What is Meniere's Disease?

Meniere's disease is a disorder of the fluid pressure in the inner ear.  The cause of this fluid pressure is not know.  It is unkown if this increase in fluid pressure represents an overproduction of fluid or an underabsorption of fluid.  There are many theories to explain the origin of Meniere's disease: congenital, inherited, traumatic, inflammatory, allergic, etc; but none have been shown to be a universal cause of Meniere's disease.  

Regardless of the cause, universally patients with Meniere's disease have swelling of the membranes of the inner ear.  This is the classic finding on pathologic examination of the temporal bones of these patients.  It is theorized that as the fluid pressure builds in the inner ear, the fine membranes of the inner are ruptured and a mixing of the two fluid compartment of the inner ear occurs, producing the classic symptoms of Meniere's Disease.

Meniere's disease can strike at any age.  Patients from adolescent years to senescence have been diagnosed with Meniere's disease.  The classic symptoms of Meniere's disease are (1) disabling vertigo, (2) low frequency hearing loss, (3) roaring tinnitus, and (4) aural pressure or fullness.

The vertigo of Meniere's disease is spontaneous and disabling.  Attacks occur without provocation, but many patients learn to interrupt signs of an impending attack.  Some patients notice an increase in the intensity of their tinnitus prior to an attack.  A full-blown attack of Meniere's vertigo can occur at any time of day and can even awaken patients from sleep.  The vertigo is so violent that patients are unable to walk.  Occasionally, patients will suffer a spontaneous loss of postural muscle tone and fall.  These events are called drop attacks.

During and especially after the vertigo subsides, patients will report a loss of hearing in one ear.  The loss is usually in low frequencies.  In the early phase of the disease, the hearing can return to normal or near-normal levels.  However, as the disease progresses, repeated attacks and insults to the inner ear causes a gradual, progressive hearing loss in the affected ear.

Accompanying the hearing loss is a low tone ringing sound in the affected ear.  Often this sound is described as a roaring sound or like the sound of listening to a seashell.  This sound can be intermittent in nature, especially early on in the disease course.  But similar to the hearing loss, as the disease progresses the roaring sound becomes permanent and can increase in its loudness.

The last classic symptom of Meniere's disease is aural fullness.  This symptom is reported by patients as a feeling of fluid or pressure in the affected ear.  Some patients will recognize that an increase in this pressure sensation will presage an attack of vertigo.

Medical Treatment for Meniere's Disease

Since the primary finding in Meniere's disease is swelling of the inner ear, the treatment is aimed at minimizing or eliminating this swelling.  The primary tools for controlling Meniere's disease are (1) a low salt diet and (2) diuretics.

Perhaps you have noticed that the morning after eating a salty dinner of pizza and spaghetti that you have slight swelling of the fingers.  Rings might not fit well or be hard to remove.  This swelling is due in large measure to the amount of salt ingested.  The typical American diet contains about 8 to 10 grams of salt per day.  You can find out about the amount of salt (expressed as Sodium) in your diet by reading the product information on packaged food that you buy.  If you've never noticed the amounts of sodium before, you'll be surprised by the amounts contained in the foods you eat.  Especially high in salt are canned foods, prepared foods, and restaurant prepared foods.

Patients with Meniere's disease are instructed to reduce their salt intake to 1500 mg per day.  The first step in this reduction is to eliminate adding salt to any food.   Secondly, by observing the Sodium amounts in portions, one can tally the amount of salt ingested.  As patients reduce the amount of salt in their diet, many will come to realize how salt-sensitive they are.  Frequently, patients who splurge or get off their low salt diet and eat a few extra pizza slices or strips of bacon for a day will pay for it by having an attack of Meniere's disease the following day.

Diuretics are used to help control swelling as well.  Commonly, a balanced diuretic such as hydrochlorothiazide/triamterene (Dyazide or Maxzide) is prescribed.  This diuretic helps your body eliminate sodium and excess water, while preserving potassium.  Other diuretics might be used depending on an individual's response to hydrochlorothiazide/triamterene.

The combination of low salt diet and diuretics can help to control vertigo spells in most patients with Meniere's disease.   However, not all patients experience relief of vertigo with these treatments.  Surgery can be performed to help patients who continue to have vertigo despite medical treatment for Meniere's disease.

Surgical Treatments for Meniere's Disease

Over the last seventy or eighty years, a number of different procedures have been devised to help eliminate vertigo spells.  A few of these have withstood the test of time and will be briefly discussed below.

Intratympanic Gentamicin - in this procedure, a small hole is made in the eardrum and a medication (Gentamicin with or without steroids) is injected into the middle ear.  The medication is absorbed through the round window into the inner ear.  Gentamicin is an aminoglycoside antibiotic that is vestibulotoxic, meaning that it destroys vestibular cells.  By destroying these vestibular cells, Gentamicin helps to prevent the perception of vertigo.  Gentamicin is also potentially cochleotoxic, meaning that it can cause hearing loss.  This procedure is performed with local anesthetic on an outpatient basis.

Endolymphatic shut - in this procedure, the endolymphatic sac is exposed in the mastoid region and opened, allowing the endolymphatic fluid to be drained off.  This procedure is performed under general anesthesia.  In this procedure, the inner ear balance organs (the labyrinth) are left alone.  Like all major ear operations, this procedure carries the risks of hearing loss, tinnitus, dizziness, facial paralysis, or loss of taste on the tongue.

Labyrinthectomy - in this procedure, the labyrinth is removed.  The labyrinth contains the vestibular organs and is reached by performing a mastoidectomy.  With this procedure, all hearing in the operated ear is permanently lost; therefore, this procedure is performed only in patients with poor hearing.  This procedure is performed under general anesthesia.  The risks of the procedure are similar to those listed for endolymphatic shut.

Vestibular nerve section - This title can be applied to an entire family of procedures in which the vestibular nerve is cut.  The vestibular nerve can be sectioned through three approaches: translabyrinthine, middle fossa, or retrosigmoid.  In the translabyrinthine approach, hearing cannot be preserved; however, both the middle fossa and retrosigmoid approaches can be used to preserve hearing while sectioning the nerve.  All three involve the possibility of cerebrospinal fluid leak and meningitis.  All three involve the potential risks of hearing loss, tinnitus, dizziness, facial paralysis, and loss of taste.