Schwannomas & Vestibular Schwannomas

What are Schwannomas?

Nerves are similar to electrical cables - the inner 'wire' transmits the signals and the outer sheath protects them. A schwannoma is a benign (non-cancerous) tumour that arises from the cells that insulate nerves.  They can develop on any nerve in the body that is 'insulated'.

​

I care for many patients with schwannomas of the brain and spinal nerves. One of the more common nerves for these to grow on is the vestibular nerve (nerve for balance) and as such I will focus the information to follow on these. However the principles of management outlined for vestibular schwannomas also applies to other schwannomas and therefore you may find the information helpful if you have been diagnosed with a schwannoma that is not located on the vestibular nerve.

​

Vestibular Schwannomas

A vestibular schwannoma is a benign (non-cancerous) tumour that arises from the cells that insulate the vestibular nerve as it travels between your inner ear and your brain.  It is also known as an acoustic neuroma.  The vestibular nerve is the nerve of balance, and it travels together with the hearing nerve, the cochlear nerve. Together they are called the vestibulo-cochlear nerve.

​

How common is a vestibular schwannoma? 

Every year, approximately 20 people out of every million in the population are diagnosed with one. They are slightly more common in women than men, and in those aged between 30 and 60 years. 

What causes a vestibular schwannoma? 

 It is not fully understood what causes the majority of vestibular schwannomas. In approximately 95% of people the cause is unknown and they are described as sporadic. In a small minority of patients they are due to a genetic condition called Neurofibromatosis Type II (NF2). However this is not common, and your vestibular schwannoma will be a sporadic tumour unless your Doctor has mentioned that you may have this genetic disorder.

What are the symptoms? 

Most commonly symptoms start with gradual hearing loss on one side. This is thought to be due to the tumour affecting the hearing (cochlear) nerve which runs together with the vestibular nerve. Hearing loss often occurs even if there is no growth in the tumour. This means that worsening hearing does not necessarily mean your tumour has grown. Other common symptoms are tinnitus, a sensation of ringing in your ear, and dizziness/vertigo. Tinnitus affects approximately 70% of patients with vestibular schwannoma. Dizziness/vertigo affects approximately half of people with vestibular schwannomas. Balance can be affected due to compression of the balance nerve by the tumour, but as vestibular schwannomas tend to grow slowly your body can often adapt and compensate for the change such that some people do not notice any change in their balance. 

Other less common symptoms, which tend to occur with larger tumours include:

• numbness or pain on one side of the face (some vestibular schwannomas can affect the nerve that supplies sensation to the face (trigeminal nerve) by putting pressure on it.

• problems with limb coordination on one side of the body

• weakness on one side of the face  

• headaches with blurred vision

• in rare cases, changes to the voice or difficulty swallowing

​

How is the diagnosis made?

Typically by performing MRI (magnetic resonance imaging) of the brain. If you have hearing loss often you will have a hearing test alongside the MRI scan. Once vestibular schwannomas are diagnosed you should be referred to a specialist team who are experienced in managing these tumours. 

Help with symptoms from vestibular schwannoma

As outlined on the previous sections common symptoms from vestibular schwannomas include hearing loss, tinnitus and dizziness/balance issues. Regardless of the treatment that you need for your tumour there are ways you can be helped with these. Often treatment of the tumour, if required, does not in itself help with these symptoms either, and the below help is required after treatment too. 

​

Hearing loss

If you have hearing loss on the side of your vestibular schwannoma the first step is to have a hearing test, also known as audiology tests. Your Doctor will be able to arrange for these to be done. This will establish your current level of hearing and is crucial information to determine if you would be helped from an appointment with an audiologist who will be able to discuss the available options with you. These include hearing aids and CROS hearing devices. It is common for hearing to decline over time in people with vestibular schwannomas, even if the tumour itself does not grow. Active treatments such as surgery and radiosurgery do not restore hearing, in fact they often result in worsened hearing. If you experience sudden hearing loss (please note this is uncommon) it is important that you contact your Doctor as soon as possible as it may be helped with a short course of steroid medications.

​

Tinnitus

This is very common even in people without vestibular schwannomas. For those with vestibular schwannomas it can persist despite active treatment (it is not an indication for active treatment because of this). Many people are able to function and sleep normally alongside having tinnitus but for some it can be so intrusive it affects everyday life and sleep. If the later is the case for you you should speak to your Doctor as you may benefit from referral to a specialist tinnitus clinic to discuss aids to help you manage better with your tinnitus.

​

Dizziness/balance problems

Again this is quite common for people with vestibular schwannomas and can persist or even worsen after active treatment if that is needed for tumour size or growth (as for tinnitus dizziness/balance issues alone are therefore not an indication for active treatment). These problems can be helped however, and I have looked after many people with both monitored and actively treated tumours who have had significant improvements/'normalisation' of their balance with some directed help and work. Please ask about referral to a specialist vestibular physiotherapist if you are struggling. In the interim you may find the resources on the following webpage helpful: https://www.generationgames.org.uk/ This website offers a wealth of information about exercise and includes videos by physiotherapists guiding you through home exercises. You may also want to consider taking Yoga classes as this helps many people with their core strength and balance.

Treatment of Vestibular Schwannomas

Although a new diagnosis of vestibular schwannoma can feel daunting, many of these are small and at least 50% may not require any intervention. Recommended treatments vary according to a wide variety of factors such as the size and position of the tumour, its growth rate, symptoms, and overall health. 

Available treatment options are: 

Active Surveillance

For small tumours this is often the recommended option and with this approach many people do not go on to require treatment. Typically patients under active surveillance will be monitored with scans for up to 15 years to check for any growth, with yearly scans for the first 5 years and becoming spaced more after that if there are no signs of growth.  

​

Specialist Radiotherapy/Radio-surgery

This is an effective technique that uses highly targeted doses of radiation with the aim of stopping the tumour from growing any more. Growth control rates with this form of treatment are up to 95%. This is usually offered to patients with small but growing tumours, as an alternative to surgery. This can be delivered in one dose (radiosurgery) or several doses (fractionated radiotherapy). What type is offered, and whether this is a safe and appropriate option, depends on the size and shape of your tumour. Sometimes it is used as an adjunct to surgery, for example if a remnant is left at the time of surgery to protect the facial nerve, but there are signs of growth in that remnant after surgery, radiosurgery or fractionated radiotherapy is often then given. 

​

Surgery

Surgery may be indicated for a wide range of reasons. Typically surgery is considered for patients with larger tumours and young patients with growing tumours. It is a widely offered treatment option, which aims to remove the tumour completely or significantly reduce its size. Surgery cannot however restore function in nerves that have already been affected by the tumour, for example your hearing. 

Surgery

If you are considering having an operation you may find this information helpful.  

Typically surgery is considered in patients with larger tumours that are seen to be growing or that are large on diagnosis in a way that is concerning for their health left untreated. It is a widely offered treatment option, which aims to remove as much tumour as safely possible to reduce pressure on the brain. Surgery cannot restore function in nerves that have already been damaged by the tumour, for example restore hearing.  It is uncommon to be able to preserve hearing with surgery and is usually only achievable in small tumours where there is good hearing before surgery.   Often the tumour is very adherent to the facial nerve and/or brainstem surface. In those situations it is sometimes safer to leave some tumour to preserve the function of those important structures than risk long term damage to them. Small amounts of tumour not removed (remnant) are unlikely to cause any problems in the future. The risk of needing further treatment from a small remnant of tumour is approximately 5% in the long term. If a larger remnant has to be left to preserve important structures, treatment with stereotactic radiosurgery at a later date may be required.  After surgery you will have follow up scans for up to 15 years.  

​

There are three main surgical approaches. Which is chosen depends on tumour size, shape, position and hearing levels. As outlined above it is uncommon to be able to preserve hearing with surgery and is usually only achievable in small tumours where there is good hearing before surgery.   Attempting to preserve hearing may lead to a larger tumour remnant being left and even if hearing is preserved immediately after surgery, it may still deteriorate in the future.  

​

The approaches will be discussed in detail with you by your surgeon before your operation. Each approach involves a general anaesthetic, an operation usually by both a Neurosurgeon and an ENT surgeon, and an incision on the affected side of the head, in slightly different places depending on the chosen surgical approach but always located behind the ear. 

Surgery is a routinely used option for managing vestibular schwannomas but does carry risks. 

​

Facial weakness:

This is one of the most significant risks from surgery. The facial nerve (the nerve that supplies muscles of facial expression) and the vestibulocochlear nerve (hearing and balance nerve) run very closely together. Due to their position, the facial nerve is always attached to the surface of the tumour and is therefore at risk of damage during tumour removal. This risk is higher in larger tumours. During surgery the nerve is specifically monitored to try and reduce the risk of injury.

If you do have facial weakness it can affect your ability to close your eye, necessitating eye drops and sometimes patches to protect the eye in the short term. It can also affect your ability to make facial expressions and the way you eat and drink with that side of your face. 

​

In the immediate and short-term period after the operation a higher number of patients do have facial weakness due to the manipulation involved in removing the tumour. In the long term (several months after surgery) most patients have normal or near-normal facial movement, as the nerve recovers from this. You will be given a more accurate idea of the risks of surgery to your facial nerve for your specific tumour by your surgeon. 

​

Hearing loss: 

Most vestibular schwannomas are diagnosed after the patient experiences a loss of hearing, which can be partial or total. Following surgery, the majority of patients will lose their hearing completely in the affected ear. Prior to an operation you would have hearing tests. With this information and your scans you will be advised as to whether an attempt can be made to preserve your remaining hearing in that ear. Hearing rehabilitation options can be discussed again after surgery, there are options whatever your level of hearing on that side to help.

​

Tinnitus: 

Some patients experience tinnitus (hearing a noise such as a high pitch noise or ringing sound) in the affected ear. Tinnitus usually occurs together with hearing loss which means tinnitus can be present even when you have no hearing in the affected ear after surgery. Even if you had no tinnitus before the operation, it may develop afterwards. For most patients tinnitus becomes less intrusive over time, however, if it persists support can be provided for managing this symptom.  

Facial numbness: 

Often patients with large tumours have numbness or pain in their face before their operation, due to compression of the trigeminal nerve which supplies sensation to your face. For those larger tumours this may not recover, and in some patients due to the adherence of the nerve to the tumour it can worsen. Again the specific risks of this happening will be discussed with you before your operation. If your trigeminal nerve is impaired and if grit or dirt gets into your eye you might not be able to feel it, which can lead to damage and later infection. If you have facial numbness, particularly in combination with facial weakness, you will need to take particular care to ensure that your eye is protected. Speak to your surgeon for advice on this if you are concerned. 

Damage to other cranial nerves:

Sometimes surgery can be complicated by damage to other cranial nerves. Other nerves that can be affected are those that control eye movements, leading to double vision, or the nerves involved in swallowing and speech. These are rare. If they do occur you will be supported by the appropriate therapist. 

​

​

Cerebral spinal fluid (CSF) leak: 

CSF bathes the brain in fluid. When the tumour is removed, the cerebro-spinal fluid pathways around the brain are opened. CSF can leak out either through the entry wound or into the ear and then down the nose. The risk of this leakage is around 4%. If a drainage tube is placed temporarily in the spinal fluid pathways in your back, the majority of leaks will settle down, but around one in three leaks will require a second operation to repair them.

​

Hydrocephalus:

Hydrocephalus is a build up of the CSF on the brain which can occur before or after surgery as a consequence of the tumour or its treatment.  If this occurs you may require a temporary or permanent drain (shunt) placed into the brain.

Infection: 

There is a risk of infection which can be of the skin/wound, or less commonly brain fluid (meningitis).   The majority of infections can be treated satisfactorily with antibiotics, sometimes surgery to wash the infected area is required (approximately 2% risk). 

​

Problems with balance: 

Patients often experience dizziness/balance problems immediately following surgery.  This usually improves over days to weeks.  Persisting balance problems can be helped with dedicated balance physiotherapy. 

​

Headache and neck pain:

It is common to experience headache and neck pain after operations on the head, particularly for the first few days. This will be controlled with painkilling medication and improves with time. 

Stroke/major neurological impairment: 

There is a very small risk (less than 1%) of significant bleeding or injury at the site of surgery which can result in loss of life or stroke with significant disability including paralysis.  

​

General Anaesthetic Risks:

As with any long operation there is a risk of developing a clot in the leg or lung (pulmonary embolus), a chest infection or heart problems.

What's it like to have surgery to a vestibular schwannoma?

For general advice please see the section on 'What is it like to have a Neurosurgery operation?' and the sections on Diet, Exercise and Psychology. You may also find the section on 'Surgical wound' helpful to read. 

​

Before your operation, in addition to meeting your surgical team, you will undergo hearing tests if you have not already had them and have a pre-operative assessment. 

You usually come into hospital the night before your operation or the morning of it. The operations usually takes all day, and you can expect to be in hospital for 5-7 days after the operation. After your operation you may be kept overnight in the intensive care unit for close monitoring before returning to the ward. You will be cared for by a team of staff who are very experienced in looking after patients recovering from this type of surgery. You may also require input from other health professionals such as physiotherapists, speech and language therapists, dieticians, pharmacists and ophthalmologists. You will be encouraged to get up and about as soon as you can from your operation to prevent complications from lying in bed and to help with recovery of your balance. Often people initially have generalised headaches that can be worse initially when they sit/get up, unsteadiness, double vision and general fatigue. All of these symptoms improve over the first week or so from surgery and your team will help you with these symptoms as you need. 

If you have a facial weakness after your operation you may be seen by an ophthalmology team while you are in hospital and a facial palsy team who will then continue to support you when you have been discharged. The same applies for the physiotherapy team and any other members the team that may be of help to you in your recovery in hospital, and on discharge home. 

It is best to try to have someone at home when you are discharged as you will be tired and possibly unsteady for a few weeks after your operation. Having the additional support will help. Recovery from this operation is slow and steady. It will take approximately six to twelve weeks.  Some people may take longer. Tiredness is common, and you will find immediately after your operation that you feel exhausted even after commonplace activities like getting washed and dressed, going to the shops, watching television, or talking with friends. Please refer to the 'Diet' and 'Exercise' sections on this webpage for advice on how to optimise your recovery. 

​

Driving

If you drive you can continue to do so after your operation and you do not need to notify the DVLA with this type of tumour. We would advise a period of recovery, normally 4 weeks, prior to returning to driving. If you develop severe giddiness or other complications such as hydrocephalus this may change so consult your Doctor or specialist nurse for further advice.

Return to work

If you work, return to work will depend on what sort of work you do and when you feel able. Often people consider returning to work from 6 weeks from their operation but some people will be require longer. It is important not to rush this as returning to work too early may prolong your recovery. If you are unsure we advise consulting your GP, surgeon, and/or work Occupational Health team. 

​

Travel

We advise that you can fly 2 months after your surgery provided of course you feel well enough to do so at that point. You should inform your insurance company of your surgery before you travel. 

​

Follow-up

Typically you will have follow-up with your surgical team within the first few weeks after returning from home. The timing of your follow-up scans will depend on how much tumour has been removed. Your surgeon will discuss this with you after your operation and at follow-up. Follow-up scans will be arranged for a minimum of 10 years following surgery to check for any re-growth. 

​