Angiography

    Has a limited role now for vascular anomaliesand tumours. MRA& CTAhave replaced it

    Rarely used as diagnostic means now.

    More for pre operative assessment and for

    therapeutic embolization Aneurysm and vascular malformations require

    selective angio.

    Gradient echo sequences used

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    Imaging Studies Adults suspected to have CPA-IAC disease, usually

    complain ofsensorineural hearing loss

    (SNHL), can be imaged either withconventional enhanced T1 fat-saturated MR orhigh-resolution T2 MR imaging. CPA-IAC diseases that may be found include

    congenital lesions such as epidermoid cyst,arachnoid cyst and CPA-IAC lipoma. Benign tumors found in this location include

    acoustic schwannoma, meningioma, facial nerveschwannoma and IAC hemangioma.

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    Acoustic Neuroma (VestibularSchwanoma) Benign, primary intracranial tumours of the myelin

    forming cells of the vestibulocochlear nerve (CN VIII).

    involves the vestibular portion of the 8th

    cranial nerve, arises from Schwann cells, which are responsible for

    the myelin sheath in the peripheral nervous system. 3,000 cases are diagnosed each year in the US, with a

    prevalence of about 1 in 100,000 worldwide.

    510% of all intracranial neoplasm in adults. Incidence peaks in the fifth and sixth decades both sexes are affected equally.

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    Imaging Studies (Contd.)Site of acoustic N/Intracanalicular Schw.

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    Acoustic Neuroma (VestibularSchwanoma)(Contd.) Ipsilateral SNHL/deafness,

    disturbed sense of balance and altered gait,

    vertigo with associated nausea and vomiting, pressure in the ear, all of which can be attributed to the

    disruption of normal vestibulocochlear nerve function.

    Tinnitus in > 80%, (usually unilateral, high-pitched

    ringing, sometimes a machinery-like roaring or hissingsound, like a steam kettle).

    Large tumours that compress the adjacent brainstemmay affect other local cranial nerves.

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    Acoustic Neuroma (VestibularSchwanoma)(Contd.) 7th cranial nerves are rarely involved pre-

    operatively

    Involvement of the trigeminal nerve (CN Vloss of sensation in the involved side's face andmouth.

    The glossopharyngeal and vagus nerves are not

    usually involved, but their involvementaltered gag or swallowing reflexes. Larger tumors increased intracranial

    pressure, with its associated symptoms such asheadache, vomiting, and altered consciousness.

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    Acoustic Neuroma (VestibularSchwanoma)(Contd.)Pathogenesis May occur idiopathically or as part ofvon Recklinghausen

    neurofibromatosis, in which case the neuroma may take

    on one of two forms. InNeurofibromatosis type I, schwannoma may

    sporadically involve the 8th nerve, usually in adult life, butmay involve any other cranial nerve or the spinal root.

    Bilateral acoustic neuroma are rare in this type. InNeurofibromatosis type II, bilateral acoustic neuroma

    are the hallmark and typically present before the age of 21. Tend to involve the entire extent of the nerve and show a

    strong autosomal dominant inheritance. Incidence is about 5 to 10%.

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    Acoustic Neuroma (VestibularSchwanoma)(Contd.) Usually in the adult, presents as a solitary

    tumour, originating in the nerve.

    From the vestibular portion of the 8th nerve,just within the internal auditory canal.

    Usually extends into the posterior fossa to

    occupy the angle between the cerebellum andthe pons (cerebellopontine angle).

    may also compress the 5th, 7th, and less often,the 9th and 10th cranial nerves.

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    Acoustic Neuroma (VestibularSchwanoma)(Contd.) Later, may compress the pons and lateral medulla,

    causing obstruction of the CSF and increased

    intracranial pressure. Schwannomas can occur in relation to other cranial

    nerves or spinal nerve roots, resulting inradiculopathy or spinal cord compression.

    Trigeminal neuroma are the second most commonform of schwannomas involving cranial nerve.

    Schwannomas of other cranial nerve are very rare.

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    Acoustic Neuroma (VestibularSchwanoma)(Contd.) Contrast-enhanced CT will

    detect almost all acousticneuromas that > 2.0 cm indiameter and project furtherthan 1.5 cm into thecerebellopontine angle.

    smaller tumors may bedetected by MRI with

    gadolinium enhancement.

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    http://en.wikipedia.org/wiki/File:Vestibularis-Schwannom.jpg
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    Acoustic Neuroma (VestibularSchwanoma)(Contd.)VESTIBULAR SCHWANOMA VESTIBULAR SCHWANOMA

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    Acoustic Neuroma (VestibularSchwanoma)(Contd.)VESTIBULAR SCHWANOMA VESTIBULAR SCHWANOMA

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    http://en.wikipedia.org/wiki/File:Akustikusneurinom_Mrt.jpg
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    MeningiomaPathophysiology 2nd most common tumour of CPA

    Arise from the meninges, the membranous layerssurrounding the CNS.

    According to the American Brain Association, theyare the most common primary brain tumour,

    representing 1/3 of all such tumors. Arise from the arachnoid "cap" cells of thearachnoid villi in the meninges.

    Usuallybenign but may be locally aggresive;however, a small percentage are malignant

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    Pathophysiology

    Meningioma(Contd.) Many are asymptomatic, producing no symptoms

    throughout a person's life, and require no treatmentother than periodic observation.

    The 4 histologic variants, (1) meningothelial, (2)fibroblastic, (3) transitional, (4)angioblastic.

    Angioblastic variant is more aggressive.

    usually arise on the posterior surface of the petrousbone, near the porous acusticus, or along the lateralvenous sinus.

    Oval / hemisphericbroad dural-based, attachedto the tentorium/petrous bone dura

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    Pathophysiology

    Meningioma(Contd.) Displace but do not invade adjacent neural

    tissue.

    Meningiomas usually have a thin investingcapsule.

    They can invade bone without destruction by

    extension along haversian canals. 60% extend into the middle fossa.

    Intense contrast enhancement

    Usually does not extend into the IAC

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    EpidermoidPathophysiology Originate from epithelial restswithin the

    temporal bone or CPA. Usually grow very slowly with undulating margins. Patients often do not present until the 2nd to 4th

    decade of life. Tend to envelop rather than displace adjacent

    neural and vascular structures. Variable shapes, with irregular patterns. Usually develop multiple fingerlike projections

    between cranial nerves and brainstem fissures, sulciand blood vessels.

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    Pathophysiology - Epidermoid(Contd) Lamellated / onion skin appearance

    May extend through the tentorium into the middle

    fossa, forming a dumbbell shape. Imaging signal is similar to that of CSF

    May show a peripheral rim of calcification

    No enhancement with Gadolinium on MRI

    T1W Hypo / Iso intense

    T2W & Flair Iso / Hyperintense

    DWI Marked restriction bright

    appearance

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    Pathophysiology - Epidermoid(Contd) Apparent diffusion coefficient (ADC) values of

    epidermoid are significantly lower than that of

    CSF and higher than that of deep white matter Unusual imaging findings include,

    Hyperdensity on CT, T1W, T2W MRI which may

    be due to the high protein content of the cyst

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    Facial and lower cranial nerveschwannoma: Arise from Schwann cells anywhere along the

    course of the nerve.

    May be cystic / solid

    Cystic schwanomas are larger, with a thin rimenhancement of the wall

    Funnel shaped in axial MRI and club shaped oncoronal images

    Half of the solid tumours stronghomogenous enhancement

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    Arachnoid cysts CPA is the 2nd most common site for arachnoid

    cyst to occur, sylvian fissure being the 1st

    These are thin-walled sacs containing yellowcerebral spinal fluid (CSF) that most likelyoriginate as congenital developmentalanomalies.

    Smooth walled with CSF intensity on MRI

    Dont enhance with contrast

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    Arachnoid cysts(Contd.) Similar to epidermoid cysts in that they are well

    defined, homogenous low density, similar to CSF on

    CT, with no contrast enhancement and on MRI Hypointense on T1Wand hyperintense on T2W

    On FLAIR, similar intensity to CSF unlikeepidermoid which is strongly hyperintense

    Echo planar diffusion scanning, helpsdifferentiate : Arachnoid cyst is hypointensebut epidermoid is hyperintense

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    Presentation of CPA tumours

    Hearing loss - 95%

    Tinnitus - 80%

    Vertigo/unsteadiness - 50-75%

    Headache - 25%

    Facial hypesthesia - 35-50%

    Diplopia - 10% Symptoms can vary according to the size and

    location of the lesion.

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    Presentation of CPA tumours (Contd.)Meningioma Trigeminal or facial nerve symptoms are likely to occur

    earlier than hearing loss.

    Patients with larger tumours can present withobstructive hydrocephalus and/or symptoms ofbrainstem compression.

    Epidermoid These can become quite large without symptoms.

    Facial twitching (hemifacial spasm) and progressivefacial paralysis is more prominent than with othertumors in the CPA.

    Patients may present with cranial nerve or cerebellardysfunction that develops over a number of years.

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    Presentation of CPA tumours(Contd.)Facial nerve schwannoma Findings depend on the portion of the nerve affected Conductive hearing loss from middle ear involvement, parotid

    mass from extratemporal involvement, or sensorineural lossfrom internal auditory canal (IAC) or CPA involvement. CPA lesions do not cause facial weakness until tumor is very large

    (hemangiomas of the facial nerve usually cause symptoms at anearlier stage).

    Hemifacial spasm is relatively common.

    Lower cranial nerve schwannoma Patients may have weakness and hypesthesia of the palate, vocalcord, and shoulder (cranial nerves [CN] IX, X, XI) or hemiatrophyof the tongue (CN XII).

    Large tumours may cause deficits of all the lower cranial nerves

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    Imaging StudiesMeningioma Computed tomography (CT) scanning findings

    Hyperdense compared with the cerebellum on NCE images

    May demonstrate calcifications within or at the periphery of the tumor May be a broad attachment to the petrous ridge Less likely to show enlargement of the internal auditory canal (IAC) than

    vestibular schwannomas MRI findings

    Variable intensity on T2W images and either isointense or slightlyhypointense to brain on T1W images

    Can be heterogeneous as a result of calcifications and cystic foci withinthe tumor

    Dural tail sign, characterized by enhancement of the dura adjacent toan enhanced primary lesion, possible on contrast-enhanced MRI

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    CT / MRI FINDINGSLEFT IAC MASS WITH CALCIFIC. DURAL TAIL

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    Imaging Studies (Contd.)IAC MENINGIOMA WITH DURAL T. IAC MENINGIOMA

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    Imaging Studies (Contd.)Epidermoid CT scanning findings

    Hypointense to brain

    Do not enhance (enhancement suggests an associatedmalignancy or infection)

    Irregular margins with variable involvement of IAC MRI findings

    Heterogeneous and hypointense to brain on T1-weighted

    images Homogeneous and isointense or hyperintense to brain on

    T2-weighted images Nonenhancing (allows them to be differentiated from

    schwannomas, meningiomas, and chondromas, which havesimilar intensity)

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    EPIDERMOID CYSTNCE CT T1W MRI

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    EPIDERMOID CYST(Contd.)

    T1W MRI / T2W MRI T2W MRI / DWI

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    Imaging Studies (Contd.)Facial nerve schwanoma CT scanning findings

    Identical to vestibular schwannoma within the IACexcept for possible anterosuperior IAC erosion orerosion of facial nerve canal in labyrinthine segment

    May have enlargement of geniculate ganglion andfallopian canal (more common)

    MRI findings Isointense or mildly hypointense to brain on T1WI Mildly hyperintense to brain on T2WI Enhances with gadolinium contrast

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    Imaging Studies (Contd.)Lower cranial nerve schwannoma

    Enlargement of jugular foramen with cranial

    nerve IX, X, and XI tumours Enlargement of hypoglossal canal with cranial

    nerve XII tumours

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    Imaging Studies (Contd.)Arachnoid cyst

    CT scanning findings

    Typically smooth surface lesion isointense withCSF

    Non enhancing

    MRI findings

    Isointense or hypointense with brain on T1-weighted images

    Hyperintense to brain on T2-weighted images

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    Lipoma Lipomas develop from mesenchymatous cells In the IAC bone erosion, but is atypical for CPA lipomas CT scans show a typical low density mass that has attenuation

    characteristics similar to adipose tissue (-50 to -100HU). At MRI lipomas appear similar to fat, hyperintense, compared

    to brain, on T1-weighted images and hypointense on standardT2-weighted spin echo frequencies.

    With gadolinium no enhancement. DD, hemorrage or highly proteinaceous fluid. Fat suppression techniques in T1-weighted scans can be used

    to confirm the diagnosis, when it changes from the high signalintensity to an isointense aspect

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    Lipoma (Cond.)T1W T1W

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    Lipoma (Contd.)Coronal STIR MRI. Fat suppressed

    Contrast enhanced T1W + FatSuppression / T1W

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    Cerebral angiography Cerebral angiography is used when involvement of a

    large vessel is suspected or preoperative

    embolization is required. It is used to assess patency in vessels encased bytumor.

    Balloon test occlusion can aid in determininglikelihood of catastrophic stroke if internal carotidartery sacrifice is contemplated.

    Preoperative embolization can be performed invascular tumors to effectively decrease amount ofblood loss

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    Treatment Surgical intervention remains the most

    definitive means for complete tumour removal

    for most of these lesions. Radiation therapyhas gained popularity to

    arrest tumour growth with specific tumour typesand is also an option for patients who areunwilling or medically unable to undergosurgery

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    Contraindications

    Occasionally, complete resection is impossiblebecause of the intimate involvement of

    surrounding structures, which may causeunnecessary morbidity if complete excision isattempted, and planned partial resection isconsidered.

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    WORK UP OF PATIENTS WITH CPA/IACTUMOURSAudiovestibular assessment.

    Audiology and vestibular tests should be

    concurrently evaluated using air conductionandbone conduction threshold testing toassess for sensorineural versus conductionhearing loss.

    MRI: T1W & T2W with or without contast;DWI

    CT Scan: CE / NCE

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    MORE IMAGES Ac.Neur.

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    IAC MASS

    Glomus tympanicum (arrow)

    in a patient with right-sidedpulsatile tinnitus. Axial CTimage demonstrates lobulatedsoft-tissue attenuation in themiddle ear overlying thecochlear promontory.

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    Aberrant course of the ICA presenting with pulsatile tinnitus.A, CECT shows an abnormallateral course of the right ICA through the middle ear (white arrow), destruction of the

    overlying bony plate.

    B, AP from the MR angiogram shows decreased caliber and lateral deviation of the aberrantICA on the right reversed-7 sign (black arrow).

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    P di f h k ll A i l CT

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    Paget disease of the skull. Axial CT scandemonstrates diffuse expansion andsclerosis of the bones of the skull base.Note the sparing of the maxillofacial

    bones, which is a helpful indifferentiating it from fibrous dysplasia.

    Temporal bone Meningioma. Axial CT imagedemonstrates diffuse sclerosis of the temporal boneand soft-tissue attenuation (black arrows) in the leftmastoid and middle ear cavity.

    B, Axial enhanced MR image showing enhancementwithin the left middle ear (white arrow).

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    http://www.ajnr.org/content/31/2/211/F6.expansion.htmlhttp://www.ajnr.org/content/31/2/211/F5.expansion.html
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    THANK YOU&

    BEST OF LUCK

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