ACOUSTIC NEUROMA

(VESTIBULAR SCHWANNOMA)
LITERATURE READING

Supervisor : Dr. dr. Wijana, M.Kes., Sp.THT-KL(K), FICS

Dept of Otorhinolaryngology – Head and Neck Surgery

Hasan Sadikin General Hospital
Bandung
2018
 Introduction
Definition:
• Tumour of eighth cranial Nerve
• Originate from Schwann cell

Eponyms:
• Vestibular Schwannoma

Bush ML, Welling DB. Cerebellopontine Angle Tumors, Bailey’s Head & Neck Surgery, 5th edition, Lippincott William&Wilkin, 2014,
p.2557-2586
 Location (Microscopic)
• The junction between the central and peripheral
components of the cranial nerves/Obersteiner-Redlich
zone
• Locations with the greatest concentration of Schwann
cells

Bush ML, Welling DB. Cerebellopontine Angle Tumors, Bailey’s Head & Neck Surgery, 5th edition, Lippincott William&Wilkin, 2014,
p.2557-2586
 Location (Macroscopic)
• CP angle’s tumors
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.

Bush ML, Welling DB. Cerebellopontine Angle Tumors, Bailey’s Head & Neck Surgery, 5th edition, Lippincott William&Wilkin, 2014,
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Anatomy
Cerebellopontine
Angle

Bush ML, Welling DB. Cerebellopontine Angle Tumors, Bailey’s Head & Neck Surgery, 5th edition, Lippincott William&Wilkin, 2014,
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 AICA travels
laterally in IAC
to supply
cochlea and
labyrinth

Bush ML, Welling DB. Cerebellopontine Angle Tumors, Bailey’s Head & Neck Surgery, 5th edition, Lippincott William&Wilkin, 2014,
p.2557-2586
6
 • Nerve sheath
tumors of the
superior and
inferior vestibular
nerves.
• Arise in the medial
part the IAC or the
lateral part of the
CPA → clinical
symptoms by
displacing ,
distorting or
compressing
adjacent
structures.

Bush ML, Welling DB. Cerebellopontine Angle Tumors, Bailey’s Head & Neck Surgery, 5th edition, Lippincott William&Wilkin, 2014,
p.2557-2586
 Epidemiology
Vestibular schwannomas :
• 10% of all intracranial tumors and 80% of
cerebellopontine angle tumors
• 30% of brainstem tumors
• The incidence rate is variable globally  reported
1.9 tumors per 100.000 people per year

Bush ML, Welling DB. Cerebellopontine Angle Tumors, Bailey’s Head & Neck Surgery, 5th edition, Lippincott William&Wilkin, 2014,
p.2557-2586
 Patophysiology
• Schwannomas in NF2 patients : Defect on the long
arm of chromosome 22, production of a tumor
suppressor protein called merlin.
• Without NF2, mutations must occur on the long arm
of both chromosomes
• Increased genetic expression of neurotrophic factors,
brain derived neurotrophic factor
• Small RNA molecules called microRNA →
overexpressed, which can lead to downregulation of
tumor suppressor pathways.
Bush ML, Welling DB. Cerebellopontine Angle Tumors, Bailey’s Head & Neck Surgery, 5th edition, Lippincott William&Wilkin, 2014,
p.2557-2586
 Histopatology

• Histologic examination reveals two discrete cell arrangements

• Antoni type A arrangement : Regions of compact spindle cells
with bland cytoplasm and lack of nuclear atypia along with
whorls of palisading nuclei aligned in rows (Verocay bodies).
• Antoni type B possesses a less dense and more loosely
arranged cellular histology.
Bush ML, Welling DB. Cerebellopontine Angle Tumors, Bailey’s Head & Neck Surgery, 5th edition, Lippincott William&Wilkin, 2014,
p.2557-2586
GROSS
• Smooth surface with a
yellow to gray color,
with occasional cystic
components and
therefore has a firm to
soft texture depending
on solid to cystic
components.
 Tumor Development
• Periods of growth – intermixed : Slow
• 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

Bush ML, Welling DB. Cerebellopontine Angle Tumors, Bailey’s Head & Neck Surgery, 5th edition, Lippincott William&Wilkin, 2014,
p.2557-2586
Stage 1

Stage 2

Stage 3
Jackler Staging system
STAGE TUMOR SIZE
Intracanalicular Tumor confined to IAC
I (small) < 10 mm

II (medium) 11-25 mm

III (Large) 25-40 mm

IV (Giant) > 40 mm
 Symptoms and 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 : Hitzelberger’s sign, loss of taste and reduced
lacrimation on Schirmer’s test ,facial weakness ( late)
CN II , IV , VI : visual acquity and diplopia
Bush ML, Welling DB. Cerebellopontine Angle Tumors, Bailey’s Head & Neck Surgery, 5th edition, Lippincott William&Wilkin, 2014,
p.2557-2586
 Symptoms and 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
towards affected side
Bush ML, Welling DB. Cerebellopontine Angle Tumors, Bailey’s Head & Neck Surgery, 5th edition, Lippincott William&Wilkin, 2014,
p.2557-2586
Frequency ofSymptoms
Hearing Loss (85-97% ; 94% )
Vertigo (5-70 % ; 39% )
Dysequilibrium (46-70% ; 56 %)
Tinnitus (56-70% ; 64 %)
Facial nerve (10-77% ; 38 %)
Trigeminal nerve (16-63% ; 26 %)
Headache (12-38% ; 25% )
Visual symptoms (1- 15 % ; 7% )
Lower cranial nerves: Dysphagia, Hoarseness,
Aspiration,
Shoulder weakness (Jugular foramen syndrome)

Jackler RK. 2000, p. 182: Tumors of the Ear and Temporal Bone
 Hearing Loss
• Most frequent initial symptom

• Most common symptom ~ 95% AN patients

• Asymmetric SNHL

• Down-sloping / High Frequency

• Decreased Speech Discrimination

Bush ML, Welling DB. Cerebellopontine Angle Tumors, Bailey’s Head & Neck Surgery, 5th edition, Lippincott William&Wilkin, 2014,
p.2557-2586
Normal or Hearing in Acoustic Neuroma

Selesnick Shaan Lustig Magdziarz

1993 1993 1998 2000

AN 126 100 546 369

patients
Normal 5 6 29 10
hearing (4%) (6%) (5%) (3%)
 SSNHL

1-2 % SSNHL

10- 26 % VS patients have a history of SSNHL

Most experts advocate obtaining MRI in all

patients who present with SSNHL

Bush ML, Welling DB. Cerebellopontine Angle Tumors, Bailey’s Head & Neck Surgery, 5th edition, Lippincott William&Wilkin, 2014,
p.2557-2586
 Audiology Testing
Audiogram
ABR
OAE
Vestibular testings
Radiography
MRI Gold Standard
CT-Scan

Bush ML, Welling DB. Cerebellopontine Angle Tumors, Bailey’s Head & Neck Surgery, 5th edition, Lippincott William&Wilkin, 2014,
p.2557-2586
Pure Tone Audiometry
ABR Pathology
Increased interpeak intervals

I-to-III interval of 2.5 ms, III-to-V interval of 2.3 ms, and I-

to-V interval of 4.4 ms

Interaural wave V latency difference (IT5) Greater than

0.2 ms

Poor waveform morphology ie. only some of the waves

are discernible

Absent waveform
 10-20 % with
only wave I and
nothing
thereafter
40-60 % with
wave V latency
delay
10-15 % have
normal findings

Fraysse B et al. First International Conf. on Acoustic Neuroma. 1992

 Vestibular Complain
- 36-50% of patients describe disequilibrium.

- Vague, transient lightheadedness  that may be

exacerbated by positional changes.

- Acute vertigo is presenting symptom in 27% of

patients but is associated with smaller tumors.

Bush ML, Welling DB. Cerebellopontine Angle Tumors, Bailey’s Head & Neck Surgery, 5th edition, Lippincott William&Wilkin, 2014,
p.2557-2586
 Vestibular Testing
Not sensitive nor specific

Shows reduced caloric response in the affected ear.

The extent of vestibular function present predicts the

amount of post op vertigo.

The location of AN on the inf or sup Vestibular N may also

be predicted.

Bush ML, Welling DB. Cerebellopontine Angle Tumors, Bailey’s Head & Neck Surgery, 5th edition, Lippincott William&Wilkin, 2014,
p.2557-2586
Imaging Studies
Intracanalicular tumors & tumors extending < 5

mm into the CPA, missed with contrast enhanced

CT.

Accuracy improved by air-contrast

cisternography.

MRI was introduced in 1980 & has become the

GOLD standard.
CT - Scan
90% of AN will
enhance with contrast
Frequently misses
tumors that are not
intracanalicular and do
not extend >5mm to the
CPA.
63% Accuracy at
diagnosis
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..
AN are iso-to hypointense on T2.
MRI

T1 T2 T1+Gad
 Treatment
• Observation : more than 60% of vestibular schwannomas, grow
very slowly, stable in size or involute during 4 years of follow-
up.
• Stereotactic radiation : 12 to 13 Gy using highly acurate tumor
targeting technology , complications can be minimized while
tumor growth is controlled in approximately 95%, not
recommended for tumor greater than 3 cm in diameter or
symptomatic with significant brainstem compression.
• Surgical excision : Curative therapy available. The major
disadvantage is morbidity associated with cranial nerve injury,
cerebrospinal fluid leak, and central nervous system damage.

Bush ML, Welling DB. Cerebellopontine Angle Tumors, Bailey’s Head & Neck Surgery, 5th edition, Lippincott William&Wilkin, 2014,
p.2557-2586
Thank You