Meniere’s disease is a disorder of the inner ear that causes a sensation of spinning (vertigo), mild-to-moderate hearing loss, tinnitus, pain, and a feeling of pressure in the ear. It is estimated that about 90% of people with Meniere’s have the disease in only one ear when first diagnosed. Around 50% of them may go on to develop the disease in both ears.1
People between 40 and 50 years of age are most prone to this syndrome, but it can occur in adolescents and children as well. Meniere’s disease is considered a chronic condition; however, various treatment strategies can help relieve symptoms and minimize the disease’s long-term impact. This article will review the signs and symptoms, pathophysiology, diagnosis, and current treatment of this disease.
SIGNS AND SYMPTOMS
Episodes of Meniere’s symptoms typically last between 20 minutes and 4 hours, after which they improve. Episodes often occur in clusters, with long periods of either mild symptoms or no symptoms in between.1 The severity, frequency, and duration of each of these symptoms vary in the early stage of the disease. A person may experience mild vertigo and hearing loss with frequent tinnitus that disturbs sleep, or could have severe episodes of vertigo with few other symptoms.2
Sudden episodes of vertigo are the major symptom of Meniere’s disease; these episodes usually last from 20 minutes to 2 hours, but may last up to 24 hours. Vertigo is a sensation similar to what a person would experience after spinning around quickly and suddenly stopping. It would feel as if the room were still spinning, and the person would lose balance. The vertigo episodes often force the person to lie down for several hours and lose time from work or leisure activities, and can cause emotional distress. Vertigo can also increase the risk of falling; of having accidents while driving a car or operating heavy machinery; of experiencing depression or anxiety in dealing with the disease; and of permanent hearing loss.3
Hearing loss in Meniere’s disease may fluctuate, particularly early in the course of the disease. Eventually, most people experience some degree of permanent hearing loss. Patients may experience noise in the ear, or tinnitus, the perception of a ringing, buzzing, roaring, or whistling sound. People with Meniere’s disease often feel pain and aural fullness, or increased pressure in the ear. Nausea and vomiting followed by severe vertigo can happen in some patients.
Because any one of the above problems may be the result of other illnesses, it is important to get an accurate diagnosis as soon as possible. Vertigo could be a possible sign of other disorders, such as stroke, brain tumor, multiple sclerosis, or diseases of the heart or blood vessels. One should be extremely cautious if vertigo is accompanied by any of the following signs or symptoms: severe headache, double vision, speech impairment, leg or arm weakness, loss of consciousness, numbness or tingling, and chest pain.1,2
PATHOPHYSIOLOGY
Although the etiology of Meniere’s disease is not well understood, it appears to be the result of the abnormal volume or composition of fluid in the inner ear.
The inner ear consists of a system of connected tubes and cavities called a labyrinth. The outer ear consists of bone, and is known as the bony labyrinth. A membranous labyrinth, made up of a soft structure of membrane, is located inside the bony labyrinth, and is similar in shape. The membranous labyrinth contains a fluid; hairlike sensors that line the membranous labyrinth respond to movement of the fluid.2 This fluid needs to retain a certain volume, pressure, and chemical composition in order for all of the sensors in the inner ear to function properly. The following factors may change the properties of inner-ear fluid and cause or trigger Meniere’s disease.3
• Improper fluid drainage due to blockage or anatomical abnormality
• Abnormal immune response
• Allergies
• Viral infection
• Genetic predisposition
• Head trauma
• Migraines
No single cause has yet been identified for Meniere’s disease; it may be caused by a combination of factors.
DIAGNOSIS
Primary care physicians may perform a preliminary evaluation of the condition, and may refer patients to an ear, nose, and throat (ENT) specialist, an audiologist, or a neurologist. A diagnosis of Meniere’s disease requires two spontaneous episodes of vertigo, each lasting 20 minutes or longer, hearing loss verified by a hearing test on at least one occasion, and tinnitus or aural fullness. Exclusion of other known causes of these sensory problems is also a key factor in properly diagnosing Meniere's.3,4 The evaluation process may include the following:
Hearing evaluation: A hearing test (audiometry) assesses how well a person detects sounds at different pitches and volumes and how well he or she distinguishes between similar-sounding words. The test not only reveals the quality of hearing but also may help determine if the source of hearing problems is in the inner ear or the nerve that connects the inner ear to the brain.
Balance evaluation: Between episodes of vertigo, most people regain their sense of balance. In some cases, however, people experience ongoing balance problems. There are several tests that assess the balance function of the inner ear, including videonystagmography (VNG), rotary-chair testing, vestibular evoked myogenic potentials (VEMP) testing, and posturography. Some or all of these tests can yield abnormal results in a person with Meniere’s disease.2,4
Other tests may be used to rule out disorders that can cause problems similar to those of Meniere’s disease, such as a tumor in the brain or multiple sclerosis. These tests include MRI that produces a 3-D image of the brain, CT that produces cross-sectional images of internal structures in the human body, and brainstem response audiometry. The latter is a computerized test that can help detect the presence of a tumor that is disrupting the function of auditory nerves.1
TREATMENT
No cure exists for Meniere’s disease, but a number of strategies, including medication, surgery, supplemental therapies, and dietary changes may help manage the symptoms. Research shows that most people with Meniere’s disease respond to treatment, but have to cope with long-term hearing loss.5
Drug Treatment
Short-term medications: Physicians may prescribe short-term medications to be taken during an episode of vertigo to lessen the severity of an attack. Medications such as meclizine or diazepam may reduce the spinning sensation of vertigo and help control nausea and vomiting. Drugs such as promethazine may also control nausea and vomiting during an episode of vertigo.2,6
Long-term medications: The purpose of long-term medication, such as the drug combination triamterene and hydrochlorothiazide. is to reduce fluid retention (diuretic). Reducing the amount of fluid the body retains may help regulate the fluid volume and pain and pressure in the inner ear. For some people, diuretics help control the severity and frequency of Meniere’s disease symptoms. Owing to frequent urination, the body may deplete certain minerals, such as potassium. While taking diuretics, the body must be supplemented weekly with three or four extra servings of potassium-rich foods, such as bananas, cantaloupe, oranges, spinach, and sweet potatoes.2,6
Injectable medications: Certain medications injected into the middle ear may improve vertigo symptoms: gentamicin, an antibiotic toxic to the inner ear (ototoxic), reduces the balancing function of one ear, and allows the other ear to assume responsibility for balance. This is an outpatient procedure, and it often reduces the frequency and severity of vertigo attacks. There is a risk, however, of further hearing loss. Corticosteroids, such as dexamethasone, also may help control vertigo attacks in some people. This is also an outpatient procedure that is done in a physician’s office under local anesthesia. Dexamethasone may be slightly less effective than gentamicin, but it is less likely to cause further hearing loss.2,6
Surgical Treatment
People with severe and debilitating cases of Meniere’s disease may resort to surgery. Surgical procedures may include7
Endolymphatic sac procedures: These surgical procedures may alleviate vertigo by decreasing fluid production or increasing fluid absorption in the endolymphatic sac, which plays a role in regulating inner ear fluid levels. In endolymphatic sac decompression, a small amount of bone is removed from over the endolymphatic sac. This procedure may be coupled with the placement of a shunt, a tube that drains excess fluid from the inner ear.
Vestibular nerve section: In this procedure the nerve in the inner ear that connects balance and movement sensors to the brain (vestibular nerve) is cut. This usually corrects problems with vertigo and pain while attempting to preserve hearing in the affected ear.
Labyrinthectomy: This procedure involves removing the balance portion of the inner ear, thereby removing both balance and hearing function from the affected ear. This procedure is performed only if the patient already has near-total or total hearing loss in the affected ear.7
Supplemental Therapies and Procedures
Vestibular rehabilitation: The goal of this therapy is to help a person’s body and brain regain the ability to process balance information correctly between episodes of vertigo.2 Vestibular rehabilitation may include exercises and activities during therapy sessions and at home.
Hearing aid: A hearing aid in the ear affected by Meniere’s disease may improve hearing. Audiologists will discuss what hearing aid options are available and most suitable for the patient.
Meniett pulse generator: This device is for vertigo that is hard to treat. The therapy involves the application of positive pressure to the middle ear to improve fluid exchange. The device applies pulses of pressure to the ear canal through a ventilation tube. The treatment is performed at home, usually three times a day for 5 minutes at a time. It is reported that this technique has shown improvement in symptoms of vertigo, tinnitus, and aural pain and pressure, but its long-term effectiveness has not been determined.2,8
In addition to pharmacotherapy, surgery, and supplemental therapies, patients can be educated about diet modifications that can reduce the body’s fluid retention and help decrease fluid in the inner ear (Table 1).
CONCLUSION
Although there is no cure for Meniere’s disease, it can be effectively managed, and most patients respond to treatment. In addition to the physical effects, Meniere’s disease may affect interaction with friends and family, productivity at work, and the overall quality of life. Patients may find encouragement and understanding in a support group. Group members can provide information, resources, and coping strategies. Physicians may be able to recommend a support group in the area, or patients may find information about local groups from the Vestibular Disorders Association.
REFERENCES
1, Lalwani AK. Current Diagnosis & Treatment Otolaryngology–Head and Neck Surgery. 3rd ed. New York, NY: The McGraw-Hill Companies; 2012.
2. Mayo Clinic. Meniere’s disease. December 11, 2012. www.mayoclinic.org/diseases-conditions/menieres-disease/basics/definition/con-20028251 Accessed February10, 2015.
3. Rauch SD. Clinical hints and precipitating factors in patients suffering from Meniere’s disease. Otolaryngol Clin North Am. 2010; 43:1011-1017.
4. Neff BA, Staab JP, Eggers SD, et al. Auditory and vestibular symptoms and chronic subjective dizziness in patients with Meniere’s disease, vestibular migraine, and Meniere’s disease with concomitant vestibular migraine. Otol Neurotol. 2012;33:1235-1244.
5. Vestibular Disorders Association. Meniere’s disease. http://vestibular.org/menieres-disease. Accessed December 20, 2014.
6. Dinces EA. Meniere’s disease. UptoDate. www.uptodate.com/index. Accessed Nov 22, 2014.
7. American Academy of Otolaryngology–Head and Neck Surgery. Meniere’s disease. www.entnet.org/HealthInformation/menieresDisease.cfm. Accessed Dec 14, 2014.
8. National Institute on Deafness and Other Communication Disorders. U.S. Department of Health and Human Services. Meniere’s disease. July 2010. www.nidcd.nih.gov/health/balance/pages/meniere.aspx. Accessed Nov.12, 2014.
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