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|Fig 1. Normal inner ear.|
In 1861, the French physician Prosper Ménière described a condition which now bears his name. Ménière's disease is a disorder of the inner ear which causes episodes of vertigo, ringing in the ears (tinnitus), a feeling of fullness or pressure in the ear, and fluctuating hearing loss. In figure 1, the area of the ear affected is the entire labyrinth, which includes both the semicircular canals and the cochlea.
A typical attack of Ménière's disease is preceded by fullness in one ear. Hearing fluctuation or changes in tinnitus may also precede an attack. A Ménière's episode generally involves severe vertigo (spinning), imbalance, nausea and vomiting. The average attack lasts two to four hours. Following a severe attack, most people find that they are exhausted and must sleep for several hours. There is a large amount of variability in the duration of symptoms. Some people experience brief "shocks", and others have constant unsteadiness. An unusual sensitivity to visual stimuli is common. (Lacour, 1997)
A particularly disabling symptom is a sudden fall that may occur without warning. These are called "otolithic crisis of Tumarkin", from the original description of Tumarkin (1936). These are attributed to sudden mechanical deformation of the otolith organs (utricle and saccule), causing a sudden activation of vestibular reflexes. Patients suddenly feel that they are tilted or falling (although they may be straight), and bring about much of the rapid repositioning themselves. This is a very disabling symptom as it occurs without warning and can result in severe injury. Often destructive treatment (e.g. labyrinthectomy or vestibular nerve section) is the only way to manage this problem. Other otologic conditions also occasionally are associated with Tumarkin type falls (Ishiyama et al, 2003). See here for more information about drop attacks.
Ménière's episodes may occur in clusters; that is, several attacks may occur within a short period of time. However, years may pass between episodes. Between the acute attacks, most people are free of symptoms or note mild imbalance and tinnitus.
Menieres affects roughly 0.2% of the population (click here for more details about the epidemiology). Ménière's disease usually starts confined to one ear but it often extends to involve both ears over time so that after 30 years, 50% of patients with Meniere's have bilateral disease (Stahle et al, 1991). There is some controversy about this statistic however -- some authors, for example Silverstein, suggest that the prevalence of bilaterality is as low as 17% (Silverstein, 1992). We suspect that this lower statistic is due to a lower duration of followup and that the 50% figure is more likely to be correct. Other possibilities, however, are selection bias and different patterns of the disease in different countries. Silverstein suggested that 75% of persons destined to become bilateral do so within 5 years.
In most cases, a progressive hearing loss occurs in the affected ear(s). A low-frequency sensorineural pattern is commonly found initially, but as time goes on, it usually changes into either a flat loss or a peaked pattern (click here for more information about hearing testing). Although an acute attack can be incapacitating, the disease itself is not fatal.
What causes Ménière's Disease?
|Figure 2a: Normal membranous labyrinth||2b. Dilated membranous labyrinth in Meniere's disease (Hydrops)|
An acute attack of Ménière's disease is generally believed to result from fluctuating pressure of the fluid within the inner ear. A system of membranes, called the membranous labyrinth, contains a fluid called endolymph. The membranes can become dilated like a balloon when pressure increases. This is called "hydrops". One way for this to happen is when the drainage system, called the endolymphatic duct or sac is blocked. In some cases, the endolymphatic duct may be obstructed by scar tissue, or may be narrow from birth. In some cases there may be too much fluid secreted by the Stria Vascularis. Hydrops is not found in all persons with Meniere's disease, and hydrops is also commonly found (6%) on autopsy studies of persons who had no Meniere's type symptoms (Honrubia, 1999; Rauch et al, 2001). Because Meniere's disease occurs in roughly 0.2/100 persons, and Hydrops is found in 6/100 temporal bones, the there is roughly an order of magnitude more people with hydrops than Meniere's disease. Thus logically, there must be something more than simply hydrops involved in the origin of Meniere's disease (see following paragraph about immunologic function as well as page on autoimmune inner ear disease).
Abnormally enlarged fluid pathways into the ear such as the vestibular aqueduct or cochlear aqueduct may also be associated with Meniere's like symptoms. Recent evidence is against a relationship between the cochlear aqueduct and MD (Yilmazer et al, 2001). Enlarged vestibular aqueducts are one of the most commonly identified inner ear bony malformations in children with sensorineural hearing loss of unknown cause (Oh et al, 2001). The vestibular aqueduct extends from the medial wall of the vestibule to the posterior surface of the petrous temporal bone. Most persons with enlarged vestibular aqueducts with ear disorders have hearing loss, but occasionally there is an association with vestibular problems (Shessel and Nedzelski, 1992). There may be a genetic link between enlarged vestibular aqueduct and Pendred syndrome, which is a congenital thyroid disorder.
Recently attention has been mainly focussed on the immunologic function of the endolymphatic sac -- immune disease may contribute to a substantial percentage of Meniere's disease. However, for the most part the underlying cause of Menieres disease is unknown. It is most often attributed to viral infections of the inner ear, head injury, a hereditary predisposition, and allergy. Click here for more about the causes of Menieres disease.
What damage is done by Ménière's Disease?
1. Hair cell death: Conventional thought is that repeated attacks of Meniere's kills hair cells in the inner ear. This is a gradual process over years, but frequently resulting in unilateral functional deafness. Cochlear (hearing) hair cells are the most sensitive. Vestibular hair cells seem more resilient but there is also a slow decline in the caloric response in the diseased ear over roughly 15 years (Stahle et al, 1991).
2. Mechanical changes to the ear. Mechanical disruption of the inner ear is also likely with dilation of the utricle and saccule of the ear being a well known pathological finding. The saccule may dilate so that in later stages, it is adherent to the underside of the stapes footplate. This mechanical disruption and distortion of normal inner ear structures may result in the gradual onset of a chronic unsteadiness, even when patients are not having attacks. The periodic dilation and shrinkage of the utricle is also a reasonable explanation for periodic attacks of another inner ear disorder, BPPV. Finally, it also seems likely that there may be rupture of the suspensory system for the membranous labyrinth. This might create some mechanical instability of the utricle and saccule and consequently some chronic unsteadiness.
There is presently no evidence that Meniere's disease kills the cochleovestibular nerve (see Kitamura et al, 1997).
Studies have shown that Meniere's disease affects about 200 out of 100,000 people (or in other words, 2/1000). This is roughly the same prevalence as multiple sclerosis (MS). The majority of people with Ménière's disease are over 40 years of age, with equal distribution between males and females. Interestingly, the Framingham study found that 2/100 people believe they have Meniere's disease in the US, suggesting that there is considerable chance of misdiagnosis. (click here for more .)
At the present time there is no cure for Ménière's disease, but there are ways to manage the condition and help control symptoms.
Diagnosis is based on a combination of the right set of symptoms (usually episodic dizziness and hearing disturbance), hearing tests which document that hearing is reduced after an attack, and then gets better, and exclusion of alternative causes. The differential diagnosis is broad and includes perilymph fistula, recurrent labyrinthitis, migraine, congenital ear malformations of many kinds, syphilis, Lyme, tumors, MS, etc. The process of diagnosis usually includes hearing testing (audiometry), an ENG test, several blood tests (ANA, FTA), and an MRI scan of the head. Electrocochleography (ECOG) is helpful in difficult cases. As aural fullness can be caused by eustachian tube malfunction, tympanometry is sometimes useful.
Ruckenstein and others recently obtained a large number of blood tests in a group of 40 patients with unilateral Meniere's disease including CBC, ANA, Anti-Sjoegren, RF, Complement, antiphospholipid antibody, western blot for heat shock protein, MHA (syphilis test), and Lyme. Only elevations in the antiphospholipid antibody test were found 27% of patients, above the expected 6-9%. anti-heat shock protein was found in 6%. They concluded that it is unlikely that autoimmune etiologies play a significant role in their population of unilateral Menieres (Ruckenstein, 2002).
Hearing tests often begin with showing a fluctuating low-frequency sensorineural hearing loss. Over years, this gradually progresses to a "peaked" pattern with both low and high-tone reduction, and finally a "flat" pattern, typically 50 db loss (110 db would be completely deaf). While some people have hearing that fluctuates like this without any further symptoms of dizziness or tinnitus, in most cases, this does not progress to Meniere's disease (Schaaf et al, 2001). Some recent studies have suggested that the pattern and severity of the hearing deficit does not correlate with the duration of illness (Mateijsen et al, 2001), but it seems more likely to us that the hearing pattern is rather variable and that these authors simply did not study enough patients.
Medications commonly used for an acute attack include the following:
Additional information about prevention of vomiting is found here.
During an acute attack, lay down on a firm surface. Stay as motionless as possible, with your eyes open and fixed on a stationary object. Do not try to drink or sip water, as you'd be very likely to vomit. Stay like this until the severe vertigo (spinning) passes, then get up SLOWLY. After the attack subsides, you'll probably feel very tired and need to sleep for several hours.
If vomiting persists and you are unable to take fluids for longer than 24 hours (12 hours for children), contact your doctor. He can prescribe nausea medication, and/or vestibular suppressant medication. He may wish to see you. Antivert and Ativan are commonly used vestibular suppressant medications and Compazine or Phenergan are commonly used medications for nausea.
Agents that are controversial
Between attacks, medication may be prescribed to help regulate the fluid pressure in your inner ear, thereby reducing the severity and frequency of the Ménière's episodes. Dyazide is the most common medication for this purpose. Neptazine can be used when response to Dyazide isn't good enough. Verapamil (typical dose: 120 SR) sometimes reduces the frequency of attacks. Some physicians prescribe Histamine injections. Prednisone or other steroids (e.g. Decadron) are occasionally helpful in short bursts. Vestibular suppressants such as Antivert (meclizine) or Klonazepam are used on an as-needed basis.
There are a number of new medications that do not have much of a track record that can be tried under the supervision of your doctor. There also some unusual medications which are either considered "alternative" or which are available only outside the US which might be worth considering.
The hydrops diet regimen will probably be recommended (see following). This is an important part of treatment for virtually all patients with Ménière's disease. Experience has show that STRICT adherence to this dietary regimen will result in significant improvement in most patients.
The purpose of treatment between attacks is to prevent or reduce the number of episodes, and to decrease the chances of further hearing loss. A permanent tinnitus (ringing in the ears) or a progressive hearing loss may be the consequence of long-term Ménière's disease. Hearing aids may be necessary.
In extremely severe cases, treatments that deaden the inner ear such as gentamicin injections or surgery may be considered. This is a last resort for persons who have severe attacks which are disabling. At present, we favor gentamicin for most instances where destructive treatments are being considered. Injections of gentamicin are given through the ear drum, through a small hole or through a small tube. This procedure allows one treat one side alone, without affecting the other. Typically about four injections are given, over a period of a month. Dizziness may reoccur one year later, requiring another series.
Although intratympanic steroids injections have also been recently used to treat Menieres by Shea, the present consensus is that treatment has not yet been clearly proven to be effective or to have a reasonable scientific basis. Some studies suggest that it is no better than placebo (Silverstein et al, 1998). Others suggest that their results are about as good as gentamicin (SENNAROGLU et al, 2001). We are dubious at the present writing (end of 2002), as it is difficult to understand why they should work.
Alternatively, a surgical treatment is used in which the vestibular nerve is clipped. This operation, called a vestibular neurectomy or vestibular nerve section is very effective in eliminating vertigo. While very effective, this procedure, especially the hearing-sparing variant, is presently generally felt to be of much higher risk than gentamicin injection. We presently do not favor vestibular nerve section, except in situations where gentamicin injection has failed.
Another operation, called a labyrinthectomy is recommended in persons who have lost all usable hearing or in whom vestibular nerve section is considered too dangerous. Again, this procedure seems most applicable to situations where gentamicin has failed.
A third procedure, the endolymphatic shunt procedure, is used by some doctors to relieve pressure in the inner ear. Unfortunately, while the shunt would seem to be a logical thing to do, in most studies, the shunt procedure does not appear to be better than doing nothing (e.g. Silverstein and Rosenberg, 1992). This may be because the shunt can easily get plugged up. We do not presently recommend this procedure for our patients except in unusual situations. These may include a person who has perfectly normal hearing, or an older person who might not tolerate the gentamicin procedure described above (Pensak and Friedman, 1998; Gianoli et al, 1998). A recent trend is to make attempt to damage the sac, in addition to improving its drainage. As the sac is the immune organ of the ear, this idea makes sense if one is attempting to immunosuppress the ear.
There is a mixed literature about implantation of ventilation tubes. Eustachian tube malfunction does not appear to generally modulate Meniere's disease (Maier et al, 1997). However, in the "Danish Sham Study", ventilation tubes were felt to be helpful.
For bilateral Meniere's disease, when the patient is incapacitated and it cannot be determined which ear is causing the dizziness, intramuscular streptomycin (1 gm twice/day) can be given on an outpatient basis until the first sign of ototoxicity develops. This treatment can generally reduce or eliminate vertigo spells without affecting hearing. This treatment however damages the inner ear and causes bilateral vestibular paresis, which has it's own set of symptoms and disability.
Surgical treatment have not been shown to preserve hearing to any greater extent than medical treatments (Kinney et al, 1996). A review of surgical treatment has recently been published by LaRouere (see references).
Since the acute symptoms of Ménière's disease are episodic, it is important to explain to your family and friends what might happen when you have an attack. Then, if the symptoms occur when they are present, they will understand and not be overly frightened.
You may be able to protect yourself from injury if you feel that an attack is about to begin. Some attacks may occur during the night, so be sure you have a night light on; you'll be relying more on vision to help maintain your balance. You will want to make sure that the path to the bathroom is free of throw rugs, furniture or other obstructions.
Many studies have documented that patients with Menieres disease tend to have more psychological disability than the normal population, possibly including depression and/or anxiety, in reaction to their disease. It may be necessary to take antidepressants or anti-anxiety drugs, under the supervision of an appropriate health care professional.
The fluid-filled hearing and balance structures of the inner ear normally function independent of the body's overall fluid/blood system. In a normal inner ear, the fluid is maintained at a constant volume and contains specific concentrations of sodium, potassium, chloride and other electrolytes. This fluid bathes the sensory cells of the inner ear and allows them to function normally.
With injury or degeneration of the inner ear structures, independent control is lost, and the volume and concentration of the inner ear fluid fluctuates with changes in the body's fluid/blood. This fluctuation causes the symptoms of hydrops--pressure or fullness in the ears, tinnitus (ringing in the ears), hearing loss, dizziness and imbalance.
How does what I eat affect my dizziness?
Your inner ear fluid is influenced by certain substances in your blood and other body fluids. For instance, when you eat foods that are high in salt or sugar, your blood level concentration of salt or sugar increases, and this, in turn, will affect the concentration of substances in your inner ear.
People with certain balance disorders must control the amount of salt and sugar that is added to food. You must also become aware of the hidden salts and sugars that foods contain. Limiting or eliminating your use of caffeine and alcohol will also help to reduce symptoms of dizziness and ringing in the ears.
The goal of treatment is to provide stable body fluid/blood levels so that secondary fluctuations in the inner ear fluid can be avoided.
Where can I get additional help in modifying my diet?
Dietitians can help you work out a nutritional program which meets your special needs. They can also suggest ways to prepare your favorite foods for a restricted-salt or low-sugar diet. With their assistance, you'll find that modifying your eating habits can help you control the symptoms of your balance disorder.
There are several excellent books which can help in determining which foods are high in salt. Barbara Kraus's "Complete Guide to Sodium" (Signet, 1987) is a paperback book which lists most foods.
Written By: Timothy C. Hain, MD
Acknowledgements: Figures are courtesy of Northwestern University.Return to Index