Ménière’s disease

Ménière's disease is a rare disorder that affects the inner ear.

Introduction

Ménière's disease is a rare disorder that affects the inner ear. It can cause vertigo, tinnitus, hearing loss, and a feeling of pressure deep inside the ear.

The symptoms of Ménière's disease usually appear as attacks without warning, often lasting around two to three hours. The symptoms may take one to two days to disappear completely.

It's worth noting, however, that the symptoms and severity of Ménière's disease vary widely from person to person.

Ménière's disease is thought to be caused by a problem with the pressure deep inside the ear. However, this has not yet been proven (see causes of Ménière's disease for more information).

How the disease progresses

Ménière's disease progresses through different stages. In the early stages, some people may have 6-11 attacks a year.

During the late stage of Ménière's disease, the episodes of vertigo tend to occur less frequently and sometimes stop altogether within 5 to 10 years. However, you may be left with balance problems, and you may be unsteady on your feet, particularly in the dark.

For many people, hearing loss (which can affect one or both ears) and/or tinnitus get progressively worse.

Who is affected?

Ménière's disease can occur at any age, but it most commonly affects people aged 40-60 years. Ménière's disease is slightly more common in women than in men. Sometimes there may be a family history of the disease.

Treating the symptoms

A variety of medications are available to treat Ménière's disease which allow most people with the disease to continue with their normal daily activities. There are also a range of techniques and therapies that can help with the tinnitus and balance problems. Read more about the treatment of Ménière's disease.

Support

People with Ménière's disease may find the condition has a significant impact on their emotional health. The unpredictable attacks can cause anxiety and the hearing loss may lead to depression. There are a number of support groups, such as the Meniere's Society, that can provide assistance and advice (see the 'Useful links', right).

Vertigo is the sensation that you or the environment around you is moving or spinning.

Tinnitus is the medical name for the perception of noise in one ear, both ears or in the head. The noise comes from inside the body rather than an outside source.

Symptoms

The symptoms of Ménière's disease vary from person to person and can occur without warning.

The main symptoms are:

  • vertigo and other balance problems – vertigo is the sensation that you, or the environment around you, is moving or spinning
  • tinnitus – the perception of noise in one ear, both ears, or in your head, where the noise comes from inside the body rather than from an outside source
  • hearing loss – this can affect one or both ears; many people struggle to hear low sounds in particular
  • a sense of pressure or fullness deep inside the ear

Vertigo and tinnitus are described in more detail below.

Vertigo

Vertigo is one the most common and noticeable symptoms of Ménière's disease.

A vertigo attack can last for just a few minutes, but it will often last for two to three hours. It is difficult to predict exactly when an attack will take place, so keep any vertigo medication that you have with you at all times. Read more about vertigo.

In addition to vertigo, you may also feel dizzy, sick and off-balance. You may have difficulty standing or walking. Occasionally, you may have 'drop attacks' (falling to the ground suddenly and unexpectedly).

During a severe attack of vertigo or dizziness, you may experience sweating, diarrhoea and/or palpitations (pronounced heartbeats).

Tinnitus

Tinnitus is usually more noticeable when you are tired or when it is quiet, as there is less background noise to distract you from the sounds that come from inside your body.

The sounds that you hear could be:

  • ringing
  • buzzing
  • humming
  • whistling
  • music

Read more about tinnitus.

Emotional impact

Ménière's disease can often have a significant impact on a person's day-to-day activities. The condition can sometimes affect work and family life. For example:

  • you may need to change your job if it previously involved using ladders or scaffolding, or operating machinery
  • you may need to stop driving, which could limit your social contact
  • the hearing loss can make it difficult to interact with others, both in the workplace and at home
  • you may find it difficult to sleep as a result of the tinnitus

These changes may leave you feeling stressed,anxious, or depressed. You should speak to your doctor if you are finding it difficult to come to terms with the effect that Ménière's disease is having on your life. They will be able to offer advice and support. See Ménière's disease – treatment and the 'Useful links' for more information.

Stages of Ménière's disease

Ménière's disease is categorised into three stages:

  • early stage
  • middle stage
  • late stage

The progression of Ménière's disease varies between individuals, and you may not necessarily pass through each of these stages. In general, people experience more attacks during the first few years, and then as the attacks decrease in frequency, the hearing loss becomes progressively worse.

The stages of Ménière's disease are described in more detail below.

Early stage

The early stage of Ménière's disease consists of sudden and unpredictable attacks of vertigo. These are usually accompanied by nausea, vomiting and dizziness. You may lose some hearing during the attack, and there is often tinnitus at the same time. Your ear may also feel blocked and uncomfortable, with a sense of fullness.

Attacks of vertigo at this stage can last from 20 minutes to 24 hours, but usually last around two to three hours. The length of time between attacks will also vary. It is estimated that most people experience 6-11 attacks a year. Your hearing and the full sensation in your ear will usually return to normal between attacks.

Middle stage

The middle stage of Ménière's disease consists of continuing attacks of vertigo, with the attacks becoming less severe. Tinnitus and hearing loss often become worse.

During the middle stage, you may experience some periods of remission (where your symptoms go away), which can last for up to several months.

Late stage

During the late stage of Ménière's disease, the episodes of vertigo occur far less frequently and may stop altogether. However, you may be left with balance problems, and you may be unsteady on your feet, particularly in the dark.

Your hearing problems and tinnitus may become progressively worse during the late stage of Ménière's disease. The longer the condition lasts, the more likely it is to affect both ears. Around 40% of people may find that both ears are affected.

Vertigo is the sensation that you or the environment around you is moving or spinning.

Tinnitus is the medical name for the perception of noise in one ear, both ears or in the head. The noise comes from inside the brain rather than an outside source.

Causes

The cause of Ménière's disease is not yet known, although it is thought to be caused by a problem with the pressure in the inner ear.

The inner ear is made up of:

  • the cochlea – a coiled, spiral tube that contains two fluid-filled chambers and is responsible for hearing
  • the vestibular apparatus – a complex set of tubes that help to control balance

The fluid inside the inner ear is called endolymph.

If the pressure of the endolymph fluid changes – for example, because there is too much fluid – it can result in symptoms such as vertigo and tinnitus. It is therefore thought that this pressure change is responsible for Ménière's disease, although this has not yet been proven.

In most cases, the cause of the fluid pressure change is unknown, but there are a number of possible risk factors.

Possible risk factors

The following factors may increase the risk of pressure changes deep inside the ear:

  • An allergy to a substance, such as a food.
  • Autoimmuity – when your immune system starts producing antibodies that attack your own tissues and organs.
  • Genetic (inherited) factors – for example, if there is a family history of the condition.
  • A chemical imbalance in the fluid in your inner ear, as a result of too little or too much sodium or potassium in your body.
  • A problem with the blood vessels – there is a link between Ménière's disease and migraines, which are thought to be caused by the narrowing and widening of blood vessels.
  • Some viral infections such as meningitis (an infection of the membranes that surround the brain and spinal cord).

Vertigo is the sensation that you or the environment around you is moving or spinning.

Tinnitus is the medical name for the perception of noise in one ear, both ears or in the head. The noise comes from inside the body rather than an outside source.

Allergy is the term used to describe an adverse (bad) reaction that the body has to a particular substance.

The immune system is the body's defence system, which helps protect it from disease, bacteria and viruses.

Genetic is a term that refers to genes. Genes are the characteristics inherited from a family member.

Blood vessels are the tubes in which blood travels to and from parts of the body. The three main types of blood vessels are veins, arteries and capillaries.

Diagnosis

You should visit your doctor if you experience any of the symptoms of Ménière's disease.

There is no single test for Ménière's disease, but your doctor will ask about your symptoms and carry out a physical examination.

Ménière's disease can be difficult to distinguish from other diseases because there are several conditions that can also cause vertigo and hearing problems.

For example, migraines and ear infections can also affect your balance and hearing. A viral infection of the balance nerve (vestibular neuronitis) or the inner ear (labyrinthitis) can also produce similar vertigo attacks. However, in these conditions the hearing is not affected.

Ménière's disease may only become evident after a period of time, as a pattern of reoccurring attacks develops. Your doctor will ask you to describe your symptoms to find out if a pattern is emerging.

How a diagnosis is reached

For Ménière's disease to be diagnosed, you will need to have the following symptoms:

  • vertigo (a feeling that the environment around you is spinning) – at least two episodes of vertigo lasting 20 minutes or more within a single Ménière's disease attack
  • hearing loss – tests must confirm that hearing loss is caused by damage to the sensitive hair cells inside the cochlea (the coiled tube in the inner ear)
  • tinnitus (perception of noise in your head) or a feeling of pressure in your ear

Note: to be diagnosed with Ménière's disease, you must have vertigo and hearing loss, and either tinnitus or a feeling of fullness in your ear.

Physical examination

Your doctor will carry out a general physical examination to rule out other possible causes of your symptoms. This may involve:

  • listening to your heartbeat
  • checking your blood pressure
  • examining the inside of your ears

If your doctor suspects that something else is causing your symptoms, you may be referred for further tests.

If it is thought that you may have Ménière's disease, you will probably be referred to an ear nose and throat (ENT) specialist to test your hearing. This will usually be carried out at the ENT department of your local hospital.

A patient can also be referred to a specialist in audiovestibular medicine for heaing and balance assessment, although this service may not be available in every hospital.

Audiometry and other tests

The ENT or audiovestibular medicine specialist will be able to assess the extent of your hearing loss and confirm a diagnosis of Ménière's disease. Hearing loss that is caused by Ménière's disease can be investigated using an audiometry test.

An audiometry test is carried out using a machine that produces sounds of different volume and pitch. You will listen to the sounds through headphones and will be asked to signal when you hear a sound, either by raising your hand or pressing a button.

If your Ménière's disease is in the early stage, an audiometry test will not always demonstrate hearing loss because hearing loss in this stage is often temporary.

Other diagnostic tests the specialist may use are:

  • otoacoustic emission test – to establish the degree of damage to the hearing cells of the inner ear
  • balance tests – to assess the damage to the balance system
  • electrocochleography – to measure the electrical force generated in your inner ear and nerve in response to stimulation by sound

Vertigo is the sensation that you or the environment around you is moving or spinning. s Tinnitus is the medical name for the perception of noise in one ear, both ears or in the head. The noise comes from inside the body rather than an outside source.

Treatment

There is no single treatment for Ménière's disease, mainly because the exact cause is still unknown.

However, your ear, nose and throat (ENT) specialist and your doctor will be able to help you manage your symptoms using a variety of techniques. Possible treatments include:

Your doctor and ENT specialist will provide you with advice and information tailored to your individual needs, and will develop a management plan to enable you to cope more effectively with your symptoms. In some cases, the treatment available may depend on what your local primary care trust can provide.

Treatment during an attack

During an attack of Ménière's disease, you may be prescribed medication for 7-14 days to treat the symptoms of:

  • vertigo
  • nausea (feeling sick)
  • vomiting

The medications usually prescribed are prochlorperazine or an antihistamine.

If these medicines are successful in treating your symptoms, you may be given a supply to keep at home so that you can take them the next time you have an attack.

Prochlorperazine

Prochlorperazine can cause side effects including:

  • tremors (shaking)
  • involuntary body and facial movements

It can also make some people feel sleepy. Check the patient information leaflet that comes with your medicine for the full list of possible side effects.

If you are vomiting, you can take a type of prochlorperazine called Buccastem. This comes as a tablet that you place between your gums and your cheek on the inside of your mouth. The tablet dissolves and is absorbed into you body.

Antihistamines

Possible antihistamines include:

  • cinnarizine
  • cyclizine
  • promethazine teoclate

Antihistamines can make you feel sleepy. Headaches and an upset stomach are also possible side effects. Check the patient information leaflet that comes with your medicine for the full list of possible side effects.

Serious attacks

If you suffer severe vertigo, dizziness, nausea and vomiting during an attack of Ménière's disease, your doctor may inject you with prochlorperazine.

If you suffer severe vertigo with or without nausea during an attack, your doctor may also prescribe you a tablet of Buccastem that dissolves very quickly in your mouth (see above).

In very severe cases, you may need to be admitted to hospital to receive intravenous fluids (through a vein) to keep you hydrated.

Preventing attacks

Your doctor can prescribe a medication called betahistine to help reduce the frequency and severity of attacks of Ménière's disease, or you may be advised to change your diet.

Special diets

Although it has not been proven, following a diet without added salt does seem to help control Ménière's disease. It is possible that this type of diet might alter the fluids in your inner ear. Avoiding excessive caffeine, found in drinks such as tea and coffee, is also usually recommended.

If you have migraines, avoiding possible migraine triggers may prove beneficial. Known migraine triggers include chocolate, nuts and some red wines.

Betahistine

It is thought that betahistine reduces the pressure of the fluid in your inner ear, relieving the symptoms of Ménière's disease. Research that has been carried out into betahistines has not found enough evidence to confirm whether they are effective. However, your doctor may recommend trying them.

Betahistines are usually available as tablets to be taken three times a day. Your doctor will discuss how long you need to take them for, which could be a few weeks, or up to a year. Possible side effects include a headache, upset stomach and a skin rash.

Read more about betahistine.

Treating tinnitus

There are a number of different treatment options that can be used to treat tinnitus caused by Ménière's disease. Some possibilities are described below.

  • Sound therapy – the effects of tinnitus are often more pronounced in quiet environments. Sound therapy works by reducing the difference between the tinnitus sounds and the background sounds. This makes the tinnitus sounds less intrusive.
  • Relaxation techniques – tinnitus can be a distressing and intrusive condition. Relaxing by practising yoga or special breathing techniques may help you to avoid stress and anxiety.
  • Cognitive behavioural therapy (CBT) – this is a therapy based on the knowledge that severe Ménière's disease symptoms (especially tinnitus and vertigo) have a significant psychological effect, resulting in negative thoughts and avoidance behaviour, which in turn aggravates your symptoms and creates a vicious circle. This treatment is given by a specially trained professional, who aims to change the way you think and behave, which would allow you to cope better with your symptoms and engage more effectively in the treatment.

Your doctor can refer you to a hearing therapist to discuss these techniques in more detail. Read about the treatment of tinnitus for more information.

Treating hearing loss

If your Ménière's disease is in the middle or end stages, you may experience some permanent hearing loss. Ménière's disease tends to make you more sensitive to loud sounds, and also makes it more difficult for you to distinguish low-pitched sounds.

Read more about the treatment of hearing impairment.

Physiotherapy

A physiotherapist can help improve your balance by teaching you vestibular rehabilitation techniques. These exercises teach you how to cope with the abnormal and disorientating signals coming from your inner ear. You are taught to use alternative signals from your eyes, ankles, legs and neck, to keep you balanced.

Physiotherapy is usually used in the middle or late stages of Ménière's disease, after the severe attacks of vertigo have stopped but when balance problems may remain. Your doctor will be able to refer you to a physiotherapist if they feel it would benefit you.

Read more about physiotherapy.

Surgery

In severe cases, surgery can be performed to control the attacks of vertigo. However, surgical procedures are usually only used if other treatments have failed. About 10% of people with Ménière’s disease will require surgery.

The type of surgery you have will depend on your symptoms, and whether both ears are affected. You could have:

  • non-destructive surgery
  • selectively destructive surgery
  • destructive surgery

Non-destructive surgery

Non-destructive surgery may be used if your hearing in the affected ear is 'socially adequate' (you can hear sounds that are below 50 decibels). This type of surgery aims to change the progression of Ménière's disease by reducing the severity and frequency of your symptoms.

Non-destructive surgery could be:

  • endolymphatic sac decompression – this reduces the pressure in your inner ear by increasing the drainage of the fluid of your inner ear
  • inserting ventilation tubes – these are inserted into your ear to reduce the changes in pressure that cause Ménière's disease
  • injecting steroid medication through the eardrum – this is a new type of treatment that has not yet been proven to be beneficial

Selectively destructive surgery

In selectively destructive surgery, the balance part of the inner ear is destroyed with a medicine called gentamicin. This is injected through the ear drum (the thin layer of tissue that separates the outer ear from the middle ear) and enters the labyrinth (the system of tubes in the inner ear).

Gentamicin should mainly cause damage to the balance part of your ear. However, it does carry a risk of damaging your hearing too.

Some surgeons prefer to apply the gentamicin directly to the inner ear during a minor operation. This means they can control the exact dose of gentamicin that enters your ear.

Destructive surgery

Destructive surgery may be considered if only one ear is affected by Ménière's disease. The hearing in the affected ear must be considered to be 'socially inadequate' (you cannot hear enough to function in social situations). As an approximate guide, if you cannot hear sounds that are below 50 decibels, this may count as socially inadequate.

Destructive surgery is used to destroy the part of your inner ear that is causing your vertigo attacks. Therefore, this type of surgery will only be considered if the hearing loss from the affected ear is permanent.

The destructive surgery could be done by:

  • destroying the balance part of your audio-vestibular nerve (the nerve that transmits sounds and balance information to the brain), or
  • destroying part of your vestibular labyrinth (the system of tiny, fluid filled channels in the ear) – this procedure is called a labyrinthectomy

After the surgery, your other ear will take over your hearing and balance functions.

Results

There are very few clinical trials (medical tests) looking into the effect of surgery on Ménière's disease, which is why surgery is rarely considered. If your ENT specialist thinks that you may benefit from having surgery, they will discuss the procedure and any risks in more detail with you.

Support groups and charities

There are several support groups and charities that can provide you with useful information and advice about living with Ménière's disease. They can also put you in touch with other people who have the condition so you can share experiences and provide support to one another.

Vertigo is the sensation that you or the environment around you is moving or spinning.

Tinnitus is the medical name for the perception of noise in one ear, both ears or in the head. The noise comes from inside the body rather than an outside source.

Real stories

Jan, 30, was diagnosed with Ménière’s disease a few years ago and lives with hearing loss and tinnitus. Thankfully, the high-pitched ringing in her ears doesn’t bother her that much because she's had time to get used to it. She tells her story.

“It wasn't long after I’d moved into a new, much quieter house in Edinburgh when I noticed a strange noise in my ear, a bit like a radio transmitter. It started off fairly quiet but then gradually got more noticeable. I was 27.

“It worried me because I’d also been hearing heartbeat-like noises in my other ear. I'd had that on and off since I was small. I went to my doctor to get it checked out and was referred to an ear, nose and throat specialist. After some hearing tests I was diagnosed with Ménière’s disease, with symptoms of tinnitus and hearing loss.

“I thought, ‘Surely I’m too young to get tinnitus?’, and remember wondering how I was going to cope with this ringing in my ears for the rest of my life. It was really upsetting, as I was told there was no cure.

“During this time I had an attack of vertigo on holiday, which lasted for three days. It felt like the ground was constantly moving beneath me, like being on a ship. It didn’t make me physically sick but I felt queasy. Thankfully I’ve not had it since, unlike many other people with Ménière’s disease, who get this feeling a lot.

“As well as my tinnitus I have low-level hearing loss, which means I struggle to hear people, especially in meetings at work. I keep getting tested for hearing loss and it hasn’t got any worse, but I think the tinnitus might be getting louder. It's there all the time in the background, but I notice it more when I’m stressed, when I think it gets a bit louder. It’s a high-pitched noise that changes randomly and sounds like someone trying to tune a radio.

“I’ve really got used to my tinnitus. It’s not so loud that it stops me hearing everything that’s going on. And when there’s lots of other background noise I don’t really hear it.

“I still go to clubs where there is loud music, but I wear a special set of earplugs to protect my hearing. I love listening to music on my iPod. When I play it with the volume low I drown out the tinnitus and get some relief.

"The hearing loss upsets me more than anything. But my employer has been fantastic and measures have been put in place to ensure I don’t miss important information in meetings. I get hard copies of the notes and am always seated near the speaker.

“I don’t take any treatments for tinnitus but I try not to get too stressed. I now have a 17-month-old daughter and was warned that the Ménière’s might worsen during pregnancy, but luckily this didn’t happen.

“Being positive is the key: if you let the condition get to you, like I did at the beginning, you start to think about it all the time and notice it a lot more. If you can learn to live with it then it makes life a lot easier.”

Risks

During a Ménière's disease attack your balance and hearing may be significantly impaired. This can make certain activities very dangerous.

The unpredictable nature of the condition means that you may need to change your daily activities so that you do not place yourself, or others, in danger. Situations that you may need to avoid include:

  • going swimming
  • climbing ladders or scaffolding
  • being out on your own
  • operating heavy machinery
  • driving (see below)

Driving

If you drive and you are diagnosed with Ménière's disease, or you are experiencing vertigo and hearing loss, you must inform your driver licence issuer about your condition.

If you drive a large goods vehicle or a passenger-carrying vehicle and you are diagnosed with Ménière's disease, your license will either be revoked or refused. You must be symptom-free for a year before reapplying for a licence.

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