Patient Information

Leaflet

Newly diagnosed
Vestibular Schwannoma
Patients

Date agreed: December 2017 Version 1 Review December 2019 1

 Vestibular Schwannoma

Introduction

This information is designed to help you answer the common questions that
are asked by patients after the initial consultation. It is hoped that it will help
you understand your diagnosis.

Aintree Hospital and The Walton Centre has a specialist skull base team who
deal with rare inner ear tumours known as vestibular schwannomas. Our team
aim to help you understand about acoustic neuroma, its treatment options and
its effect on you.

What is Vestibular Schwannoma?

You have been diagnosed as having a Vestibular Schwannoma (VS) also

commonly known as Acoustic Neuroma. This is a benign tumour which grows
from the lining of the hearing and balance nerve (sometimes called the
vestibular-cochlear nerve, acoustic nerve or VIII nerve). It is a benign tumour,
and therefore not a cancer, which grows very slowly usually over many years.

The vestibular-cochlear nerve (the nerve of hearing and balance) travels

through a narrow channel in the bone of the skull to the inner ear (the internal
auditory canal). The tumours originate inside the bony channel and grow into
the space where the brain is situated. Once a tumour reaches a certain size, it
can press on the brain. It does not grow or spread into the brain tissue itself.

What causes Vestibular Schwannoma?

It is not fully understood what causes vestibular schwannomas. These

tumours are caused by damage to the genetic material inside the lining of the
nerve. The cause of this damage is unknown. It is not caused by anything you
have done and it is not passed on to your children.

In a small minority of patients they are due to a genetic abnormality called

neurofibromatosis type 2 (NF2), where people may have multiple benign
lesions. If we feel that you need any further investigation for this condition, we
will discuss this with you at your outpatients’ appointment.

How common are Vestibular Schwannoma?

Vestibular schwannomas are very rare. There is one new vestibular

schwannoma diagnosed each year for every 100,000 people in the
population. They can occur at any age but are most common in people in their
50’s and 60’s. They affect men and women equally.

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 How fast do Vestibular Schwannomas grow?

The average growth rate of a vestibular schwannoma is 1-2mm per year

though occasionally the tumour can grow more quickly (4-5mm per year), and
frequently it stops growing all together. The timing of follow up scan allows for
this very slow potential growth which means if surgery is required it is
generally safe to plan surgery ahead of time.

What symptoms do vestibular schwannoma cause?

Hearing loss

Hearing loss on one side is usually the most common symptoms that people
with vestibular schwannoma experience. This is due to the tumour interfering
with the function of the nerve as it grows. Hearing loss may be sudden or
gradual and therefore you may not have noticed the hearing loss in its early
stages. Approximately 90% of people with a vestibular schwannoma
experience some degree of hearing loss.

Tinnitus

Tinnitus, a ringing, buzzing or wooshing noise in the ear, is also another

common symptom. This can be quite troublesome but there are a number of
effective therapies, which can help manage this. Other symptoms include
feeling of fullness in the ear or aching of the bone behind the ear.

Imbalance or dizziness

Some people also experience imbalance or dizziness. As the vestibular

portion of the nerve is compressed your ability to manage balance may
decrease. It is common to feel like you are veering to one side when walking.

The severity of the hearing loss, tinnitus or balance disturbance does not
reflect the size of the tumour.

There are other possible symptoms due to the pressure on the facial nerve
(VII nerve) and the trigeminal nerve (V nerve) that sits next to the vestibular-
cochlear nerve (VIII nerve).

The trigeminal nerve (V nerve) controls sensation on the side of the face and
chewing muscles. Symptoms may be in the form of altered sensation on the
side of the face, numbness and occasionally pain or simply altered feeling.

The facial nerve (VII nerve) controls the facial muscles on the same side of
the face. Pressure to the nerve can cause weakness of the facial muscles, it
is very rare to experience this as a result of the VS, but has been reported by
some people with vestibular schwannomas.

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 Some people have no symptoms at all, and the vestibular schwannoma is
discovered incidentally while they are being investigated for something
entirely unrelated.

Are vestibular schwannomas dangerous?

Vestibular schwannomas are not cancerous and do not spread to other areas
of the body. If they grow into the space where the brain is situated, they can
compress the brain. This may cause symptoms such as headaches or your
mobility and balance worsening.

If a vestibular schwannoma is allowed to grow very large, this compression

can lead to life-threatening complications. However we monitor and treat
these tumours to prevent this from happening.

How are vestibular schwannomas diagnosed?

Your doctor may have examined your balance, your hearing and your nerve
function if a vestibular schwannoma was suspected.

Confirmation of a vestibular schwannoma is usually through a MRI scan

(magnetic resonance imaging). Your surgeon may also send you for a CT
scan (computer tomography) to gather more information.

Once a vestibular schwannoma is diagnosed, its relative rarity means it is

important that you are referred on to a specialist team who are used to
managing these tumours. That is why you have been referred to Aintree
Hospital.

Aintree and Walton’s Skull Base Team

The Skull Base Team consists of specialist both from Aintree Hospital and
The Walton Centre and is made up of:

ENT (ear, nose and throat) surgeons

Miss N Munir
Mr A Youssef
(Mr T Lesser)

Neurosurgeons
Miss C Gilkes
Miss A Visca

Oncologists (Radiation Specialists)

Dr D Husband
Dr A Shenoy
Dr A Haridas

Neurosurgical Clinical nurse specialist

Emma Wilby

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 ENT nurse specialists & Advanced Nurse Practitioners
Caroline Smith
Rebecca Donald
Nicky Carmichael
Charlotte Halpin

Lead Audiologist
Tony Kay

Balance Physiotherapy Team

Nova Mullin
Cathy Morrow

There are specialists’ senior trainees working with the team that you may
encounter who are undergoing subspecialty training in this kind of surgery.

There is also a wider multidisciplinary team who are involved in your

treatment including radiologists, radiographers and therapists. Together they
work with other disciplines and ward staff to ensure that the highest quality of
care is delivered to you.

How can vestibular schwannomas be treated?

You are now at a stage where treatment options will be discussed with you.
There are three main ways of treating vestibular schwannomas. Your surgeon
will have discussed the findings on your MRI scan with a team of specialists at
our multidisciplinary team meeting.

Treatment for your vestibular schwannoma will depend upon many factors
including your age, overall health, symptoms, and size and growth rate of your
tumour as well as your symptoms and your personal preference.

The options available are:

Watch, Wait and Rescan

If your tumour is small we will almost always suggest no active treatment until
clear tumour growth is demonstrated. This means that we do not perform any
intervention and simply monitor the situation by repeating your MRI scan of
the head to see whether the tumour is growing or not. Therefore you will
undergo a period of observation known as ‘watch, wait and rescan’.
This has no potential side effects unlike the other treatment options but you
do require periodic scans to make sure that the tumour is not growing.

How often will I have a scan?

Patients undergoing observation of their vestibular schwannoma are usually

scanned:

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  Six months or a year after the original scan depending on size. If the
growth is stable:
 Scan yearly for three years
 Then scan every two years for the following six years
 Then scan every three years for the following six years
 Five yearly scans lifelong

If your tumour shows slight growth, you may continue to have scans every
year in case any intervention is needed.

An MRI scan is the best type of scan to use for monitoring these tumours.
However, occasionally a CT scan will be used instead if an MRI scan is not
possible, for instance if you have any magnetic metal work inside your body.
These scans are generally performed at Aintree Hospital so that your
specialist can look at the scans carefully themselves. The MRI scan takes
around 45 minutes and you may have an injection in your hand. The scan is
painless but it can be quite loud inside the scanner.

Once the scan is done, the specialist will arrange an outpatient appointment in
our Wednesday morning skull base clinic to discuss the findings with you.

It may seem like nothing is being done for you, however vestibular
schwannomas are benign and often do not pose any immediate risk to you,
the risks of surgery or radiotherapy may outweigh the benefits at this point.

The close monitoring would enable the team to reassess your options at any
given time. It is important that you inform the specialist nurse of any new or
worsening symptoms or concerns in between hospital visits.

What happens if my vestibular schwannoma grows?

If the scan shows there is growth, we will discuss with you and what your
treatment options are. These options include, continuing to watch and wait
and further scans in some cases or intervention by way of radiotherapy or
surgery in order to treat the tumour. These forms of treatment will be fully
discussed with you so that you can be informed about which type of treatment
would be best for you taking all factors for you as an individual into account.

Surgery

Surgery on vestibular schwannomas is an option, which aims to deal with this

condition by removing all, or as much of the tumour as is safely possible. The
operation is performed under a general anaesthetic and is a major operation.
It is not always possible to remove the entire tumour, because vestibular
schwannomas are often stuck onto the facial nerve or important parts of the
brain tissue. If this is the case, it is safer to leave a tiny amount of tumour
rather than risk permanent damage. After all, this is a benign, slow-growing
tumour, that you will have had for many years and a small remnant is unlikely
to cause you any problems in the future.

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 There are two main approaches for surgery. The choice depends upon the
tumour size, location and your hearing. The risks are similar and your
surgeon will discuss the best option for you.

It is rarely possible to save the hearing on the affected side. In patients who
have small tumours and very good hearing (such as can use the telephone),
an attempt can be made to preserve the remaining hearing on the affected
side.

With larger tumours and almost no socially useful hearing, it can be assumed
that hearing will be permanently lost on that side after surgery.

Who performs the surgery?

An ENT and neurosurgeon will perform your surgery jointly. The operation will
take place in the Walton Centre and you will be cared for on a neurosurgical
ward. The ward which you will be admitted to have highly experienced staff
that are familiar with the complex needs of patients following removal of a
vestibular schwannoma.

If surgery is decided as a treatment option a pre-operative appointment will be

made for you at The Walton Centre to discuss the process and recovery
period in more detail.

What are the risks of surgery?

The most significant risk from surgery is damage to the facial nerve. The risk
of this is directly related to the size of the vestibular schwannoma. Overall the
majority of patients have normal or near-normal facial movements after the
surgery. In a small number of patients the surgery may cause a temporary or
permanent damage to the facial nerve resulting in weakness/droopiness of
the face on the affected side. There is also risk of injury to the trigeminal
nerve which may lead to facial numbness/pain/altered sensation.

If you do have a facial weakness, it may affect your ability to close your eye,
make facial expressions and eating or drinking may be difficult with that side
of your face. This will often recover but it may take several months.

Following any type of surgery there is a risk in the immediate post-operative

period of infection (wound infection or meningitis), bleeding, hydrocephalus
(fluid on the brain) and external leakage of cerebro-spinal fluid (CSF leak). All
neurosurgery under anaesthetic carries a very small risk of death.

Your balance may be worse after surgery but generally this recovers with
time. You will experience worsening hearing/dead ear after surgery and if you
have tinnitus it may become louder. New onset tinnitus can also occur after
the operation. Other risks are very rare and include speech, swallowing and
breathing difficulty after surgery that may require a feeding or breathing tube
(tracheostomy) to be inserted.

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 Before undergoing surgery an opportunity will be provided to discuss the risks
with the surgeon who is due to perform the operation.

Most people who have surgery will need to stay in hospital for approximately
five to seven days.

We recommend that you have somebody at home when you are discharged
home as you will be tired and possibly dizzy for the first two weeks after
surgery. We advise that you gradually increase your levels of activity in order
to recover at a safe pace.

The nurse specialist will initially follow you up via the telephone. This takes
place approximately 1-2 weeks after discharge following surgery. You will
have an MRI scan around 8 -12 weeks after surgery and the consultant will
see you in clinic after the scan to monitor your recovery and arrange follow-
up.

Radiotherapy

Radiotherapy is increasingly used as a treatment for vestibular schwannoma.

It involves radiation targeted onto tumours. It works by stopping cells from
dividing and therefore stopping tumours growing. There are two ways of
carrying out radiotherapy: stereotactic radiosurgery and fractionated
radiotherapy.

Stereotactic radiosurgery

This is often referred to by people as gamma knife although the gamma knife
is only one type of machine which delivers stereotactic radiosurgery. There
are several different types of machine which deliver similar treatment, for
example the Cyber knife and Novalis. This treatment involves delivery of high
dose focused radiation onto the tumour in a single treatment.

To do this you are brought into hospital on the same day as the treatment.
The radiotherapy machine delivers a high dose of radiation to the tumour but
very little radiation to the surrounding normal brain. The aim is to stop the
tumour growing. The tumour is not removed. For most small tumours this is a
very effective treatment and in 95% of small tumours it is effective in stopping
the tumour from growing any further. There are risks including damage to the
facial nerve (causing weakness or paralysis of one side of the face), the
trigeminal nerve (causing numbness on one side of the face) and the hearing
and balance nerve (causing deafness on the side of the tumour and loss of
balance), but these risks are lower with this technique than with open surgery.
Therefore for small tumours we would usually advise stereotactic radiosurgery
in preference to surgery.

Fractionated radiotherapy

This is also a type of radiation treatment which tries to stop the tumour from
growing but it is delivered in more than one session. It is used in treatment of

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 large acoustic neuromas if surgery can not be performed, or in treatment of
small acoustic neuromas if preservation of hearing is very important, for
example in people who have no hearing on the opposite side.

If I have radiotherapy do I need follow-up after treatment?

Because the radiotherapy will not remove the tumour, we would recommend a
follow-up schedule that includes MRI scans in order to check that the
vestibular schwannomas do not show evidence of further growth. An
outpatient appointment will be made following your scheduled MRI scans to
discuss the findings with you. You are not likely to need any further treatment
for your vestibular schwannoma if it stops growing and should be able to
return to a normal daily routine.

What happens after my treatment?

Aftercare of patients with a vestibular schwannoma depends largely on the

treatment given. You will be given the number for the specialist nurse in case
of any questions or worries you may have.

Other follow-up while under observation, or after surgery or

radiotherapy

Depending on your symptoms you may also need the input from other
disciplines such as nurse specialist, speech and language therapists,
dieticians, vestibular (balance) physiotherapists, audiology and
ophthalmology.

We are able to offer other services that you may require after diagnosis or
treatment for a vestibular schwannoma.

Nurse specialist led clinic

If your condition remains stable and your consultant feels it is appropriate then
you may be followed up by our nurse specialist team. However if your
condition changes you will be referred back to your consultant.

Hearing Tests

We may offer you hearing tests called PTA (pure tone audiogram) and
Speech Audiometry on your arrival at the skull base clinic prior to seeing you
in your outpatient appointment. This enables us to see how the tumour is
affecting your hearing, and how you are able to communicate with other
people.

Hearing Aids

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 If we feel you would benefit from a hearing aid, we will refer you to your local
audiologists to be assessed and discuss the options available to you. You
may also wish to discuss this with your GP and they can also refer you to your
local audiologist.

If your hearing has been affected by the vestibular schwannoma and you are
also hard of hearing on the side with no tumour, then it may be worth
considering a lip reading class to gain additional skills and confidence with
communication. Information on support groups are listed at the end of this
document.

Tinnitus and Balance Problems

Tinnitus, a ringing, buzzing or wooshing noise can be quite troublesome and

often becomes worse after any form of intervention. There are a number of
effective therapies, which can help with management of tinnitus.

For those patients with balance disturbance or dizziness we can arrange a

vestibular assessment by a specialist physiotherapist and teach you exercises
to retrain the balance system and provide strategies for dealing with difficult
environments.

Who to contact?

Many questions can arise after diagnosis of a vestibular schwannoma. We

advise that if you have any questions, no matter how small, or if you have any
changes in your symptoms to contact the clinical nurse specialist.

Contact details:

Consultant seen in clinic: ___________________________

Clinical Nurse Specialist: ___________________________

Telephone: 0151 525 5648

Bleep via the switchboard 0151 529 3611 bleep 5342 / 5391

E-mail: tumournurse@thewaltoncentre.nhs.uk

Alternatively, please call:

Carol Taylor, secretary to ENT surgeons on:

Telephone: 0151 529 3404

Fax: 0151 529 5263

E-mail: carol.taylor@aintree.nhs.uk

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 Other useful support groups and websites are:

British Acoustic Neuroma Association

The British Acoustic Neuroma Association (BANA) was formed in 1992. BANA
is organized and administered by people affected by acoustic neuroma. It is a
registered charity and exists for mutual support, information exchange and
listening.

The British Acoustic Neuroma association

Oak House B, Ransom Wood Business Park
Southwell Road west
Mansfield
Nottinghamshire
NG21 0HJ

Tel: 01623 632143

Fax: 01623635313

Website: http://www.bana-uk.com

The Neuro Foundation

The Neuro Foundation is a UK charity that aims to improve the lives of people
with neurofibromatosis (either type 1 or type 2).

For more information, you can visit The Neuro Foundation website, call its
helpline on 020 8439 1234, or email info@nfauk.org

British Tinnitus Association

The British Tinnitus Association

Ground Floor, Unit 5
Acorn Business Park, Woodseats Close
Sheffield, S8 0TB

Tel: 0800 018 527

Website: info@tinnitus.org.uk

Action on Hearing Loss

Information Line:
Telephone 0808 808 0123
Text phone 0808 808 9000

Email: informationline@hearingloss.org.uk

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 The Brain Charity

The Brain Charity provides support that complements medical provision, to

people with a neurological condition, their families and professionals who care
for them throughout their journey.

Norton Street
Liverpool
England
L3 8LR

Tel: 0151 298 2999

Website: www.thebraincharity.org.uk

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