Overview

Meniere's disease is a condition that affects your inner ear, which is the deepest part of your ear responsible for both hearing and balance. It happens because of an abnormal build-up of fluid, called endolymph, within the delicate tubes and chambers of your inner ear, known as the labyrinth. This fluid build-up and increased pressure, medically termed 'endolymphatic hydrops', can damage the parts of your inner ear that control balance (the vestibular system) and hearing (the cochlea).

If doctors can identify a specific reason for this fluid imbalance, the condition is more accurately called Meniere's syndrome. However, in most cases of Meniere's disease, the exact cause of the fluid build-up isn't known.

This condition is considered rare and typically affects adults between 20 and 60 years old, with men and women being equally affected. It usually starts by affecting only one ear, but over time, it can sometimes involve both ears in about 15% to 25% of people.

The main feature of Meniere's disease is that its symptoms come and go in unpredictable episodes. These attacks can be very severe and debilitating, often lasting from minutes to several hours, typically around 2 to 3 hours. After a severe attack, you might feel very unwell and exhausted, sometimes needing to rest or sleep for several hours. It can take up to a day or two for all symptoms to completely settle down. While Meniere's disease is not life-threatening, it can significantly impact your daily life and overall well-being.

Symptoms and Causes

Meniere's disease occurs when there is an abnormal build-up of fluid in your inner ear. This extra fluid increases pressure within the inner ear structures, which are vital for sending signals to your brain about balance and sound. This pressure imbalance is what causes the symptoms you experience.

Symptoms

The main symptoms of Meniere's disease often happen together in episodes and can vary in how severe they are and how often they occur. The four core symptoms are:

• Vertigo: This is a severe spinning sensation, where you feel like you or your surroundings are moving or spinning. It can come on suddenly and unpredictably, lasting from minutes to several hours. Vertigo attacks are often accompanied by severe nausea and vomiting, making you feel very ill and often needing you to lie down until the attack passes. You might feel slightly unsteady for a day or two after an attack.
• Hearing Loss: The hearing loss you experience is called sensorineural hearing loss, which means it's caused by damage to the inner ear or the nerve pathways to the brain. This hearing loss fluctuates, often getting worse during an attack and improving between episodes, especially in the early stages. Low-pitched sounds are usually affected first. Over time, the hearing loss can become permanent and gradually worsen, though it's rare to become completely deaf in the affected ear.
• Tinnitus: This is the sensation of hearing internal noises, like ringing, roaring, or buzzing, in your affected ear that aren't coming from an outside source. Tinnitus can increase during attacks and may become a constant presence as the disease progresses.
• Aural Pressure/Fullness: You might feel a sensation of pressure or fullness deep inside your affected ear. This feeling can sometimes happen before a vertigo attack.

Less common symptoms can include being very sensitive to loud sounds (called hyperacusis) or experiencing distorted sound perception. Some people, though rarely, might also have sudden falls without losing consciousness, known as 'drop attacks' or Tumarkin's Otolithic Crisis. These falls are usually very brief with minimal vertigo but can be alarming and potentially dangerous.

Meniere's disease is often described in three stages, though not everyone goes through every stage:

• Early-stage: This stage is mostly marked by sudden, unpredictable attacks of vertigo. Your hearing gets worse and tinnitus increases during these attacks, but you usually recover well between episodes. Periods where you have no symptoms can last from days to years.
• Middle-stage: Vertigo episodes continue, and you might feel dizzy or unsteady before and after attacks. Sensorineural hearing loss develops, and tinnitus becomes more noticeable. Symptom-free periods can vary, sometimes lasting several months.
• Late-stage: Your hearing loss increases, and vertigo attacks tend to become less frequent or even stop completely after 5 to 15 years, a process sometimes called the condition 'burning out'. However, you might be left with persistent tinnitus, ongoing hearing loss in one ear, aural pressure, and a general sense of imbalance. Balance difficulties, especially in the dark, can also occur due to permanent damage to your balance system.

Causes

The exact cause of Meniere's disease is not fully understood. However, we know it's linked to that abnormal build-up of fluid (endolymph) in your inner ear, which increases pressure within the labyrinth. This fluid imbalance is thought to disrupt how your ear sends signals to your brain about balance and sound.

Several factors are thought to contribute to this fluid imbalance and increase your risk of developing Meniere's disease:

• Poor fluid drainage in the inner ear.
• Abnormal immune responses or autoimmune disorders, where your body's immune system mistakenly attacks healthy tissues.
• Viral infections, such as meningitis.
• Genetic predisposition, meaning it can sometimes run in families.
• Stress or allergies.
• Head injury.
• Migraines.

Diagnosis and Investigations

Diagnosing Meniere's disease can be challenging because its symptoms can be similar to other conditions. There isn't one single test that can definitively confirm it. Instead, your doctor will make a diagnosis based on a careful review of your symptoms, your medical history, and by performing tests to rule out other possible conditions.

Diagnosis

Your GP will typically start by taking a detailed history of your symptoms to identify a pattern consistent with Meniere's disease. They will ask about the nature, duration, and frequency of your vertigo, hearing changes, tinnitus, and any feelings of ear fullness. If Meniere's disease is suspected, you will usually be referred to an Ear, Nose, and Throat (ENT) specialist for further assessment.

For a diagnosis of Meniere's disease to be made, you generally need to meet specific criteria:

• You must have had at least two episodes of vertigo or dizziness, with each episode lasting between 20 minutes and 24 hours.
• You must experience fluctuating symptoms in the affected ear, such as hearing loss, tinnitus, or a feeling of fullness.
• Your symptoms cannot be better explained by another balance or ear condition.

It's important to note that a definitive diagnosis usually requires the presence of all three core symptoms: vertigo, fluctuating hearing loss, and tinnitus or aural fullness.

Investigations

To help confirm the diagnosis and rule out other conditions, your ENT specialist may recommend several tests:

• Hearing Tests (Audiometry): This is a key test that measures your ability to hear sounds of different pitches and volumes. It helps to assess the degree and type of hearing loss, particularly looking for low-frequency sensorineural hearing loss, and to track any changes over time.
• Balance Tests: Tests like Video Nystagmography (VNG) or Video Head Impulse Tests (vHITs) are used to check how well your inner ear's balance system is working. These tests record your eye movements to see how they respond to head movements.
• MRI or CT Scans: These imaging scans are primarily used to rule out other potential causes of dizziness, vertigo, or hearing loss. For example, they can check for conditions like acoustic neuromas (benign, non-cancerous swellings that can grow on the balance nerve) or other neurological conditions like multiple sclerosis. In most people with Meniere's disease, the MRI scan will appear normal.
• Blood Tests: These may be performed to check for underlying autoimmune diseases or infections like syphilis, which can sometimes cause similar symptoms.
• Electrocochleography (ECoG): This test measures electrical activity in your inner ear in response to sound and can help confirm the presence of endolymphatic hydrops (fluid build-up).
• Vestibular Evoked Myogenic Potentials (VEMPs): This is another test used to assess the function of specific parts of the inner ear involved in balance.
• Attack Diary: Keeping a detailed diary of your vertigo episodes, including when they happen, how long they last, and any associated hearing loss, tinnitus, or ear fullness, can be very helpful for your doctor in making a diagnosis and planning your treatment.

Management and Treatment

Currently, there is no cure for Meniere's disease, but there are many treatments available to help manage your symptoms, reduce the frequency and severity of attacks, and improve your quality of life.

Medications for Acute Attacks

These medications are used to relieve severe symptoms during a sudden attack:

• Anti-vertigo and Anti-sickness Medications: Drugs like prochlorperazine and cinnarizine can help to lessen severe vertigo, nausea, and vomiting during an acute attack. It's important to carry these medications with you if you are prone to sudden attacks. These are typically for short-term use only and should not be taken regularly for prevention due to potential side effects.

Preventative Medications

These medications are taken regularly to try and reduce the frequency and severity of vertigo attacks:

• Betahistine: This is often prescribed to help reduce the number and intensity of vertigo attacks.
• Diuretics: Medications such as bendroflumethiazide may be used to help reduce fluid build-up in the inner ear.

Intratympanic Injections

For severe cases that don't respond well to oral medications, injections directly into the middle ear (intratympanic injections) may be considered:

• Steroid Injections (e.g., Dexamethasone, Methylprednisolone): These are injected into the middle ear to reduce inflammation and help control vertigo. They often have fewer side effects than gentamicin injections and have shown similar long-term vertigo control.
• Gentamicin Injections: This is an 'ablative' treatment, meaning it aims to damage the balance function of the affected ear to reduce vertigo. While effective for vertigo, it carries a risk of worsening hearing loss in the treated ear. It is generally considered when other non-ablative treatments have not worked.

Surgical Management

Surgery is usually reserved for very severe cases where other treatments have failed and your quality of life is significantly impacted. These procedures are often irreversible:

• Grommets: These are tiny tubes placed across your eardrum to help relieve pressure. A short-term grommet is usually inserted first, and if it proves effective, a longer-term one can be considered. This is the least invasive surgical option.
• Endolymphatic Sac Surgery: This procedure aims to reduce the fluid build-up in the inner ear.
• Vestibular Nerve Section: This involves surgically cutting the balance nerve to stop dizziness and vertigo, while aiming to preserve your hearing.
• Labyrinthectomy: This is a more extensive surgical procedure that involves removing the semicircular canals and the balance nerve, effectively destroying both the balance and hearing function of the affected ear. It is typically considered when hearing in the affected ear is already severely impaired.

Other Management Strategies

• Hearing Aids: If you have persistent hearing loss, hearing aids can be provided to help improve your hearing.
• Tinnitus Management: Various therapies and strategies are available to help you cope with tinnitus, such as white noise generators or counselling.
• Balance Training (Vestibular Rehabilitation): This is an exercise-based program designed to help your balance system function as effectively as possible. It is particularly useful for persistent balance problems between attacks or in the later stages of the disease.
• Counselling and Relaxation Therapy: Techniques like Cognitive Behavioural Therapy (CBT) and relaxation exercises can provide support and help you manage the anxiety and stress often associated with Meniere's disease.

Prevention

While you can't prevent Meniere's disease itself, certain lifestyle adjustments and dietary changes can help reduce fluctuations in inner ear fluid pressure and potentially lessen the frequency and severity of your symptoms. You might notice benefits from these changes after a few weeks.

• Dietary Modifications:
• • Low-salt diet: Aim for 1-2 grams of sodium daily. Distribute your food and fluid intake evenly throughout the day and avoid skipping meals. Limit high-salt foods such as processed foods and canned foods. This approach is thought to help lower inner ear pressure.
  • Hydration: Drink adequate amounts of fluid daily, including water and low-sugar fruit juices. Remember to anticipate and replace fluid loss during exercise or in hot weather.
  • Avoid Caffeine: Limit or avoid caffeine found in coffee, tea, cola, and chocolate, as its stimulant properties may worsen your symptoms.
  • Limit Alcohol: Restrict your alcohol intake to no more than one glass of beer or wine per day.
• Stress Management: Try to reduce stress in your life where possible, as stress can make your symptoms worse. Relaxation techniques, counselling, and meditation apps can be very beneficial.
• Avoid Nicotine: Do not smoke or chew nicotine products.
• Regular Sleep: Ensure you get adequate and restful sleep.
• Driving: If you have been diagnosed with Meniere's disease, you must inform the Driver and Vehicle Licensing Agency (DVLA) and stop driving if you experience sudden dizziness or vertigo. You can usually resume driving once your symptoms are well-controlled and you have been advised it is safe to do so.
• Safety Precautions: During periods when you are experiencing active symptoms, avoid activities where sudden dizziness or vertigo could pose a risk, such as climbing ladders or swimming alone. Implement home safety measures to prevent falls, such as good lighting and clear pathways.

Outlook / Prognosis

Meniere's disease is a long-term condition, and its outlook can vary significantly from person to person. Initially, symptoms and hearing loss often fluctuate, with complete resolution between episodes.

Over time, however, hearing loss typically progresses, and tinnitus may become a persistent symptom. The good news is that the frequency of vertigo attacks often decreases, and in many cases, the severe spinning sensations may resolve completely after 5 to 15 years, a phenomenon sometimes referred to as the condition 'burning out'.

Despite the potential reduction in vertigo, permanent damage to the vestibular system (your balance system) can lead to ongoing balance problems, especially in challenging conditions like darkness. You might also experience severe hearing loss in the affected ear, along with sound distortion and recruitment (where loud sounds are perceived as much louder and more uncomfortable than they should be).

While Meniere's disease is not fatal, the debilitating nature of the attacks and the potential for long-term hearing and balance issues can significantly impact your quality of life. However, with proper treatment and lifestyle adjustments, many people with Meniere's disease can lead full and active lives.

In about 60% to 80% of people, symptoms improve and may even disappear after two to eight years. However, some individuals may be left with permanent hearing loss, persistent tinnitus, or both. In approximately 15% to 25% of patients, the disease can eventually affect both ears, often many years after the initial onset.