Ménière's Disease

Meniere’s Disease is a symptom complex of vertigo, ringing noises in the ear (tinnitus), feeling of ear (aural) pressure, and fluctuating hearing loss. Prosper Meniere initially described this symptom complex in 1861. He was the first physician to suggest that this symptom complex was due to an inner ear problem as opposed to central nervous system disorder such as a stroke or tumor.

The attack of vertigo is commonly preceded by aural fullness in one or both ears. Hearing may fluctuate or diminish along with changes in tinnitus prior to the attack of vertigo. An episode of vertigo may last for a few hours. Imbalance, nausea and vomiting may also occur. After a severe attack of vertigo most people are exhausted and need to sleep for a few hours.

The frequency of attacks of vertigo is variable: frequent attacks every few days to periods without and attacks for several months. In other situations, the attacks may occur on a regular basis. Meniere’s Disease is usually confined to one ear. There is bilateral ear involvement in about 5 – 50% of cases, depending on which reports are cited. Meniere’s Disease affects approximately 0.2% of the population. While acute attack can be incapacitating and frightening, the disease itself is not life threatening.

Between the acute attacks most people are free of symptoms or have minimal imbalance. The tinnitus and hearing loss may persist even though it can fluctuate. In its most severe presentation, Meniere’s Disease can progress to permanent loss of hearing in the affected ear and unexpected falling attacks (“drop attacks”).

What causes Ménière’s Disease?

Symptoms of Meniere’s Disease (vertigo, tinnitus, and hearing loss) are thought to occur from paralysis of inner ear nerve endings by abnormal release inner ear fluid toxic to inner ear structures after rupture of inner ear compartments. It is felt that there is either an overproduction of inner ear fluid or inadequate absorption of this fluid. Repeated rupture release of the irritative inner ear fluid and healing leads to cumulative scarring of the inner ear structures resulting in permanent decrease in hearing and balance function. The cause of inner ear fluid leakage is unknown but this process may be due to a variety of causes such as autoimmune disease, inner ear head injuries, allergies, viral infection, and genetic predisposition. It is possible that these symptoms may be a common expression of an inner ear injured by different events.

How is Ménière’s Disease Diagnosed?

A diagnosis of Meniere’s Disease is based on clinical symptoms, hearing tests, examination by your physician and exclusion of other diagnoses. Supplementary tests may include audiological testing, balance tests such as electronystagmography (ENG), blood tests for other causes of dizziness, and radiological studies. At times, a difficult diagnosis of atypical Meniere’s Disease will become more apparent as the disease process is monitored, which may exclude other diseases causing similar symptoms.

How is Ménière’s Disease Treated?

While there is no cure for all the symptoms associated with Meniere’s Disease at this point, there are several strategies for managing the condition and controlling its symptoms.

An initial approach to managing Meniere’s Disease is usually with a low salt diet regimen. Decreased sodium intake also reduces the fluid in the inner ear. Generally, this means that salt intake should be less than 1.5gms per day. There are several books that can assist in determining which foods are high in salt. These can be obtained in any major bookstore of health food store. A highly recommended book is Barbara Kraus’s Complete Guide to Sodium, Signet, 1987, a paperback book listing the sodium contents of most foods. A consultation with a dietician or nutritionist may be of assistance. A diuretic such as Dyazide or Amiloride is frequently used. Other medications such as Neptazane, Diamox, or Verapamil may be utilized. Prednisone, a corticosteroid, may be beneficial in certain clinical situations. Vestibular suppressants such as Meclizine or Lorazapam may be used as needed.

If medical therapy is successful, one should see a clinical response within two months. If this regimen is helpful, then our patients usually follow this diet for two years before “graduation to a regular diet”.

In addition to avoiding salt containing foods, one should also consider the elimination of caffeine containing fluids and food such as coffee, tea and chocolate. Caffeine may exacerbate symptoms, particularly tinnitus. For those patients who have migraine associated vertigo and headaches. alcohol may also trigger migraine-associated vertigo. Foods containing MSG (Monosodiumglutonate), frequently found in Chinese food, may exacerbate symptoms for some patients. With a heightened awareness of the possible contribution of the above foods and substances towards triggering symptoms, one may identify aggravating substances for his/her type of Meniere’s Disease.

Nicotine and cigarettes may constrict blood vessels, which may decrease the blood supply to the inner ear making one more symptomatic.

Ménière’s Disease Refractory to Medical Therapy

Failure to control one’s symptoms with a medical regimen of diet and diuretics may require management with surgery. The type of surgical treatment is frequently based upon the patient’s hearing, vestibular function in the contralateral ear, general health, and age.

The least invasive surgical treatment involves placement of a pressure equalizing (PE) tube, which is also known as tympanostomy tube, into the tympanic membrane (ear drum). This is a very common procedure in young children for recurrent ear infections or persistent middle ear fluid. In the adult, the procedure can be performed in the office with anesthesia. Several days after the tympanostomy tube is inserted, a low-pressure air generator is used to administer air pressure through the tympanostomy tube to the inner ear for two minutes three times a day. It is felt that the air pressure to the round and oval windows of the inner ear displace excess inner ear fluid to eliminate vertigo, reduce tinnitus, and improve hearing. The Low Pressure Generator (Meniett Device) is purchased from the Medtronic division of the Xomed Company when prescribed by your physician. If this treatment is not successful, then other surgical treatments can be considered.

Recently, we have been using corticosteroid, “steroid,” (usually Dexamethasone or methylprednisolone) injections into the middle ear to control vertigo, especially for patients with usable hearing. Preliminary studies have demonstrated good response for control of vertigo in a large number of patients, to the point of eliminating the need for surgical therapy. This procedure, called intratympanic (IT) injection with dexamethasone or methylprednisolone, is performed in the office with topical anesthesia to numb the tympanic membrane (ear drum). Good response to this treatment is usually seen with two to four injections. The potential side affects are minimal with a possible hole in the tympanic membrane from incomplete healing following the injection (less than 1%), and transient middle ear pain from the medication in about 20% of the patients treated with methylprednisolone.

Chemical labyrinthectomy (destruction of the inner ear, labyrinth) with intratympanic gentamicin, injected into the middle ear in the outpatient office setting is an alternative method to “destroy” the defective nerve endings of balance. Gentamicin is an antibiotic that is toxic to the nerve endings of balance. One to several injections over one to two months may be required before therapeutic ablation of the nerve endings is achieved. This is usually a very effective procedure to control vertigo in the ear that has lost hearing.

Surgical Options for Control of Vertigo

If there is no usable hearing in the affected ear, a destructive surgical procedure called labyrinthectomy may be utilized to eradicate the deficient nerve endings in the balance system. If there is intact hearing in the affected ear, then a selective vestibular nerve section may be indicated. The vestibular nerve can be reached via three routes for a selective vestibular nerve section:

1. with a retrolabyrinthine approach through the mastoid behind the affected ear.

2. with a retrosigmoid craniectomy approach with a neurosurgeon via the back of the skull, and

3. with a middle fossa approach, also with a neurosurgeon

These two surgical procedures (labyrinthectomy and selective vestibular nerve section) have a very high rate of success in eliminating vertigo. This is an inpatient surgery, requiring hospitalization for at least three days. These two procedures are generally not used if there is Meniere’s Disease or inadequate balance function in the opposite ear.

Some physicians believe that an alternative procedure such as endolymphatic sac shunt procedure to relieve pressure in the inner ear may be helpful. This procedure has not been shown to have the same high success rate in controlling vertigo as the fore mentioned surgical labyrinthectomy or selective vestibular nerve section. The endolymphatic shunt procedure is a less invasive procedure for some patients and may be an initial surgical approach, with a success rate of 75 – 85% for control of vertigo. This operation is usually performed under general anesthesia and takes about two hours. Most patients go home the same day of surgery.

It should be noted that surgical treatment is primarily used to prevent attacks of vertigo. These procedures have not been demonstrated to affect the long-term progression of sensorineural hearing loss associated with Meniere’s Disease. The severity of your symptoms will also help determine the type of treatment you will receive. For example, if one is having falling attacks not controlled with medical management, then there will be a greater need for a definitive treatment such as intratympanic therapy (corticosteroid or gentamicin) or surgical control of vertigo.

Acute Vertigo Attacks

Management of an acute attack of Ménière’s may be best achieved with chewable Meclizine (Antivert). Lorazopam (Ativan) 0.5mg can be placed under the tongue. Sometime Prednisone taken orally for a few days will also help reduce symptoms of acute vertigo.

During an acute attack, one is essentially confined to bed rest. If vomiting is persistent and one is unable to take fluids for more than 24 hours, your physician may advise anti-nausea medication and/or a vestibular suppressant.

Since the acute symptoms of Meniere’s Disease are episodic, it is important to explain to your family and friends what might happen during an attack. This way they will be less frightened and more supportive during your attack.

If you anticipate an attack of vertigo, you need to remove yourself form situations of potential injury. For example, if you are driving a car, obviously you need to pull off the road. If you are having an attack during the night, be sure to use a night-light since you will be relying more on vision to help maintain your balance. There should be no potential obstruction on the way between the bedroom and the bathroom.

• More information may be obtained from self-help groups such as Vestibular Disorders Association (VDA) PO Box 4467, Portland, Oregon 97208-4467.
• Ménière’s Disease, What you need to Know by P.J. Haybach, R.N, MS is an excellent informative book for the lay reader. It may be ordered through the Vestibular Disorders Association (VDA) at $24.95 for softbound edition.
• NIDCD Fact Sheet on Ménière’s Disease [PDF]

*Meniere’s Disease Treatment Study

Dr. Hammerschlag is principal investigator for multicenter clinical study for new medical treatment for Meniere’s Disease at New York University Langone Medical Center. If you are interested in finding more about this study and to determine if you qualify to participate in this FDA approved study, please contact Jose Martinez, Senior Clinical Coordinator, at 201 478 2145.