Ménière's disease affects your inner ear and, as a result, causes problems with balance, tinnitus and hearing. For every 100,000 people in the UK, up to 190 will develop Ménière's disease at some point in their life. 

Here, we will discuss what causes Ménière's disease and its symptoms, as well as how it is diagnosed and treated. First, to understand Ménière's disease, it helps to know a bit more about your inner ear. 

Inner ear: what does it do?

Your ear can be divided into 3 sections: the outer ear, middle ear and inner ear. As the name suggests, your inner ear is the innermost part of your ear. It contains the bony labyrinth, that is, several bony cavities called the cochlea, vestibule and semicircular canals. 

Your cochlea allows you to hear, while your vestibule and semicircular canals are responsible for your balance and awareness of your spatial orientation. These structures are filled with fluid called endolymph. 

In your semicircular canals and vestibule, movement of this fluid helps you sense how you are orientated and, therefore, allows you to balance. 

In your cochlea, vibrations from sounds you hear cause this fluid to move. This, in turn, moves tiny projections on specialised cells called hair cells. This causes the hair cells to send electrical signals to your brain, allowing you to perceive sounds. 

What happens in Ménière's disease?

In Ménière's disease, too much fluid builds up in the inner ear. More specifically, too much endolymph builds up in the bony labyrinth of the inner ear. This is called endolymphatic hydrops.

It causes fluid-filled channels in the inner ear to swell and expand, which results in the symptoms associated with Ménière's disease.

Symptoms of Ménière's disease

Ménière's disease usually affects one ear but in around 1 in 5 people with the condition, both ears are eventually affected. Common symptoms include: 

• A feeling of pressure in your ear
• Hearing loss that causes impaired hearing of low-frequency sounds eg deep voices, the bass in music or heavy footsteps
• Severe bouts of vertigo that can last anywhere from 10 minutes to several hours
• Tinnitus, specifically a low-frequency sound coming from within your ear that is often described as similar to the hum of a fridge 

Vomiting may also occur.

These symptoms tend to come and go, often with several weeks or months of intense clusters of attacks followed by weeks, months or even years without any symptoms. 

During periods where symptoms are absent, any hearing loss may recover, but usually does not return to the level prior to the clusters of attacks. This means that over time hearing loss becomes worse. 

You may not experience all of these symptoms. Some individuals only ever develop 1 or 2 of these common symptoms eg only vertigo or only fluctuating hearing loss or tinnitus. 

Stress can worsen symptoms of Ménière's disease.

Conditions associated with Ménière's disease

Individuals with Ménière's disease often have other associated conditions. 

Around 2 in 5 people with Ménière's disease have migraines. Around 1 in 5 people with Ménière's disease have BPPV (benign paroxysmal positional vertigo), where small crystals in the inner ear dislodge and move around the semicircular canals. 

As a result, Ménière's disease can be difficult to diagnose and needs a specialist to investigate and diagnose the condition. This may be an ENT (ear, nose and throat) surgeon or audio-vestibular physician.

Causes and triggers of Ménière's disease

While Ménière's disease can run in families, most cases occur spontaneously. 

It can sometimes be triggered by an ear infection or head injury. Allergies and autoimmune conditions may also be involved in the onset of Ménière's disease.

Your risk of Ménière's disease is higher if you are aged between 40 and 60 years.

Conditions similar to Meniere’s disease

The symptoms of Ménière's disease overlap with several different conditions, which is why it can be challenging to diagnose. The condition that most closely mimics Ménière's disease is vestibular migraine. 

Migraines affect around 1 in 10 people. Of those who have migraine, around 1 in 10 have vestibular migraines, which are characterised by dizziness, vertigo, tinnitus and a feeling of fullness in the ears. Vestibular migraines can also cause mild reversible hearing loss. 

While the symptoms of vestibular migraines overlap with Ménière's disease, vestibular migraines are more common — they affect 1 in 100 people while Ménière's disease affects around 1 to 2 in 1,000 people. 

Many individuals with Ménière's disease also have vestibular migraines at the same time. 

A perilymph fistula (a hole in the inner ear) can also cause some of the same symptoms as Ménière's disease, namely dizziness, vertigo, tinnitus and a feeling of fullness. Episodic ataxia type 2, which is a rare genetic condition, can cause vertigo similar to Ménière's disease too.

Diagnosing Ménière's disease 

To get a diagnosis of Ménière's disease, you need to see an ENT surgeon or audio-vestibular physician. 

They will speak to you about your symptoms and take a detailed medical history. You will also need to have a hearing test, specialist balance testing and vestibular investigations, which together will tell your doctor about the function of your inner ear. It is likely an MRI scan of your inner ears and head will be required too.

An  MRI scan of the inner ear can help identify excessive fluid accumulation in the inner ear (endolymphatic hydrops). This is only available in specialist centres.

If you receive a diagnosis of Ménière's disease and you drive, you should check whether you need to inform the DVLA of your condition.

Treating Ménière's disease 

Although there is no “cure” for Ménière's disease, it can usually be very effectively treated once the diagnosis is confirmed. It also often burns out over years or decades.

Treatment focuses on improving your quality of life and reducing your symptoms, especially the attacks of vertigo. 

Lifestyle changes

Your doctor may recommend following a low-salt diet (2.5 grams of salt per day). 

Following a healthy balanced diet, staying hydrated, avoiding caffeine and red wine, and exercising regularly can also help manage your symptoms. 

As stress can worsen symptoms, it is important to regularly engage in activities that help you relax and destress. 

Medications and injections

Your doctor may prescribe certain medications, such as betahistine and bendroflumethiazide, to lower the fluid pressure in your ears. The benefits of tablets in Meniere’s disease are uncertain. These medications are not effective for everyone with Meniere’s disease but some find them helpful in managing their symptoms. 

If medications are not helpful or not thought to be appropriate in your case, your doctor may recommend intratympanic steroid or gentamicin injections, that is, injections into your middle ear. Research has shown these injections can reduce the number of vertigo attacks by 90%. They can be very effective and are performed on an outpatient basis.

Surgery 

If all other treatments are unsuccessful, your doctor may recommend surgery. 

One option is endolymphatic sac surgery which involves drilling into the bone behind your ear (the mastoid bone) to decompress the endolymphatic sac. This is a relatively low-risk, day case operation that has a high success rate, with an 80–95% reduction in vertigo reported. 

If endolymphatic sac surgery is not effective, surgery to remove the labyrinth of the inner ear can be performed (labyrinthectomy). This removes the structures needed for balance and hearing to eliminate symptoms of Ménière's disease. However, it also results in complete hearing loss in the ear operated on. 

Alternatively, the vestibular nerve can be cut (vestibular nerve section) to stop nerve signals from the vestibular apparatus of the inner ear reaching the brain. In most cases, this does not cause any hearing loss. It is a major operation and is rarely needed; most patients are well-managed with injections performed as an outpatient procedure.