What is Meniere Disease?
Meniere’s disease (MD) is a condition that affects the inner ear and causes problems like hearing loss, ringing in the ears (known as tinnitus), and a spinning sensation known as vertigo. Over time, these symptoms can slowly get worse and can greatly affect a person’s social life.
It’s common for people to go to the doctor with symptoms like hearing loss, tinnitus, and vertigo, but Meniere’s disease – which also has these symptoms – is actually quite rare. It’s often determined by ruling out other possible causes for the symptoms. There are certain criteria created by the Barany society, a group of health professionals specialized in balance disorders, that can help to identify whether a person has definite or probable MD.
According to these criteria, a person is considered to have definite Meniere’s disease if they have:
1. Two or more unexpected episodes of vertigo, each lasting from 20 minutes to 12 hours;
2. Hearing loss that affects the lower to middle range of sounds, which has been confirmed by hearing tests, happening in one ear on at least one occasion before, during, or after one of the episodes of vertigo;
3. Symptoms related to the ear, such as a feeling of fullness in the ear, changes in hearing, or ringing in the ears located in the affected ear, that come and go;
4. Symptoms that can’t be better explained by any other condition affecting the balance systems of the body.
Probable Meniere’s disease might be the diagnosis if someone has:
1. Two or more episodes of dizziness or vertigo, each lasting from 20 minutes to 24 hours;
2. Symptoms that come and go, such as a feeling of fullness in the ear, changes in hearing, or ringing in the ears, located in the affected ear;
3. Symptoms that are best explained by another condition affecting the balance systems of the body.
What Causes Meniere Disease?
Meniere’s disease is a condition that affects parts of the ear, specifically, the cochlea and the vestibular organ. Research shows that fluid build-up known as “endolymphatic hydrops” often happens in these parts of the ear in people with Meniere’s disease, and this fluid build-up is linked to a significant hearing loss of more than 40 decibels. Vertigo, a sensation of feeling off balance, may or may not be present along with this hearing loss.
However, it’s worth noting that this fluid accumulation isn’t exclusive to Meniere’s disease, and it’s also seen in individuals experiencing unexplained hearing loss.
The exact cause of Meniere’s disease is still not known. Various theories exist suggesting a mix of genetic factors and environmental influences, but their connection to common co-existing health conditions is still not understood fully.
Risk Factors and Frequency for Meniere Disease
Meniere’s disease has quite a varied incidence rate, ranging from 3.5 to 513 cases per 100,000 individuals. It is mostly found in older, white, female patients. This condition has a few associated health issues, known as comorbidities, which include migraines, autoimmune diseases, and a genetic component.
- Migraine: People diagnosed with Meniere’s disease tend to experience migraines more often. However, there’s a possibility that some of these patients have been misdiagnosed, having basilar migraines instead. Interestingly, there are unproven theories suggesting a vascular cause for Meniere’s disease.
- Autoimmune diseases: Meniere’s disease is often linked with several autoimmune diseases, including rheumatoid arthritis, systemic lupus erythematosus, and ankylosing spondylitis. Some studies suggest that IgE, a type of antibody, could play a part in Meniere’s disease.
- Genetic component: Meniere’s disease can be influenced by multiple genes and is therefore referred to as a polygenic disorder. About 10% of European patients have a family history of the disease. The condition can be inherited in different ways, but most cases are sporadic, meaning they occur in people without a family history of the disease. The genetics of Meniere’s disease is currently a hot area of research.
Signs and Symptoms of Meniere Disease
When a doctor evaluates a patient who reports dizziness, they are tasked with determining whether the cause is central (relating to the brain), peripheral (relating to the ear), or cardiovascular (relating to the heart). Certain signs such as additional neurological symptoms, sudden deafness, a new or different kind of headache, or specific patterns of uncontrollable eye movements could indicate that the vertigo has a brain-related cause.
A thorough examination of the patient’s ear health history is crucial in any case related to hearing or balance. The doctor will ask detailed questions about the person’s experiences of vertigo, distinguishing between the sensation of spinning and a general feeling of imbalance or near fainting. They’ll also ask about any hearing loss and past incidents of these symptoms. Other important factors include how long the vertigo and hearing loss episodes last, what triggers them (for example, certain head movements suggesting Benign Paroxysmal Positional Vertigo, or feeling lightheaded when standing up suggesting low blood pressure), and whether the patient’s family has a history of hearing and balance issues.
The doctor will then carry out a thorough physical examination. This involves a detailed neurological exam, including an evaluation of the patient’s cranial nerves to exclude any other specific areas of concern. They may use simple hearing tests, such as the Rinne and Weber tests, to have a basic assessment of audiological nerve function, but more detailed audiology tests will be necessary. The patient’s sensation in all their limbs is evaluated, as well as their walking pattern. There will also be tests to assess the part of the brain involved in balance. If positional vertigo is suspected, a Dix-Hallpike maneuver may be performed, ideally with special goggles to help spot abnormal eye movements, and the doctor will measure the patient’s standing blood pressure.
In some conditions like Meniere disease, certain tests could indicate sensorineural hearing loss depending on the extent of hearing loss, which is why a more detailed audiology test is needed. Special goggles might show horizontal eye movements occurring away from the affected inner ear organ. The Dix-Hallpike move is a test for positional vertigo where the doctor quickly moves the patient from sitting to lying down with their head turned and neck extended backwards. If the patient’s eyes show certain movements or they experience vertigo during the test, it’s a sign of positional vertigo.
Some tests have a low effectiveness, like the head impulse test which may not detect Meniere disease as compared to other peripheral vestibular disorders.
Testing for Meniere Disease
All patients diagnosed with Meniere’s disease, a condition that affects balance and hearing, must have their hearing tested. The disease typically starts with intermittent hearing loss in the lower sound frequencies in one ear. Over time, this hearing loss can get worse and affect all sound frequencies. A ringing or buzzing noise in the ear, known as tinnitus, is also common.
Patients who have hearing loss in one ear should have an MRI scan. This scan helps rule out problems with the nerve pathways involved in hearing and balance. Some countries might use a BERA (brainstem evoked response audiometry). This test measures the electrical activity in the brain in response to sound to check your hearing. You don’t need to have these tests immediately when the symptoms appear, but they should be done within a few weeks.
High-resolution MRI imaging can show fluid build-up in the affected ear, a key feature of Meniere’s disease. However, more research is being done to determine how this information can be best used for patient care.
Tests of the balance function of the ear, known as vestibular or caloric tests, may be performed. These can reveal decreased function in the affected ear in 42% to 74% of cases, and a total loss of function in 6% to 11% of cases.
Treatment Options for Meniere Disease
The treatment options for Meniere’s disease, a disorder of the inner ear, can differ from country to country, but none of them can completely cure the disease. Many of these treatments can affect the function of other systems in our body, so it’s generally best to start with the least harmful, non-invasive approaches and move to more invasive ones if necessary.
Some possible initial treatments include:
Sodium restriction diet: Cutting down on your salt intake could potentially help to prevent attacks brought on by Meniere’s disease, although this is based on limited evidence.
Use of Thiazide diuretics: These are medicines that can help to reduce the occurrence and severity of symptoms which can be caused by too much fluid in the body. While they can make the attacks less frequent and severe, they may not prevent hearing loss.
There are other possible treatments with varying degrees of disagreement and evidence:
Betahistine: Some in the medical community are in disagreement about the use of Betahistine which is a type of medication. It is believed to work by improving blood flow in the inner ear, which can help reduce the build-up of pressure. A Cochrane review, an in-depth analysis of healthcare research, found limited evidence supporting its use, with variations in findings across different studies.
Steroid injections in the ear: Injecting steroids into the ear may reduce the number of vertigo (feeling of dizziness and loss of balance) attacks in patients with Meniere’s disease while preserving hearing.
In contrast, other treatment methods are more severe:
Gentamycin injections in the ear: Gentamycin is a type of antibiotic and it has a strong destructive effect on the cells in the inner ear that help with balance. Unfortunately, this treatment may cause hearing loss due to its toxic effects on cells that help hearing.
Vestibular nerve section or labyrinthectomy: There are two types of surgeries that can be considered when the symptoms do not respond to less invasive treatments. The vestibular nerve section involves cutting a nerve in the ear that plays a role in balance, and labyrinthectomy involves removing some of the inner ear structures. However, labyrinthectomy results in complete hearing loss on the affected side.
What else can Meniere Disease be?
If you’re feeling dizzy and experiencing vertigo, there could be several possible reasons. Here’s a list of different conditions that could be the cause:
- Basilar migraine: Typically comes with vertigo but doesn’t result in symptoms related to the ear
- Vestibular neuronitis: This condition causes vertigo lasting for several days, without any ear-related symptoms
- Benign paroxysmal positional vertigo: This causes vertigo related to head movements that lasts from seconds to minutes, and it doesn’t cause ear-related symptoms
- Medications: Certain medications like aminoglycosides and loop diuretics could be responsible
- Central vertigo: Possible causes include stroke, multiple sclerosis, seizure disorder, and others
- Peripheral vertigo of non-ear related origin: This is commonly seen in older patients with peripheral neuropathy and deconditioning
- Orthostatic hypotension: While not exactly vertigo, patients might describe themselves as feeling “dizzy”
- Neoplasm: Certain tumors like vestibular schwannoma, meningioma, malignancy could be the cause
- Infectious causes: Certain infections like meningitis, syphilis, HIV cerebritis, among others could be the culprits
What to expect with Meniere Disease
According to research by Perrez-Garrigues and colleagues, the instances of dizziness or spinning sensations, known as vertigo, are more frequent in the early stages of the disease and tend to decrease over time whether one is undergoing treatment or not. Most patients eventually reach a phase where they do not experience vertigo, but they might face significant loss of hearing.
Similarly, this loss of hearing is majorly experienced in the initial years of the disease and tends to stabilize in the later years. Unfortunately, once the loss occurs, there is usually no recovery. It is worth noting that individuals with Meniere’s disease in one ear have a higher chance of developing the same in the other ear. In fact, one review reports that up to 47% of patients over a span of 20 years end up with the condition affecting both their ears.
Possible Complications When Diagnosed with Meniere Disease
In more advanced stages of the condition, patients may suddenly lose muscle strength and fall down without fainting. This is known as Tumarkin crises.
Furthermore, people living with Meniere’s disease often report a lower quality of life compared to those without it. They also tend to experience anxiety and depression at a higher rate than the general population.
Key Points:
- In the later stages of this condition, patients may experience sudden and unexpected muscle weakness, causing them to fall. This is known as Tumarkin crises.
- People with Meniere’s disease often have a lower quality of life compared to healthy individuals.
- The chances of having anxiety and depression are significantly higher in people suffering from Meniere’s disease.
Preventing Meniere Disease
If you notice a sudden loss of hearing in one ear along with episodes of dizziness that can last from a few minutes to several hours, and ringing in your ear (a condition known as tinnitus), it’s possible you might have a condition called Meniere’s disease. These symptoms are a cause for concern and require immediate medical attention. This is important not just to rule out any serious health issues that could potentially threaten your life, but also to confirm the diagnosis and start the right treatment.
