ACOUSTIC NEUROMA

DR.AYESHA FAYYAZ
TR ENT, SZH
11-1-2018
 DEFINITION

• ALSO CALLED VESTIBULAR SCHWANNOMA

• BENIGN, SLOW GROWING, NERVE SHEATH
TUMOR OF SUPERIOR AND
INFERIOR VESTIBULAR NERVE
 INTRODUCTION

• 78% OF ALL CPA TUMORS

• ARISES IN MEDIAL PART OF THE IAC OR THE LATERAL PART OF THE CPA
• AGE OF PRESENTATION:
SPORADIC: 40-60 YR.
NON-SPORADIC < 20-30 YR.
• MEAN GROWTH RATE : 1.2 MM / YR.
• RARELY MALIGNANT
 ANATOMY OF CP ANGLE
• POTENTIAL CSF SPACE IN POSTERIOR CRANIAL FOSSA
• ROUGHLY TRIANGULAR IN SHAPE
• ANTERIORLY: TEMPORAL BONE
• POSTERIORLY: CEREBELLUM
• MEDIALLY: CISTERNS OF THE PONS &
MEDULLA AND OLIVE
• SUPERIORLY: INFERIOR BORDER OF PONS
& CEREBRAL PEDUNCLE
 STRUCTURES CROSSING CPA

• CN V ( SUPERIORLY )
• CN IX,X,XI ( INFERIORLY )
• CENTRE: CN VII AND VIII WITH ARACHNOID SHEATH TO IAC
• AICA
 ETIOLGY

Defective chromosome Mutated tumor

22q12 suppressor “MERLIN”

Somatic mutation in U/L VS

both copies of MERLIN
 GROSS ANATOMY

• SMOOTH SURFACE
• YELLOW TO GRAY COLOR
• USUALLY SOLID WITH OCCASIONAL CYSTIC COMPONENT
 HISTOLOGY
ANTONI A: ANTONI B:

• CLOSELY PACKED CELLS • LOOSE CELLULAR AGGREGATION OF

VACUOLATED PLEOMORPHIC CELLS
• SMALL SPINDLE-SHAPED AND DENSELY
STAINED NUCLEI
• WHORLED APPEARANCE
• VEROCAY BODY
 CLASSIFICATION
• ACCORDING TO SIZE

Intrameatal tumour Extrameatal size mm

Grade 1 Small 1-10

Grade 2 Medium 11-20

Grade 3 Moderately Large 21-30

Grade 4 Large 31-40

Grade 5 Giant >40

 SIGNS & SYMPTOMS

INTRACANALICULAR:
◦ HEARING LOSS (UL PROGRESSIVE ), TINNITUS, VERTIGO
◦ LOSS OF SPEECH DISCRIMINATION OUT OF PROPORTION TO HL
CISTERNAL:
◦ WORSENED HEARING AND DISEQUILIBRIUM
 SIGNS & SYMPTOMS

COMPRESSIVE:
◦ OCCASIONAL OCCIPITAL HEADACHE
◦ CN V: MIDFACE, CORNEAL HYPOESTHESIA
◦ CN VII : HITZELBERGER’S SIGN, LOSS OF TASTE AND REDUCED LACRIMATION ON
SCHIRMER’S TEST , FACIAL WEAKNESS ( LATE)
◦ CN II , IV , VI : DEC. VISUAL ACUITY AND DIPLOPIA
 SIGNS & SYMPTOMS

HYDROCEPHALIC:
◦ FOURTH VENTRICLE COMPRESSED AND OBSTRUCTED
◦ HEADACHE, VISUAL CHANGES, ALTERED MENTAL STATUS
◦ NAUSEA AND VOMITING
◦ O/E : RAISED ICP AND PAPILLEDEMA.

COMPRESSION OF CN IX & X
◦ DYSPHAGIA , ASPIRATION AND HOARSENESS
◦ POOR GAG REFLEX AND VC PARALYSIS.
 SIGNS & SYMPTOMS

CEREBELLAR INVOLVEMENT( LATE )

◦ INCOORDINATION , WIDELY BASED GAIT , TENDENCY TO FALL
TOWARDS AFFECTED SIDE

BRAINSTEM INVOLVEMENT:
• ATAXIA, WEAKNESS AND NUMBNESS OF ARMS AND LEGS
WITH EXAGGERATED TENDON REFLEXES.
 INVESTIGATIONS

MRI WITH GADOLINIUM CONTRAST

• HYPOINTENSE GLOBULAR MASS IN IAC ON T1
• ENHANCEMENT WITH GADOLINIUM
• ISO/HYPOINTENSE ON T2

CT SCAN WITH IODINE CONTRAST

• OVOID MASS IN IAC WITH NONHOMOGENEOUS ENHANCEMENT.
SPECIAL INVESTIGATIONS
 AUDIOLOGY

PURETONE AUDIOMETRY:
• ASYMMETRIC, DOWNSLOPING, HIGH-FREQUENCY SNHL

WORD RECOGNITION SCORE (WRS):

• ROLLOVER PHENOMENON

ACOUSTIC REFLEX THRESHOLDS

• ACOUSTIC REFLEX DECAY
 VESTIBULAR TESTING

ELECTRONYSTAGMOGRAPHY (ENG)
• REDUCED CALORIC RESPONSE IN THE PROBLEMATIC EAR
 AUDITORY BRAINSTEM RESPONSE

• MEASURES YOUR BRAIN'S RESPONSE TO SOUND

• FULLY OR PARTIALLY ABSENT
• DELAY IN THE LATENCY OF WAVE V IN THE AFFECTED EAR
• INTER AURAL DELAY OF WAVE V LATENCY > 0.2 MS
• SENSITIVITY >90%
• SPECIFICITY = 90%
 DIFFERENTIAL DIAGNOSIS
D/D S/S INVESTIGATIONS

MENINGIOMA LESS PROMINENT HL NO EXPANSION OF IAC

MAY HAVE DURAL TAIL (CT/MRI)

EPIDERMOID LESS PROMINENT HL NON-ENHANCHING T1 & T2

IMAGES

FACIAL NERVE SHWANNOMA EARLY PROMINENT FACIAL ENHANCEMENT IN FACIAL CANAL

WEAKNESS

TRIGEMINAL SCHWANNOMA LESS PROMINENT HL DUMBELL SHAPED MASS OVER

MORE PROMINENT FACIAL PETROUS APEX
NUMBNESS
 TREATMENT

• SURGICAL REMOVAL
• OBSERVATION
• RADIOTHERAPY
 SURGICAL REMOVAL

• TRANS LABYRINTHINE CRANIOTOMY

• RETRO SIGMOIDAL CRANIOTOMY
• MIDDLE FOSSA CRANIOTOMY
 TRANSLABYRINTHINE APPROACH

• PRIMARY APPROACH
• HEARING ABLATING METHOD
• PT SELECTION:
POOR HEARING ( HL > 50DB)
UNRESECTABLE WITH HEARING PRESERVING METHODS
 STEPS

Identification Skeletonization sigmoid sinus Identification

post-auricular of incus, of middle and decompressed of facial nerve Tumor removal
incision Mastoidectomy tegmen, post fossa with a medial to bill’s from lateral to
sigmoid sinus & dura diamond burr bar medial side
facial nerve
 ADVANTAGES DISADVANTAGES

• ANY SIZE TUMOR CAN BE RESECTED • HEARING LOSS

• MINIMUM BRAIN RETRACTION
• FACIAL NERVE PRESERVATION: 97%
 RETROSIGMOIDAL APPROACH

• HEARING PRESERVING METHOD

• GOOD VIEW OF CPA
• PT SELECTION:
GOOD HEARING LEVELS
TUMOR LIMITED TO MEDIAL IAC
 STEPS

Posterior
Retromastoid Mastoid & dura is Cerebellum wall of IAC Closure
curvilinear retromastoid Craniotomy opened visualized & CPA removed for done in
skin incision bone performed along the retracted identified exposure of layers
exposed sigmoid sinus canalicular
portion
 ADVANTAGES DISADVANTAGES

• HEARING PRESERVATION • PERSISTENT POST-OPERATIVE HEADACHE

• INABILITY TO EXPOSE FACIAL NERVE
• PERSISTENT CSF LEAK
 MIDDLE FOSSA APPROACH

• HEARING PRESERVING APPROACH

• INTRACANALICULAR TUMORS WITH <1.5 CM CISTERNAL COMPONENT
• ENTIRE IAC IS ACCESSIBLE WITHOUT VIOLATING THE INNER EAR
• POOR SURGICAL POSITION OF THE FACIAL NERVE
• TRANSIENT SPEECH AND MEMORY DISTURBANCES AND AUDITORY HALLUCINATIONS
 STEPS

inverted Extradura IAC

U-shaped Squamous Cranioto l identified Dura Closure
incision temporal my over elevation and exposed Tumor done in
over the bone zygomati of skeletoniz posteriorl removed layers
ear exposed c root temporal ed y in IAC
lobe
 OBSERVATION

• LIFE EXPECTANCY < DURATION REQUIRED FOR VS GROWTH

• RADIOLOGICAL FOLLOW-UP :
AFTER 6 MONTHS
THEN YEARLY
• TREATMENT: 2-3MM GROWTH / 1ST YEAR
 STEREOTACTIC RADIATION

• PRECISELY TARGETED HIGH DOSE RADIOTHERAPY

• PREVENTS FURTHER GROWTH
• PRESERVE HEARING & FACIAL NERVE
• COMPLICATION: HYDROCEPHALUS
• PT SELECTION:
• SHORT LIFE EXPECTANCY
• HIGH SURGICAL RISK
 OPERATIVE COMPLICATIONS

• VASCULAR INJURY
• AIR EMBOLISMS
• PARENCHYMAL BRAIN INJURY
• CRANIAL NERVE INJURY
THANKYOU