ACOUSTIC NEUROMA

DR.MUHAMMAD ALI
SENIOR REGISTRAR ENT
FMC / PAF HOSPITAL ISLAMABAD
IAC
 EPIDEMIOLOGY
 Male = female distribution
 Typically present 40 to 60 years (younger if
neurofibromatosis 2 [NF2])
 Incidence 1/100,000 per year (autopsy studies show up to
0.7% population may have vestibular schwannoma at
death)
 95% unilateral, sporadic

 5% bilateral—associated with NF2

 Account for 80% of cerebellopontine angle (CPA) tumors and

6% of all intracranial tumors.

 Most commonly arise from the superior vestibular division of

CN VIII.
 PATHOPHYSIOLOGY

 Mostly originate from Schwann cells of vestibular nerve,

within internal auditory meatus.

 Hemorrhage can occur in reticular type leading to sudden

increase in size
 Cystic degeneration possible
 NF2—autosomal dominant; aberration on long arm of chromosome
22 leading to defect in tumor suppressor protein merlin (aka
schwannomin, neurofibromin 2)

 Vestibular schwannomas demonstrate two typical histologic

architectures. Elongated spindles are predominant in either pattern:
Antoni A pattern shows dense nests of cells while Antoni B is
interwoven and less densely packed.
 CLINICAL FEATURES

 Otological phase: small tumor compresses structures in the internal

auditory meatus
 Gradual and progressive unilateral deafness (90%)—degree of hearing loss
(HL) not related to size of tumor
 Sudden-onset HL (10%)
 Normal hearing (5%)
 Associated with unilateral tinnitus (70%)—can be the only symptom
 Imbalance unusual unless acutely due to bleed inside tumor (slow
compression allows for gradual compensation); dysequilibrium often present
with rapid head movements. true vertigo is seldom seen
Neurological phase: expansion into CPA:
 Trigeminal nerve symptoms: facial pain, numbness,
loss of corneal reflex. V nerve is earliest to be involved.

 Headache—posterior fossa dura irritation leading to

dull aching around ear

 Hitzelberger sign: anaesthesia / hypoaesthesia of

posterior meatal wall (sensory fibers of VII n are
affected early.
 Late symptoms
 Facial weakness—uncommon (gradual compression allows
for compensation as normal number of end plates are
innervated by fewer neurons)

 Ataxia/unsteadiness—brainstem/cerebellar involvement

 Diplopia (VI n)—rare

 Hoarseness and dysphagia (IX n, X n)—rare

 Terminal symptoms (raised ICP):
 failing vision (papilloedema), headache, reduced GCS /
coma
 DIFFERENTIAL DIAGNOSIS OF CPA
TUMORS

Meningioma Epidermoid Arachnoid Cerebellar

(cholesteatoma) cyst
Schwannoma Aneurysm of Cholesterol Metastatic
of other cranial basilar/vertebral granuloma of
nerves (e.g. CN arteries petrous apex
V >VII>IX,
X, XI)
INVESTIGATIONS

Specific

fitness
 Imaging
 MRI brain with contrast with special attention to
CPA is the gold standard for diagnosis.
 Vestibular schwannomas is isointense on T1 and
T2, while enhancing with contrast on T1.
 (CT with contrast if MRI contraindicated).
Audiology

 PTA: SNHL, mostly at high-frequency.

 Speech audiometry: poor speech discrimination / Roll-

over phenomenon, i.e. reduction of discrimination
score when loudness is increased
  Brainstem evoked response: 95% sensitive. Inter-aural
latency of wave V. A delay of > 0.2 ms in wave V
between two ears is significant.

 Stapedial reflex decay test

Caloric test will show diminished / absent response in

96% of patients. When tumor is very small, caloric test
may be normal.
 MANAGEMENT

•Multidisciplinary team approach

•Considerations in treatment planning

Tumor size growth rate hearing levels

severity of symptoms anesthetic risk Age

 CONSERVATIVE

 65% do not show any signs of growth, so watchful wait

with serial scanning, especially in elderly or medically unfit

 Has best rates of hearing preservation compared with

surgery or radiotherapy

 If grow, usually <2 mm/year

 If no growth over 5 years, unlikely to ever grow.

 RADIOSURGERY OR RADIOTHERAPY

 Considered for tumors <3 cm diameter at CPA

 Hyperfractionated stereotactic radiotherapy
 Gamma knife / CyberKnife—uses an alternative radiation source
 Benefits
 Some studies suggest better hearing preservation compared with surgery
 Risks are delayed, versus upfront risks of surgery
 Outpatient procedure
 Avoids risks of craniotomy
 SURGERY

 The larger the tumor the greater the risks (e.g., to facial
nerve). If tumor >1.5 cm at CPA, risks to hearing and facial
nerve increase. If tumor >2.5 cm, generally requires surgery.
 There are three typical surgical approaches: middle fossa,
retrosigmoid, and translabyrinthine
MIDDLE FOSSA APPROACH

 It is appropriate for tumors less than 1.5 cm (mostly

intracanalicular).

 Hearing preservation is possible, and the entire IAC

can be accessed.

 Most commonly used for small, lateral tumors.

RETROSIGMOID (SUBOCCIPITAL) APPROACH

 It can be used for hearing preservation for

tumors up to 2 cm.
 TRANSLABYRINTHINE APPROACH

 It can be used for any size tumor, but has no chance of

hearing preservation (useful for patients with no serviceable
hearing).
 It has the best exposure of the facial nerve and is the most
commonly used approach.
 It also requires very little brain retraction.
 Excellent CPA exposure
Thank
you