By

DR Mohammed
Riyas
INTRODUCTION

An acoustic neuroma (also called vestibular schwannoma)
is a benign tumor arising from abnormally proliferative
shwann cells , which envelope the lateral portion of the
vestibular nerve in the internal auditory meatus
Little things about
CP angle
CP angle tumors

Represents 10 % of all intracranial tumors.
Fatal without treatment.
VS account for 78 % of CPA tumors - mostly from
vestibular branch of VIIIth Nerve.
Variety of other tumors arise from this area like
meningioma , CN swannomas , dermoid tumors , arachnoid
cysts ,lipomas , metastatic tumors , vascular tumors.
Anatomy of CP angle

CPA Irregularly shaped potential space in the posterior
fossa of the brain .
Anteriorly posterior surface of temporal bone .
Posteriorly anterior surface of the cerebellum.
Medially cisterns of the pons & medulla and olive.
Superiorly inferior border of pons & cerebellar peduncle.
Inferiorly cerebellar tonsil.
Anatomy of CP angle

CN s V ( superiorly ) , IX,X,XI (inferiorly ) transverse the
cephalic and caudal extent of the CPA.
The central structures crossing the CPA to & from the IAC
are CN VII & VIII s carrying with them a fine sheet of
arachnoid tissue upto IAC.
Schwann cells sorround these nerves beginning in the IAC ,
near the porus at the Obersteiner- Redlich zone.
Divisions of IAC
Anatomy of CP angle

CN s V ( superiorly ) , IX,X,XI (inferiorly ) transverse the
cephalic and caudal extent of the CPA.
The central structures crossing the CPA to & from the IAC
are CN VII & VIII s carrying with them a fine sheet of
arachnoid tissue upto IAC.
Schwann cells sorround these nerves beginning in the IAC ,
near the porus at the Obersteiner- Redlich zone.
Divisions of IAC
Anatomy of CP angle

AICA is the main artery in the CPA and is the
source of the labrynthine artery .
The labrynthine artery courses via the IAC & is
an end artery for the hearing and balance organs.
Vestibular schwannoma

Nerve sheath tumors of the superior and inferior
vestibular nerves.
They arise in the medial part of the IAC or the
lateral part of the CPA and cause clinical
symptoms by displacing , distorting or
compressing adjacent structures in the CPA.
Vestibular schwannoma

Mean incidence range 9.1 tmr/yr to 13 tmr/yr( as per SB)
0.7 to 1.2 VS per lakh population/yr ( ballenger )
Types - Sporadic ( 95%) and non sporadic ( 5%)
Age of presentation 40 to 60 yrs.
Age of presentation is less in non sporadic ( 20-30 yrs )
Mean growth rate 1.1 mm /yr.


Tumor biology

Equal frequency in sup and inf vestibular ( but recent
japanese studies suggested 85 % from inf vestibular )
Arise from schwann cells within the IAC lateral to O-R
zone in the area of scarpa ganglion.
Schwannomas rarely arise from the cochlear nerve & are
rarely malignant .



Tumor pathogenesis

Owing to mutations in the gene for the tumor suppresor
protein MERLIN located on chr 22q12.
Formation of VS requires mutation of both copies of the
merlin gene.
Somatic mutations in both copies of merlin gene results in
sporadic VSs .
Familial VS occuring in NF 2 requires only one somatic
mutation event .( inherit one )



Tumor pathogenesis

NF2 is autosomal recessive at gene level but inheritance is
autosomal dominant ( pseudodominant )
A mutation in the normal allele leads to bilateral VS by the age
of 20.
Genetic screens for the NF2 mutation have been developed and
are the basis for genetic counselling for family members of NF2
patients

Tumor pathogenesis

Biochemical factors- VS express neuregulin ,which controls
survival and proliferation of schwann cells and its receptors
erbB2 & erbB3.
FGF ,TGF B1 , PDGF & VEGF all these contribute to VS
proliferation.
VS may accelerate during pregnancy.


pathology

GROSS :
VS have a smooth surface with a yellow to gray color.
Tumor is usually solid ,with occasional cystic components and therefore
has a firm to soft texture depending on solid to cystic components.

MICROSCOPIC :
Capsule 3 to 5 micrometer in thickness.
Two morphological tissue types Antony A & Antony B areas
VEROCAY bodies
VS sections stain S 100 immunoperoxidase.

T UMOR DEVELOPMENT

Develops in nerve sheath
Compresses rather than invading the nerve
Gradually fills all the IAC
Protrudes out of the porus
Resorption of bone sorrounding the porus

T UMOR DEVELOPMENT-
extrameatally

Extrameatal expansion into the large & empty pontine cistern
Displacement and stretching of the VII & VIII th CN on the anterior
Compress cerebellum and trigeminal N
Compression & displacement of the brainstem & fourth ventricle


aspect of the tumor & of the AICA on the inferior aspect
(During this time IAM continues to become more & more widened )
which leads gradually to hydrocephalus
T UMOR DEVELOPMENT-
extrameatally

Tumor may extent to the tentorium & can obstruct the
cochlear aqueduct
The AICA & lower cranial nerves are also displaced & become closely
Overtime , the trigeminal & abducens nerves become stretched

Adherent to the inf surface of tumor.
over the surface of the tumor and get thinned.
1. 2.
3.
4.
INTRACANALICULAR CISTERNAL
COMPRESSIVE HYDROCEPHALIC
Tumor development..

Periods of growth are intermixed b/w slow growth &
peroid of quescence.
Occasionally tumor may undergo rapid expansion owing to
cystic degeneration or hemmorhage into the tumor.
The initial intracanalicular growth effects the
vestibulocochlear nerve in the rigid IAC &
causes unilateral HL ,tinnitus and vertigo or dysequilibrium.
Stage 1
Stage 2
Stage 3
Cerebellopontine Angle:

Large Acoustic Neuroma: Tumors over 2.5 centimeters (this one is 2.6 cm) become impacted
into the brainstem and cerebellum. Complications associated with surgery and radiation are
higher. It is difficult to deliver an adequate dose of radiation to control tumor growth without
excessive dosing to the brainstem in tumors larger than this.
Symptoms & signs

Intracanalicular:
Hearing loss (UL progressive ), tinnitus, vertigo
Loss of speech discrimination out of propotion to HL
Cisternal:
Worsened hearing and dysequilibrium
Compressive:
Occasional occipital headache
CN V: Midface, corneal hypesthesia
CN VII : Hitzelbergers sign, loss of taste and reduced
lacrimation on Schirmers test ,facial weakness ( late)
CN II , IV , VI : visual acquity and diplopia

Symptoms & signs

Hydrocephalic:
Fourth ventricle compressed and obstructed
Headache, visual changes, altered mental status
Nausea and vomiting
On examination : ICP and pappiledema.
Compression of CN IX & X
Dysphagia , aspiration and hoarseness
Poor gag reflex and VC paralysis.
Cerebellar involvement( late )
Incoordination , widely based gate , tendency to fall
owards affected side

Symptoms & signs

Brainstem involvement:
There is ataxia, weakness and numbness of arms and legs
with exaggerated tendon reflexes.

Jackler Staging System


STAGE

TUMOUR SIZE

Intra canalicular

Tumour confined to IAC

I (Small)

<10mm

II(Medium)

11-25mm

III(Large)

25-40mm

IV(Giant)

>40mm
Tumor Growth Rate

Duration of Symptoms Prior to
Diagnosis
SYMPTOMS YEARS
Hearing loss 3.9
Vertigo 3.6
Tinnitus 3.4
Headache 2.2
Dysequilibrium 1.7
Trigeminal 0.9
Facial 0.6
Diagnostic evaluation
Average patient will require 4 years from the
onset of symptoms to diagnosis.

Majority will present with complaints of UHL, UT,
Vertigo , dysequilibrium, facial numbness ,
weakness or spasm.

Initial step in evaluation includes an audiologic
assessment .if it suggests a retrocochlear lesion ,
then imaging of the CPA is performed .

Vestibular testing lacks specificity in diagnosis of VS

AUDIOLOGICAL EVALUATION
Includes PTA , Speech discrimination score
(SDS) , Acoustic reflex threshold & acoustic
reflex decay

PTA of patients with VS shows assymetric ,
down sloping , high frequency SNHL in almost
70% of patients
AUDIOLOGICAL EVALUATION
Retrocochlear HL causes SDS to be lower than
predicted by the pure tone thresholds.

This out of propotion is furthur accenuated
when retested at a higher speech intensity
( roll over phenomenon )

Loss of acoustic reflex or acoustic reflex decay is
noted in most patients with VS
audiological tests

Cochlear Retrocochlear
a) Pure tone audiometry Sensorineural hearing loss Sensorineural hearing loss

b) Speech discrimination
score
<90% Very poor
c) Roll over phenomenon Absent Present
d) Recruitment Present Absent
e) SISI Over 70% 0-20%
f) Threshold tone decay
test
<25db >25db
g) Stapedial reflex Present Absent
i) Stapedial reflex decay
test
Normal Absent
VESTIBULAR TESTING
Not sensitive nor specific for diagnosing VS

The MC test used is ENG with caloric testing.

Shows reduced caloric response in the affected ear.

The extent of vestibular function present predicts the
amount of post op vertigo.

The location of VS on the inf or sup Vestibular N may
also be predicted.
AUDITORY BRAINSTEM RESPONSE
In patients with VS , the ABR is partially or
completely absent , or there is a delay in
latency of wave V on the affected side.

An interaural delay of wave V greater than
0.2 ms is considered abnormal. ( 40-60 % )

Overall ABR has a sensitivity of > 90% &
specificity of > 90 % in detecting VS.
AUDITORY BRAINSTEM RESPONSE

In 20-30 % there are no identifiable
waveforms even with insignificant HL in
higher frequencies.

In 10-20 % only wave I is present.
BERA patterns in AN

Imaging studies
VS is definitely identified MC via an imaging study.

Earlier plain film radiograghs and polytomographs

Introduction of CT in 1970 allowed axial imaging
with improved bone & soft tissue evaluation.

With the addition of iv iodinated contrast agent
,90 % of VS are enhanced furthur improving
diagnostic accuracy.
Imaging studies
Intracanalicular tumors & tumors extending less
than 5 mm into the CPA frequently are missed
with contrast enhanced CT.

Accuracy improved by air-contrast cisternography.

MRI was introduced in 1980 & has become the
GOLD standard for VS
Imaging studies
MRI :
VII & VIII nerves as well as cerebellum ,brainstem , vasculature
& other structures are well visualized on MRI

The addition of gadolinium furthur enhanced the diagnostic
accuracy

Typically a series of T1 weighed images in which CSF is dark
and fat is bright, T1 with gadolinium contrast , T2 In which
CSF is bright is used.

A hypointense globular mass centered over the IAC on T1 With
enhancement on gadolinium.

VS are iso-to hypointense on T2.

T2 fast spin echo MRI without contrast as screening.

MRI Brain

Isointense to brain,
hyperintense to CSF
Hyperintense to brain,
hypointense to CSF
management options
The primary management of VSs is surgical removal.

Roles of observation and radiotherapy are currently for
the pts,who cannot tolerate a surgical procedure or have
a life span of < 5 yrs.

Surgical approaches to the CPA include:
Translabrynthine
Retrosigmoid
Middle fossa craniotomies.

management options
The appropriate approach for a particular
pt. is based on the hearing status , size of
the tumor , extent of IAC involvement and
experience of the surgeon

The approaches are either hearing
preservative or ablating.

The retrosigmoid & middle fossa
approaches are hearing preserving, while
translabrynthine approach is otherwise.

management options

The middle fossa approach is well suited for the
pts with good hearing and tumor<2cm.

The retrosigmoid approach is well suited for
those with good hearing and tumor<4cm and
not involving the lateral part of IAC.

The translabrynthine approach causes total
hearing loss and so is appropriate for the pts with
poor hearing(PTA>30dB) or pts with good
hearing and tumors not accessible by the hearing
preserving approach.
management options
Three critical issues inherent to all the three techniques are:
Extent of exposure of IAC and CPA
Identification and preservation of the facial nerve
extent of brain retraction

These operations use electro physiologic
monitoring of CN VII and ABR in hearing
preserving approach.
Middle Fossa Approach.
Translabyrinthine Approach
Suboccipital Approach
Surgical Approaches
Factors that influence surgical approach selection

Age
Hearing status
Tumour size
Surgeons preference.

Translabrynthine approach
The primary approach for removal of VS.
Most direct route to the CPA & requires
minimal cerebellar retraction.
Identification of facial n is possible.
Surgeons can ensure complete removal bcz
fundus of IAC is widely exposed.
Immediate repair of facial n possible.
Recovery is quite rapid with minimal pain and
excellent facial n results

Translabrynthine approach
Obvious disadvantage is sacrifice of any residual
hearing.
Technique :
A postauricular incision is made 2 cm behind sulcus

Complete mastoidectomy is done,with identification of
the middle fossa dura, sigmoid sinis , LSSC , fossa incudis
& facial n

The sigmoid sinus is decompressed with a diamond burr



Translabrynthine approach
A labrynthectomy is begun by removal of bone in the
sinodural angle along the horizontal scc

Each SCC is then opened and followed into the vestibule,
with care taken to identify the ampulla of each SCC and
the subarcuate artery

A bone is removed along the posterior fossa dura medial
to sigmoid sinus , the endolymphatic duct and sac are
encountered.



Translabrynthine approach
Jugular bulb location is defined by locating ampulla of
posterior canal. ( inferior extent of dissection )

Bone is removed around the inferior aspect of IAC until
the cochlear aqueduct is identified

Posterior aspect of the canal is skeletonized until the
superior edge of the internal canal is identified

Bone is then carefully removed between th e middle
fossa dura & the IAC




Translabrynthine approach
Once the medial portion of the IAC is exposed for 270
the remaining piece of porus may be carefully removed

Laterally the transverse crest should be identified at the
fundus of the IAC.

Superiorly , the Bills Bar is identified together with the
labrynthine portion of the facial n

The posteroir fossa dura is opened inferior to and parallel
to the superior petrosal sinus over the midportion of the
IAC



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Translabrynthine approach
Using the bills bar as guide , and with a fine hook , the
surgeon seperates the superior vest n from facial nerve.

The capsule of the tumor is incised , & the tumor is
gutted with house urban dissector


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Radiotherapy :-
1. Conventional radiotherapy by external beam
has no role in the treatment of Acoustic Neuromas
due to low tolerance of CNS to radiation.
2. X or Gamma knife surgery.
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