Acoustic Neuroma

case study - February 26, 2014

An acoustic neuroma (also known as a vestibular schwannoma) is a
benign tumour that originates from the eighth cranial nerve. This nerve
passes from the brainstem to the inner ear and is involved in the
transmission of sound and balance from the inner ear to the brain.
The eighth nerve is closely associated with the seventh nerve, as they
run together in the internal auditory canal to the inner ear. The seventh
nerve is responsible for the motor supply of muscles for
facial expression. Therefore if the nerve is damaged, the facial muscles
weaken. Acoustic neuromas are a slow growing tumour and they
generally arise from the vestibular nerves. As they grow in size they can
affect the balance and hearing nerves, resulting in symptoms such as
deafness, loss of balance and tinnitus. If the tumour gets very large it can
cause numbness of the face and facial weakness, or compression of
the brainstem, causing stroke-like symptoms of arm and leg weakness.
Tumours are typically described as small (less than 1.5cm), medium (1.5
to 2.5cm) or large (more than 2.5cm).
There are three treatment options available to patients; observation,
surgery and radiation therapy or radiosurgery.
Choosing the best therapy is a decision made by both the patient and the
surgeon after consideration of the patients age,
symptoms, health, tumour size and hearing ability. Surgical treatment
options for the removal of an acoustic neuroma include the
translabyrinthine approach, the retrosigmoid/sub-occipital approach
and the middle fossa approach. The choice of approach is based on
factors such as tumour size, location, surgeon preference and whether
hearing preservation is a goal.
The main objective of the surgery is tumour removal without
causing neurological damage or other complications. However, facial

nerve preservation is also an important objective. The larger the tumour,

the more difficult it is to preserve the nerve.

This nerve is closely applied to the tumour and if damaged, facial

weakness results. This can be permanent and causes a significant
cosmetic effect with facial drooping. Severe facial weakness can impair
eye closure, resulting in corneal dryness, ulceration and even
blindness if not managed. It is therefore very important to preserve the
facial nerve.

* Illustrations from www.anausa.org

What Is It?An acoustic neuroma is a type of benign (noncancerous)

brain tumor that grows on the vestibular nerve as it travels from the
inner ear to the brainstem. It is one of the most common types of benign
brain tumors. The first sign of one is usually hearing loss.

The cochleo-vestibular nerve (also called the eighth cranial nerve) is

made up of three nerves that connect the inner ear to the brain. One
branch the cochlear nerve carries hearing information. The other
two branches the inferior and superior vestibular nerves carry
balance information to the brain. The nerves are wrapped in a layer of
specialized cells called Schwann cells. An acoustic neuroma also
called a vestibular schwannoma is a tumor of those cells. If an
acoustic neuroma is not diagnosed or treated it can grow large enough to
press on important structures in the brainstem and cause major life
threatening problems.
The main symptoms of an acoustic neuroma are hearing loss and tinnitus
(ringing in the ears). They are caused by the tumor pressing on the
auditory nerve. Although the tumor actually grows on the balance nerve,
imbalance is usually mild or absent. Since we have two balance systems,
the opposite side can compensate for the slow progressive loss caused by
the tumor.
These tumors have been linked to a mutation in a protein that regulates tumor
suppression. In most cases the tumor grows only on one side of the head and is
diagnosed between the ages of 30 and 50. Acoustic neuromas in children are very
rare. People with a hereditary disease called neurofibromatosis type 2 develop

bilateral acoustic neuromas because they lack the tumor suppressor protein merlin.
About 10% of all acoustic neuromas occur in people with neurofibromatosis.
Acoustic neuroma: A benign tumor that may develop on the hearing and balance
nerves near the inner ear. The tumor results from an overproduction of Schwann
cells -- small sheet-like cells that normally wrap around nerve fibers like onion
skin and help support the nerves. When growth is abnormally excessive, Schwann
cells bunch together, pressing against the hearing and balance nerves, often causing
gradual hearing loss, tinnitus (ringing in the ears), and dizziness. If the tumor
becomes large, it can interfere with the facial nerve, causing partial paralysis, and
eventually press against nearby brain structures, becoming life-threatening.
The early diagnosis of an acoustic neuroma is the key to preventing its serious
consequences. Unfortunately, early detection of the tumor is sometimes difficult
because the symptoms may be subtle and may not appear in the beginning stages
of growth. Once the symptoms do appear, a thorough ear examination and hearing
test are essential for diagnosis. Computerized tomography (CT) scans and
magnetic resonance imaging (MRI) are helpful in determining the location and size
of a tumor and also in planning its microsurgical removal.
If an acoustic neuroma is surgically removed when it is still very small, hearing
may be preserved and accompanying symptoms may go away. As the tumor grows
larger, surgical removal is often more complicated because the tumor may become
firmly attached to the nerves that control facial movement, hearing, and balance.
The removal of tumors attached to hearing, balance, or facial nerves can make the
patient's symptoms worse because sections of these nerves must also be removed
with the tumor. As an alternative to conventional surgical techniques, radiosurgery
may be used to reduce the size or limit the growth of the tumor. Radiosurgery,
utilizing carefully focused radiation, is sometimes performed on the elderly, on
patients with tumors on both hearing nerves, or on patients with a tumor growing
on the nerve of their only hearing ear. If the tumor is not removed, MRI is used to
carefully monitor its growth.
There are two types of acoustic neuroma: unilateral and bilateral. Unilateral
acoustic neuromas affect only one ear. They account for approximately 8 percent
of all tumors inside the skull. Symptoms may develop at any age but usually occur
between the ages of 30 and 60 years.

Bilateral acoustic neuromas, which affect both ears, are hereditary. Inherited from
one's parents, this tumor results from a genetic disorder known as
neurofibromatosis-2 (NF2). Affected individuals have a 50 percent chance of
passing this disorder on to their children. Unlike those with a unilateral acoustic
neuroma, individuals with NF2 usually develop symptoms in their teens or early
adulthood. Because NF2 patients usually have multiple tumors, the surgical
procedure is more complicated than the removal of a unilateral acoustic neuroma.
Further research is needed to determine the best approach in these circumstances.
In addition to tumors arising from the hearing and balance nerves, NF2 patients
may develop tumors on other cranial nerves associated with swallowing, speech,
eye and facial movement, and facial sensation. NF2 patients may also develop
tumors within the spinal cord and on the brain's thin covering.
Both types of acoustic neuroma occur following a loss of the function of a gene on
chromosome 22. A gene is a small section of DNA responsible for a particular trait
like hair color or skin tone. This particular gene on chromosome 22 suppresses the
growth of Schwann cells. When this gene malfunctions, Schwann cells can grow
out of control. This gene may help suppress other types of tumor growth. In NF2
patients, the faulty gene on chromosome 22 is inherited.
Acoustic neuroma is also called an acoustic neurinoma or a vestibular
schwannoma.