ACOUSTIC

NEUROMA

VESTIBULAR SCHWANNOMA/ ACOUSTIC

NEUROMA

Its the most common intracranial schwannoma

constituting 80 % of all cerebellopontine angle tumors.
Its benign encapsulated , extremely slow-growing
tumor either solid or cystic which arises from the
Schwann-cells (transition zone) of the vestibular nerve
within the internal auditory canal are histologically
composed of alternating regions of Antony type A and B
areas. It can extend into the cerebellopontine angle.
The number of newly diagnosed cases per year is
around 13 per one million individuals.
Bilateral AC is diagnostic of neurofibromatosis type 2.

ANATOMY OF CEREBELLOPONTINE
ANGLE
Its a triangular area and bounded by:
1- Laterally: medial portion of posterior surface of
temporal bone.
2- Medially: Edge of pons.
3- Posteriorly: cerebellar hemisphere and flocculus.
4- Superiorly: trigeminal nerve.
CONTENTS:
1- Anterior inferior cerebellar artery.
2- 7th and 8th Cranial nerves.

FEATURES:
1- Age most common in 40-60 yrs, but when the
tumor is found in association with neurofibromatosis
type 2 it occurs at the age of 20-30 yrs.
2- Both sexes are affected equally.
3- Its radioresistant.
4- In 90% of cases its unilateral , may be bilateral in
Vonrecking Hausen disease ( type 2 NF).
5- Most schwannomas are sporadic tumors and
6- The gene responsible for the development of
vestibular schwannomas has been isolated to
chromosome 22q12, which codes for the tumour
suppressor merlin (or schwannomin).

CLINICAL SYMPTOMS
1- Most common ; Progressive Unilateral SNHL
(retrocochlear) present in 95% of cases and often
accompanied by tinnitus which is present in 65% of
cases.
2- Marked difficulty in understanding speech out of
proportion to the pure tone hearing loss.
3- May present with sudden hearing loss.
4- True vertigo is seldom seen.
5- Earliest cranial nerve involved is 5th CN.
6- 4th , 6th, 9th , 10th , 11th and 12th can also be involved.
7- Rare presentations include facial numbness or pain,
earache or facial weakness, cerebellar ataxia or
symptoms
of hydrocephalus (headache, visual disturbance,
mental

SIGNS
Ear: normal otoscopy.
Cranial nerves:
1- 5th CN: Earliest sign is impaired corneal
reflex.
Motor functions are affected rarely.
2- 7th CN: Sensory first; loss of sensation in
the postero-superior aspect of EAC called
Hitselberger sign.
3- 9th and 1oth pulsy: palatal, pharyngeal
and laryngeal paralysis.
4- Eyes: Nystagmus.
5- Cerebellar signs present.

CLASSIFICATION OF AN ACCORDING TO
SIZE
Intameatal

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

More than 40

INVESTIGATIONS
1- Audiological tests: show features of retrocochlear
hearing loss (high frequency SNHL) ; recruitment negative,
poor speech discrimination score and presence of roll-over
phenomenon.
2- Acoustic reflex: stapedial reflex decay or missing in up

to 70% of patients.
3- Caloric test: diminished or absent normal test finding
does not eliminate the diagnosis.
4- Plain x-ray: best view perorbital view ; difference of 1
mm in the vertical height of internal acoustic meatus is
significant.
5- CT-scan; can not detect intermeatal tumors.
6- MRI; MRI with gadolinium enhancement is is gold

standard.

Conservative Treatment
Since especially in the elderly vestibular schwannomas
are slowly growing tumours, a wait and see strategy
should be considered at least in the short term (612
months) for patients with a small vestibular schwannoma
(in older patients and individuals in poor health).
Radiotherapy: Stereotactic radiosurgery (gamma
knife) may stop vestibular schwannoma growth mainly in
small intracanalicular and extracanalicular lesions.
Radiosurgery as the first therapeutic option is
recommended in bilateral vestibular schwannomas (e. g.
morbus Recklinghausen) but only when the tumours are
small.
Annual imaging is recommended for all patients being
managed conservatively for the rest of their life or until
vestibular schwannoma growth is seen to a certain
limit.

Surgical Treatment
Translabyrinthine approach in patients with
medium sized and large tumours and in patients with
small tumours with poor hearing .
Middle fossa approach in patients with
intracanalicular or small tumours with good hearing as
an attempt to preserve residual hearing.
Retrolabyrinthine or retrosigmoidal
approaches in patients with large extracanalicular
vestibular schwannomas
Additional Useful Surgical Procedures
Intraoperative facial nerve monitoring.
Intraoperative hearing monitoring: may be useful in

Differential Diagnosis
Meningioma is, in most cases, recognized on
radiologic features (tumour usually extrinsic to
internal auditory canal, sessile with peritumoral
dural enhancement and intratumoral
calcifications). An enhanced dural tail is a
distinguishing feature.
Other cerebellopontine angle lesions
(cholesteatoma, arachnoid cyst, lipoma,
haemangioma, glomus tumour, facial neuroma)
can in most cases be recognized by their
radiologic aspects and MRI characteristics

Prognosis
Conservative Therapy With radiotherapy tumour
control is around 96%, tumour regression 35%, lesion
of the trigeminal nerve less than 5.6%, facial weakness
2%, and hearing loss 32%.
There is no CSF leak and no mortality.
If irradiation cannot stop tumour growth (in about
4%
of cases), surgery is technically sometimes extremely
difficult.
Malignant transformation after radiotherapy is also
valid for neuromas.