What is Meniere's Disease?

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MENIERE'S DISEASE

What is Meniere's Disease?
Meniere's Disease, also known as endolymphatic hydrops, is a disorder affecting the fluids of the inner ear. Normally, the fluids of the inner ear move in response to sound waves to allow us to hear and in response to head movement to give us our sense of position. With Meniere's Disease, there is an excessive amount of fluid within the inner ear chamber. The cause of this disorder is unknown. In most cases only one ear is involved, although it may be seen in both ears in 15 to 20% of patients. Classic Meniere's Disease is characterized by intermittent attacks of vertigo, hearing loss, ringing and fullness of the ear. Vertigo is the sense of spinning dizziness. Attacks last from 20 minutes to as long as several hours and may be associated with nausea and vomiting. The frequency of the attacks is variable and unpredictable. The hearing loss may fluctuate, just as with the attacks of vertigo. Tinnitus and fullness may also fluctuate unpredictably. The severity of the symptoms is variable and may only be a nuisance or become disabling.

Diagnosis
The diagnosis of Meniere's is in many ways a diagnosis of exclusion, as there is no single finding or test which is definitive. A thorough otologic and neurologic history and physical examination is performed. It is important to determine the nature of the symptoms of dizziness, their duration and frequency. A review of the patient's health is important in order to rule out other systemic problems which may mimic Meniere's, including thyroid disease, diabetes, hypertension or other neurologic disorders. The physical examination is typically normal, unless the patient is seen during an attack. A hearing test (audiogram) is almost always obtained. The hearing loss seen in Meniere's disease is most often sensorineural (inner ear) in nature. Typically, the hearing loss is greater in the lower frequencies, but may involve all frequencies. Tests may also be obtained to gain information about the inner ear balance system. Electronystagmography (ENG) is test that evaluates the balance system. The study measures eye movements, which reflexively move in response to various stimuli to the inner ear. Warm and cool stimuli to the ear canal may be helpful in determining which ear is weaker. Other tests of the balance system, such as platform posturography, may be obtained depending on the nature of one's symptoms. Radiographic imaging is usually necessary to rule out other potential causes for the patient's symptoms. Magnetic Resonance Imaging (MRI) images the hearing and balance nerves to be certain that a tumor or other anomalies may or may not be causing these symptoms.

Treatment

Medical Therapy
The primary goal of medical therapy is to limit the fluid load on the inner ear. Just as with other medical conditions that cause excessive fluid accumulation in other parts of the body, a diuretic (water pill) is the key component of medical therapy. In order for the diuretic to be of optimal benefit, it is critical that patients restrict the intake of salt. In fact, "binging" with salty foods may result in attacks of vertigo. Anti-vertigo medications, such as Antivert (meclizine) and Valium (diazepam), are helpful in relieving symptoms of vertigo. It is important to remember that these medications do not prevent attacks, but only relieve symptoms. Thus, they should only be taken as needed.

Changes in diet and lifestyle are critical in the management of Meniere's Disease. A low sodium (salt) diet is critical in preventing attacks. Furthermore, it is important to avoid caffeine, nicotine and alcohol. Stress is also an aggravating factor and if possible, should be avoided.

Surgical Treatment
80% of patients with Meniere's Disease will see their symptoms come under excellent control with medical therapy. If a patient continues to have severe, disabling attacks of vertigo in spite of medical therapy, surgical alternatives should be considered.

Endolymphatic-mastoid shunt is a 45 minute surgical procedure. This is performed through an incision in the crease behind the ear. The mastoid is opened and the endolymphatic sac is identified. This structure is the natural drainage sac of the inner ear. The endolymphatic sac is then incised and a shunt is placed in order to relieve the inner ear of the excessive fluid build-up seen in Meniere's Disease. The shunt material is typically made of thin silastic. The procedure may be performed either on an outpatient basis or as an overnight stay. Recovery is typically one week. This is the only procedure we perform designed to change the way the inner ear works. As such, results are variable. Long term outcomes suggest that 65-70 % of patients will be completely cured of all symptoms and require no further medication. An additional 15% of patients will have significant improvement, but still require medical therapy for control of symptoms. In about 15% of patients, symptoms may continue to be disabling and require alternative surgical intervention. Hearing is improved or stabilized in the majority of patients.

Selective Vestibular Neurectomy is a 2 hour surgical procedure. This is performed through an incision behind the ear. A window of bone is removed and the lining of the brain (dura) is opened. The hearing and balance nerve (eighth cranial nerve) is identified as it passes between the inner ear and brainstem. The hearing and balance nerve fibers are separated. The balance fibers are cut while preserving the hearing nerve. The patient is observed overnight in the ICU and the overall hospital stay is 2 to 5 days. Recovery may take up to 6 weeks. While one can live with only one inner ear, the brain must compensate for the loss. The acute response to loss of inner ear function may result in spinning vertigo for about 24 hours. The patient's equilibrium will recover over a period of 2 to 5 days to the point where one can ambulate, although it may take 6 weeks for full compensation to be achieved. Occasionally, physical therapy is recommended to regain full balance. Attacks of vertigo are permanently eliminated in nearly all patients, while hearing is preserved.

Labyrinthectomy with or without sectioning of the eighth cranial nerve is a 90 minute surgical procedure. This is performed through an incision in the crease behind the ear. The mastoid is opened and the inner ear surgically destroyed and at times the nerve endings of the eighth cranial nerve are cut. This will permanently eliminate attacks of vertigo, but also results in permanent loss of hearing in the operated ear. Keeping in mind the 15-20% incidence of Meniere's involving both ears, this procedure is reserved for only select situations in which there is severe non-serviceable hearing in the affected ear. While compensation is still required to regain balance, this is typically not as prolonged as seen with selective vestibular neurectomy. This is because in these situations, the Meniere's has resulted in a severly weakend inner ear and compensation has already begun preoperatively. Typically the hospital stay is 1-2 days.

Transtympanic perfusion is a procedure in which an antibiotic (gentamycin), which is toxic to the inner ear, is directly injected through the eardrum. The solution is then absorbed into the inner ear through the round window. This is an office-based procedure, which is technically the least complex that we perform for Meniere's Disease. While the goal is to eliminate the function of the affected balance organ, the inner ear hearing (cochlear) function may be affected as well. Permanent hearing loss has been reported in as many as 30-40% of patients. Furthermore, there may be incomplete destruction of the balance organ, resulting in lingering disequilibrium and/or recurrent attacks of vertigo.

It is important to remember that there is no perfect surgical solution for every case of Meniere's Disease. When attacks become disabling in spite of medical therapy, surgery should be considered in consultation with your surgeon.