Meniere’s Disease

What Causes Meniere’s Disease?

Scientists don’t yet know what causes Meniere’s disease, and there’s no clear understanding of how the disorder produces its symptoms. Many researchers believe that a buildup of fluid in the inner ear causes the symptoms, but others believe that the condition has a neurological origin. The only certain thing is that the disorder’s symptoms are caused by a disruption in communication between the inner ear’s sense organs and the brain.

Some theories of the cause of Meniere’s disease and its symptoms include:

• Viral or other infections. Some scientists think that Meniere’s disease could result from a virus, allergen, or other foreign agents in the inner ear. The disorder could stem from the agent’s action or the reaction to the agent by the body’s immune system.
• Autoimmune reactions. Some scientists think that the disorder could be caused by the immune system mistakenly attacking the inner ear’s normal cells.
• Physiological problems. Some scientists believe the buildup of fluid in the ear is caused by anatomical problems that prevent fluid or blood from flowing freely in the ear.
• Neurological causes. Other researchers think the disorder’s origin is similar to that of migraines, in which abnormalities in blood flow to nerve tissues cause problems with sensory perception.

It’s possible more than one (or even all) of these theories are correct and that Meniere’s disease can develop from a variety of different factors.

Is Meniere’s Disease Hereditary?

Most of the time, Meniere’s disease is not inherited. Most cases occur in people who have no family history of the disease. However, in a relatively small number of cases, the condition does appear to run in families, suggesting an inherited component in these cases.

Scientists have not yet discovered a specific gene that appears to be associated with Meniere’s disease.

How Is Meniere’s Disease Detected?

Early detection of Meniere’s disease can be challenging to achieve, mainly because the patient may dismiss their symptoms because they’re temporary, intermittent, and/or relatively mild. When a patient does report symptoms to a doctor, the doctor has to rely on the patient’s subjective description of the symptoms because there is no objective diagnostic tool that can reliably detect early signs of the disorder. The symptoms also may be similar to those of other conditions, leading to misdiagnosis.

Early detection is important; early treatment can reduce the chance of serious complications in the future. Scientists are working to find more sensitive and reliable diagnostic tools and procedures to improve the disorder’s early detection.

How Is Meniere’s Disease Diagnosed?

After conducting a physical exam and taking a medical history, a doctor can diagnose Meniere’s disease if all of the following conditions are present:

• The patient has had at least two vertigo episodes, each lasting between 20 minutes and 12 hours.
• The patient has experienced temporary hearing loss as measured by an objective hearing test.
• The patient has experienced tinnitus or pressure in the ear.
• The patient is not affected by another condition that could be causing these problems.

As part of the diagnostic process, doctors might conduct hearing tests to determine the extent of any hearing loss. They might also conduct imaging exams or other tests to identify problems with the condition or function of the inner ear or rule out any neurological condition that could be causing the symptoms.

PLEASE CONSULT A PHYSICIAN FOR MORE INFORMATION.

How Is Meniere’s Disease Treated?

Most cases of Meniere’s disease respond well to treatment. No treatment will cure the disorder itself, but treatment programs can be effective at managing symptoms. Common treatment approaches include:

• Medication. Drugs such as meclizine, diazepam, glycopyrrolate, and lorazepam are often used to treat dizziness or vertigo. These drugs can lessen the severity of episodes and/or shorten their duration.
• Diet modifications. Doctors typically recommend patients adopt a low-sodium diet to decrease fluid retention. In some cases, diuretic medications may be recommended.
• Antibiotics and steroids. The antibiotic gentamicin is sometimes used to treat severe vertigo, but the treatment carries the risk of making hearing loss worse. Corticosteroids are another option, and they do not pose the same risk. Both of these treatments are administered by injection into the inner ear.
• Pressure pulse treatment. This treatment involves using a device to deliver pulses of pressurized air to the inner ear. The treatment has proven to relieve dizziness in some cases.
• Psychotherapy. Meniere’s disease symptoms often put patients at risk of developing associated mental health issues such as anxiety or depression. Talk therapy can often help address these problems.

Surgical Treatments

In some severe cases that don’t respond to other treatments, surgery may be recommended to treat Meniere’s disease. Surgical interventions can include:

• Endolymphatic sac procedure. This procedure relieves pressure on the part of the inner ear that helps control fluid levels. In some cases, a shunt is installed to help drain excess fluid.
• Labyrinthectomy. In this procedure, the part of the inner ear responsible for both balance and hearing is removed. Both hearing and balance-sensing will be lost in the affected ear, but the other ear will compensate for those functions. This procedure is usually only considered when the hearing loss in the affected ear is already nearly complete.
• Vestibular nerve section. This procedure severs the neurological connection between the inner ear’s balance-sensing organs and the brain. When successful, the procedure preserves hearing in the affected ear while relieving vertigo.

How Does Meniere’s Disease Progress?

About 60% of patients with Meniere’s disease get relief from symptoms on their own or with treatment. If symptoms persist or worsen over the long term, however, sufferers are at risk of both physical and psychological complications, including:

• Permanent hearing loss
• Falls and accidents
• Fatigue
• Stress
• Anxiety
• Depression
• Social isolation
• Work disruptions

How Is Meniere’s Disease Prevented?

There is no known way to prevent Meniere’s disease from occurring. Patients with the disorder may, however, be able to decrease the likelihood of future episodes by making specific lifestyle changes, including:

• Reducing salt intake
• Avoiding alcohol and caffeine
• Quitting smoking
• Avoiding noisy environments or exposure to loud sounds
• Taking steps to manage stress or anxiety

Meniere’s Disease Caregiver Tips

Providing care for someone with a disorder like Meniere’s disease can be challenging for the caregiver because the disorder’s symptoms don’t have a clearly defined physical cause. It can be hard to remain compassionate and supportive in the face of an invisible disorder, so keep these tips in mind as you care for your loved one:

• Educate yourself about the disorder. Learn all you can about Meniere’s disease so that you’re better able to understand what your loved one is going through. Aside from the physical symptoms, the unpredictability of the disease is one of its most challenging aspects. The better you understand what’s going on, the better you’ll be able to help your loved one (and yourself) to cope.
• Be supportive. Sometimes people living with Meniere’s disease don’t feel like others truly believe how profoundly they’re affected by the disorder’s symptoms. Assure your loved one that you understand and believe them.
• Know when to step away. Sometimes your loved one will need time alone, and you should be sensitive to their needs, even when you feel as if you should always be there for them. Getting some distance between yourself and the disorder can be vital to your well-being as a caregiver too. Don’t neglect your own physical and mental health needs, and don’t be afraid to step away when you’re feeling overwhelmed.

Many people with Meniere’s disease also suffer from other brain and mental health-related issues, a situation called co-morbidity. Here are a few of the disorders commonly associated with MD:

• About half of people with MD suffer from depression.
• Anxiety and phobias are more common in people with MD.
• Migraines are sometimes associated with MD.

Meniere’s Disease Brain Science

The mechanism by which Meniere’s disease produces vertigo and hearing loss is not understood. Some researchers believe that excess fluid in the inner (or the mixing of different types of fluid) interferes with the transmission of balance and hearing-related nerve signals to the brain. This theory holds that the buildup of fluid is the underlying cause of the symptoms.

Others think the cause is related to blood flow problems that can produce an effect like a mini-stroke in the inner ear’s sensory organs. This type of situation is thought by some scientists to also cause migraines, and many Meniere’s patients also suffer from migraines. Scientists who pursue this theory consider Meniere’s disease to be a “cerebrovascular” disorder, meaning that it is related to both the brain and the circulatory system. They believe that the buildup of fluid in the inner ear contributes to symptoms but is not their underlying cause.

Meniere’s Disease Research

Title: Assessing the Efficacy of a Serotonin and Norepinephrine Reuptake Inhibitor for Improving Meniere’s Disease Outcomes

Stage: Recruiting

Principal investigator: Habib Rizk, MD

Medical University of South Carolina

Charleston, SC

As of yet, the cause of Meniere’s disease is uncertain, and there is no cure. Given the lack of high-level evidence for treatments, we seek to perform a randomized, placebo-controlled, double-blind, crossover pilot trial of venlafaxine for treating Meniere’s disease. Venlafaxine is a safe and well-tolerated medication. It has never been trialed in Meniere’s disease, but there is evidence that it could help with vertigo attacks and other aspects of the disorder.

Title: CI Following VS Removal or Labyrinthectomy

Stage: Recruiting

Principal investigator: Oliver Adunka, MD

Ohio State University

Columbus, OH

This study is a prospective, clinical study to determine if it is safe and effective to use a cochlear implant over time in individuals undergoing removal of a vestibular schwannoma (VS), benign tumor of the hearing and balance nerve or undergoing a labyrinthectomy for treatment of Meniere’s disease. Individuals undergoing these surgeries will be deaf on the surgical site after the procedure. Currently, cochlear implants are approved for use and not considered investigational in individuals with hearing loss on both sides. However, using a cochlear implant for these patient populations (single-sided hearing loss) will be regarded as a new use of an approved device. Participants undergoing surgery to remove a VS or having a labyrinthectomy will have a cochlear implant inserted after the surgical procedure for clinical care. Approximately four weeks after surgery, participants will be fitted with an external speech processor on the surgical side to stimulate the internal cochlear implant. Participants will return at the following intervals after the initial processor fitting: 2 weeks, 1 month, 3 months, 6 months, 9 months, and 12 months. At each interval, participants will complete questionnaires on how they are hearing with the implant and their quality of life with the implant. They will also be tested on their ability to hear sounds and understand speech. Potential risks are those associated with all cochlear implant surgeries; these include device failure resulting in the removal of device, irritation or redness in surgical area and/or region where the processor is attached, increased ringing in the ear, facial nerve stimulation, and a change in the way speech and other sounds sound through the implant. Potential benefits to individual participants in this study include improvement in detection and speech understanding of the surgical ear. Participants may also experience improved abilities to locate a sound and understand speech in noise due to hearing on both sides.

Title: Effects of Anakinra in Subjects With Autoimmune Inner Ear Disease

Stage: Recruiting

Principal investigator:  Andrea Vambutas, MD

Northwell Health, Hearing & Speech Center

New Hyde Park, NY

A Phase II randomized, placebo-controlled study design of anakinra (Kineret) in corticosteroid-resistant Meniere’s disease (CR-MD)and corticosteroid-resistant autoimmune inner ear disease (CR-AIED) patients. Patients will be randomized by a 2:1 allocation to anakinra or placebo for 42 continuous days. After day 42, a second placebo-controlled period will begin for an additional 42 days. This will be followed by a 264 day observation period, during which hearing declines may be re-treated with anakinra after 30 days following the initial drug.