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Meniere's Disease

Disease Details

Family Health Simplified

Description
Meniere's disease is a chronic inner ear condition characterized by episodes of vertigo, hearing loss, tinnitus, and a feeling of fullness or pressure in the ear.
Type
Meniere's disease is a disorder of the inner ear. The type of genetic transmission for Meniere's disease is not well understood, but it appears to have a multifactorial inheritance pattern, suggesting that both genetic and environmental factors contribute to its development. Some cases have shown familial clustering, indicating a potential hereditary component.
Signs And Symptoms
Ménière's is characterized by recurrent episodes of vertigo, fluctuating hearing loss, and tinnitus; episodes may be preceded by a headache and a feeling of fullness in the ears. People may also experience additional symptoms related to irregular reactions of the autonomic nervous system. These symptoms are not symptoms of Ménière's disease per se, but rather are side effects resulting from failure of the organ of hearing and balance, and include nausea, vomiting, and sweating, which are typically symptoms of vertigo, and not of Ménière's. This includes a sensation of being pushed sharply to the floor from behind. Sudden falls without loss of consciousness (drop attacks) may be experienced by some people.
Prognosis
Ménière's disease usually starts confined to one ear; it extends to both ears in about 30% of cases. People may start out with only one symptom, but in Ménière's disease all three appear with time. Hearing loss usually fluctuates in the beginning stages and becomes more permanent in later stages. Ménière's disease has a course of 5–15 years, and people generally end up with mild disequilibrium, tinnitus, and moderate hearing loss in one ear.
As of 2020, there has been no recent major breakthrough in the pathogenesis research of Ménière's disease.
Onset
Meniere's disease typically has an onset in adulthood, most commonly between the ages of 40 and 60 years. However, it can occur at any age. The exact cause is unknown, but it involves abnormal fluid buildup in the inner ear. Symptoms often include episodes of vertigo, tinnitus, hearing loss, and a feeling of fullness in the ear.
Prevalence
Meniere's disease is a relatively rare chronic condition. It is estimated to affect about 0.2% of the population. This translates to about 200 people per 100,000. However, prevalence rates can vary slightly depending on the population studied and the diagnostic criteria used.
Epidemiology
From 3 to 11% of diagnosed dizziness in neuro-otological clinics are due to Ménière's disease. The annual incidence rate is estimated to be about 15 cases per 100,000 people and the prevalence rate is about 218 per 100,000, and around 15% of people with Ménière's disease are older than 65. In around 9% of cases, a relative also had Ménière's disease, indicating a genetic predisposition in some cases.The odds of Ménière's disease are greater for people of white ethnicity, with severe obesity, and women. Several conditions are often comorbid with Ménière's disease, including arthritis, psoriasis, gastroesophageal reflux disease, irritable bowel syndrome, and migraine.
Intractability
Meniere's disease can often be intractable, meaning it can be difficult to manage and cure completely. While treatments are available to help control the symptoms—such as vertigo, hearing loss, and tinnitus—there is no definitive cure. Management strategies typically focus on reducing the frequency and severity of episodes.
Disease Severity
Meniere's disease severity can vary significantly among individuals. Some may experience mild symptoms, while others might have severe, debilitating episodes. The severity is typically classified based on the frequency and impact of vertigo attacks, hearing loss, and other symptoms like tinnitus and aural fullness. Monitoring and proper management are crucial to improve the quality of life for those affected.
Healthcare Professionals
Disease Ontology ID - DOID:9849
Pathophysiology
Meniere's disease pathophysiology involves an abnormality in the inner ear, particularly in the fluid-filled structures known as the labyrinth. The key feature is an excess buildup of endolymph fluid in the cochlea and vestibular system, referred to as endolymphatic hydrops. This fluid imbalance can lead to increased pressure and swelling, causing symptoms such as vertigo, tinnitus, hearing loss, and a feeling of fullness in the ear. The exact cause of the fluid buildup remains unclear, but potential factors include genetic predisposition, viral infections, autoimmune reactions, and allergies.
Carrier Status
Meniere's disease is not associated with a carrier status as it is not a hereditary condition with a simple inheritance pattern. It is a disorder of the inner ear that can cause vertigo, tinnitus, hearing loss, and a feeling of fullness or pressure in the ear. The exact cause is unknown, but it may involve a combination of genetic and environmental factors.
Mechanism
The initial triggers of Ménière's disease are not fully understood, with a variety of potential inflammatory causes that lead to endolymphatic hydrops, a distension of the endolymphatic spaces in the inner ear. Endolymphatic hydrops is strongly associated with developing Ménière's disease, but not everyone with EH develops Ménière's disease: "The relationship between endolymphatic hydrops and Meniere's disease is not a simple, ideal correlation."Additionally, in fully developed Ménière's disease, the balance system (vestibular system) and the hearing system (cochlea) of the inner ear are affected, but some cases occur where EH affects only one of the two systems enough to cause symptoms. The corresponding subtypes of the disease are called vestibular Ménière's disease, showing symptoms of vertigo, and cochlear Ménière's disease, showing symptoms of hearing loss and tinnitus.The mechanism of Ménière's disease is not fully explained by EH, but fully developed EH may mechanically and chemically interfere with the sensory cells for balance and hearing, which can lead to temporary dysfunction and even to death of the sensory cells, which in turn can cause the typical symptoms of MD – vertigo, hearing loss, and tinnitus.An estimated 30% of people with Ménière's disease have Eustachian tube dysfunction.
Treatment
Treatment for Meniere's disease typically includes:

1. **Medications:**
- Diuretics to reduce fluid retention
- Anti-nausea and anti-vertigo medications to manage symptoms

2. **Dietary Changes:**
- Low-sodium diet to decrease fluid buildup
- Avoiding caffeine, alcohol, and tobacco

3. **Therapies:**
- Vestibular rehabilitation to improve balance
- Hearing aids for those with hearing loss

4. **Injections:**
- Gentamicin to reduce vertigo attacks
- Steroids to reduce inflammation

5. **Surgery (in severe cases):**
- Endolymphatic sac decompression
- Labyrinthectomy or vestibular nerve section to control vertigo

Regular follow-ups with a healthcare provider are essential to manage the condition effectively.
Compassionate Use Treatment
For Meniere's disease, compassionate use treatments, off-label or experimental treatments may include:

1. **Gentamicin injections**: Administered directly into the middle ear, this antibiotic can reduce symptoms by affecting the balance function.

2. **Steroid injections**: Intratympanic steroids like dexamethasone can help reduce inflammation and alleviate symptoms.

3. **Betahistine**: Although not approved by the FDA in the U.S., it is used off-label in several other countries to improve blood flow in the inner ear and reduce dizziness.

4. **Non-invasive Meniett device**: Uses low-pressure pulses to help reduce fluid buildup and alleviate symptoms, though its use is considered experimental.

5. **Anti-inflammatory and immunosuppressive drugs**: Medications like methotrexate or biologics might be used experimentally to treat suspected autoimmune aspects of Meniere's disease.

6. **Gene therapy**: Research is ongoing to explore gene therapy as a future treatment option.

These treatments are typically considered when conventional therapies fail, and their use should be carefully monitored by a healthcare professional.
Lifestyle Recommendations
### Lifestyle Recommendations for Meniere’s Disease:

1. **Dietary Changes**:
- **Reduce Salt Intake**: High salt levels can affect fluid balance in the inner ear. A low-sodium diet is often recommended.
- **Limit Caffeine and Alcohol**: Both can exacerbate symptoms like vertigo and tinnitus.
- **Hydration**: Drink plenty of fluids to help maintain fluid balance in the body.

2. **Stress Management**:
- **Practice Relaxation Techniques**: Methods such as meditation, yoga, and deep-breathing exercises can help manage stress, which may trigger symptoms.
- **Adequate Sleep**: Ensure you get enough rest as fatigue can worsen symptoms.

3. **Physical Activity**:
- **Regular Exercise**: Engage in moderate exercise to improve overall health and reduce stress levels. Activities like walking, swimming, or tai chi can be beneficial.
- **Vestibular Rehabilitation**: Specific exercises might be recommended by a therapist to help manage balance issues.

4. **Avoid Triggers**:
- **Monitor and Avoid Known Triggers**: Keep a diary of foods, activities, and other factors that trigger or worsen your symptoms, and try to avoid them.

5. **Safety Measures**:
- **Use Assistive Devices if Necessary**: For those experiencing significant balance issues, the use of canes or other supportive devices can help prevent falls.
- **Home Safety**: Install grab bars, avoid slippery surfaces, and ensure good lighting to reduce the risk of accidents.

6. **Limit Noise Exposure**:
- **Protect Ears from Loud Noises**: Use ear protection in noisy environments to prevent further damage and worsening of symptoms.

7. **Regular Medical Follow-up**:
- **Work with Healthcare Providers**: Regular visits to an audiologist and/or ENT specialist to monitor the disease and adjust treatment plans as necessary.

These recommendations can help individuals manage their symptoms and improve their quality of life.
Medication
During MD episodes, medications to reduce nausea are used, as are drugs to reduce the anxiety caused by vertigo. For longer-term treatment to stop progression, the evidence base is weak for all treatments. Although a causal relation between allergy and Ménière's disease is uncertain, medication to control allergies may be helpful. To assist with vertigo and balance problems, glycopyrrolate has been found to be a useful vestibular suppressant in patients with Ménière's disease.Diuretics, such as the thiazide-like diuretic chlortalidone, are widely used to manage MD on the theory that it reduces fluid buildup (pressure) in the ear. Based on evidence from multiple but small clinical trials, diuretics appear to be useful for reducing the frequency of episodes of dizziness but do not seem to prevent hearing loss.In cases where hearing loss and continuing severe episodes of vertigo occur, a chemical labyrinthectomy, in which a medication such as gentamicin is injected into the middle ear and kills parts of the vestibular apparatus, may be prescribed. This treatment has the risk of worsening hearing loss.
Repurposable Drugs
Currently, there are no widely recognized repurposable drugs specifically for Meniere's disease. Treatments often focus on managing symptoms, including diuretics, corticosteroids, antihistamines, and antiemetics. Emerging research may offer new insights in the future.
Metabolites
In Meniere's disease, specific metabolites have not been definitively identified as being unique to the condition. However, some studies suggest alterations in certain biochemical markers related to inner ear function and fluid regulation, including possible imbalances in electrolytes like sodium and potassium, which may contribute to the symptoms. Current research is ongoing to better understand the metabolic changes associated with Meniere's disease.
Nutraceuticals
There is limited evidence supporting the use of nutraceuticals in managing Meniere's disease. Some studies suggest potential benefits from antioxidants, vitamins (like B6, C, and E), and omega-3 fatty acids, but more research is needed. Always consult a healthcare provider before starting any new supplement regimen.
Peptides
For Meniere's disease, research into the use of peptides is still in its early stages. Peptides may offer potential therapeutic benefits due to their ability to target specific cellular mechanisms implicated in the disease, such as inflammation and ion channel regulation. However, clinical application and efficacy of peptide treatment for Meniere's disease remain under investigation. If considering any new treatments, it's crucial to consult with a healthcare professional or specialist.