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Timothy C. Hain, MD 
Page last modified:
October 7, 2008
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Fig 1. Normal inner ear. |
In 1861, the French physician Prosper Meniere described a condition which now bears his name. Meniere'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 Meniere's disease is preceded by fullness in one ear. Hearing
fluctuation or changes in tinnitus may also precede an attack. A Meniere'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. High
sensitivity to visual stimuli (visual dependence) is common. (Lacour, 1997). During the attack the
eyes jump (this is called "nystagmus"). 
Supplemental
material on the site DVD: Movie of nystagmus
during a Meniere's disease attack
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| Fall due to otolithic crisis of Tumarkin. This is a very dangerous variant of Meniere's disease, which can result in abrupt falls. |
A particularly disabling symptom is a sudden fall. These typically occur without warning. These falls are called "otolithic crisis of Tumarkin", from the original description of Tumarkin (1936). They 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 (Black et al, 1982; Ishiyama et al, 2003). See here for more information about drop attacks.
Meniere'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.
Meniere's affects roughly 0.2% of the population (click here for more details about the epidemiology). Meniere'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 follow up 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 Meniere's Disease? (Click here for a more detailed discussion)
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Figure 2a: Normal membranous labyrinth |
2b. Dilated membranous labyrinth in Meniere's disease (Hydrops) |
The origin of Meniere's disease is presently controversial. While in the past, it was felt that plumbing problems (hydrops) in the ear were responsible for the disease, the most current opinion is that the plumbing problems are are just a marker for the Meniere's disease, rather than necessarily being responsible for the symptoms.
Traditional thinking on the origin of Meniere's disease : The most prevalent opinion is that an acute attack of Meniere's disease results 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.
Besides a stoppage up of drainage pathways, 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.
More recent thought on the origin of Meniere's disease. On the other hand, 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, there is more than 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).
Recently attention has also been focused on the immunologic function of the endolymphatic sac -- immune disease may contribute to a substantial percentage of Meniere's disease. We ourselves hold this opinion, based in part on a study in which patients with Meniere's disease had a very high prevalence (about 25%) of autoimmune thyroid disease (Brenner et al, 2004). On the other hand, Ruckenstein and others recently obtained a large number of autoimmune 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 Meniere's (Ruckenstein, 2002). Another interpretation of their data is that they may simply not have had an appropriate marker for autoimmune involvement, as well as unilateral Meniere's may have less autoimmune cause than bilateral.
Thus, the bottom line is that in most patients with Meniere's disease in 2005, the underlying cause of Meniere's 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 Meniere's disease.
What damage is done by Meniere'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). Thus the term "nerve deafness" is inappropriate.
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 Meniere'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 misdiagnosis is far more common than the correct diagnosis. (click here for more)
At the present time there is no cure for Meniere's disease, but there are ways to manage the condition and help control symptoms. Some recently refined treatments are rather close to a cure (i.e. low dose gentamicin). While some have suggested that Meniere's may "burn out", recent studies suggest that this is not likely (Havia and Kentala 2004)
How does the doctor know I have Meniere's disease ?
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| Figure 3a: Audiogram (hearing test) typical of early Meniere's disease on the right side (x=left, o=right). There is a low-tone sensorineural hearing loss. | Figure 3b: Audiogram typical of middle-stage Meniere's disease, again on the right side. Hearing is reduced at all frequencies, but more so at high and low frequencies. | Figure 3c: Audiogram typical of late-stage Meniere's disease, again on the right side. Hearing is flat, and unaidable on the right side. |
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 disease, tumors such as acoustic neuroma, multiple sclerosis, posterior fossa arachnoid cysts, and other rare entities. Symptoms similar to Meniere's (fluctuating hearing, tinnitus, vertigo) can also be caused by impending strokes in the distribution of the anterior inferior cerebellar artery (Lee and Cho, 2003). Bilaterality of hearing fluctuation suggests a vascular cause such as migraine.
The process of diagnosis usually includes hearing testing (audiometry), an ENG test, a VEMP 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.
Hearing tests often begin with showing a fluctuating low-frequency sensorineural hearing loss (figure 3a). Over years, this gradually progresses to a "peaked" pattern with both low and high-tone reduction (figure 3b), and finally a "flat" pattern, typically 50 db loss (110 db would be completely deaf). After about 10 years of Meniere's disease, hearing often looks like that of figure 3c, where the "peak" has become a flat sensorineural loss.
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 from our experience, 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 immediately, 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/she may wish to see you or even admit you to the hospital if you are dehydrated. Meclizine (Antivert), Lorazepam and Clonazepam are commonly used vestibular suppressant medications and Compazine or Phenergan are commonly used medications for nausea.
Notes: as triamterine is a folate antagonist, pregnant women should take folate supplements if not otherwise contraindicated. Occasionally persons on long-term acetazolamide develop kidney stones. All of the above have sulfa in them, which persons with sulfa allergies may be unable to tolerate. In this situation, one may try amiloride by itself, or furosemide (Lasix). Laxix should be used in low doses and with caution because it is mildly ototoxic itself.
Vestibular Suppressants (click here for more details on drug treatments)
Steroids (commonly for severe bouts)
The purpose of treatment between attacks is to prevent or reduce the number of episodes, and to decrease the chances of further hearing loss and damage to the vestibular system. A permanent tinnitus (ringing in the ears), constant imbalance, or a progressive hearing loss may be the consequence of long-term Meniere's disease. Hearing aids may be necessary.
Standard medical treatments:
The hydrops diet regimen will probably be recommended (see following). This is an important part of treatment for virtually all patients with Meniere's disease. Experience has show that STRICT adherence to this dietary regimen will result in stabilization in most patients.
Between attacks, diuretic medication may be prescribed to help regulate the fluid pressure in your inner ear, thereby reducing the severity and frequency of the Meniere's episodes. Dyazide (a combination of triamterine and hydrochlorothiazide) is the most common medication for this purpose, and others are listed above. The literature suggests that diuretics are modestly helpful (Horner et al., 1989; Santos et al, 1993; Shinkawa and Kimura, 1986).
Vestibular suppressants such as Antivert (meclizine) or Clonazepam, and anti-emetics (e.g. phenergan) are used on an as-needed basis.
Not so standard treatments
Verapamil (typical dose: 120 SR) sometimes reduces the frequency of attacks. Nimodipine and Flunarizine have also been used. These medications are all calcium channel blockers. The evidence is not as good that these medications work. Because Menieres and Migraine are very often combined, this medication's main role may be to treat the migraine associated vertigo that can be confused with Menieres disease. This medication is especially logical if the dizzy attacks are associated with headaches.
Some physicians prescribe Histamine injections. Most physicians in the US, however, consider this treatment to be ineffective.
Prednisone or other steroids (e.g. Decadron) are occasionally helpful in short bursts. We would most often use these when considering a destructive treatment.
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.
One way to do it:
What the author recommends in his practice in Chicago Illinois for medical prevention of Meniere's. These drugs are administered to most of his patients, generally in the following sequence:
These are combined with symptomatic drugs such as meclizine, benzodiazepines, and antiemetics, to be taken during attacks.
In persons with severe progressive bilateral disease, the author will generally recommend a trial of Enbrel, with the thought that in this situation an autoimmune disorder is more probable.
In persons who do not respond to this, we move on to low-dose gentamicin (see "last resort" section), and then destructive treatment.
An emerging treatment that may eventually end up as a standard treatment is multiple injections of steroids into the middle ear (see "emerging" section below).
The main role of surgical treatment is to manage intractable vertigo. 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 (1996).
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| Trans (intra) tympanic injection of Gentamicin for control of intractable Meniere's disease. This method can also be used to inject steroids (see below). |
Gentamicin for Meniere's that has not responded to medical management.
In severe cases of Meniere's disease, 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 disabling attacks. 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.
While in the past, about four injections are given, over a period of a month, we recently have seen very good results with just one or two injections. This "low-dose" gentamicin protocol has become part of our standard staged management.
Dizziness may reoccur one year later, requiring another series. This is because the gentamicin may only temporarily damage the hair cells, which can recover.
Nerve section for unilateral Meniere's that has not responded to low or high-dose gentamicin.
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 and hearing is already gone. In persons with unilateral disease, one can combine a labyrinthectomy with a BAHA hearing device, and end up with more usable hearing after the labyrinthectomy.
Intramuscular streptomycin for Bilateral Meniere's disease that has not responded to low dose gentamicin.
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 (generally about a month). 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. This treatment is extremely rare as well as rather arduous.
Intratympanic steroid injections have been recently used to treat Meniere's (e.g. Shea and Ge in Otol Clin NA, 1996, 29:353-8, also Am J Otol,18:4,1997). The core methodology is to inject in enough dexamethasone solution (24 mg/ml) to fill the middle ear, on either a one-time basis or over several sessions separated by weeks or months. Thus it involves both making a hole in the ear drum (with the needle), as well as administering a drug to the inner ear (by diffusion across the round window membrane).
Use by otologic surgeons of this methodology is growing and papers supporting its use are proliferating very rapidly (as of 2007). Not all methods seem to work -- some comments about variant methods follows. This page -- intratympanic steroid injections -- has a little more detail.
Studies using a brief period of administration, suggest that it is no better than placebo (Silverstein et al, 1998). As steroids are neither curative nor long-lasting agents, brief intratympanic steroid treatments are obviously not able to cure Meniere's disease (Barrs, 2004; Dodson et al, 2004). The present consensus is that one-time treatment has not yet been proven effective (Doyle et al, 2005) or to have a reasonable scientific basis. The core scientific difficulty is that steroids don't make any permanent alterations to the inner ear, and that they are gone from the ear in a short period of time (Harugnani et al, 2006). Thus, the hypothesis that they can "cure" a chronic ear disorder is very difficult to understand.
Studies using a long term protocol, or an "as needed" methodology (no more than 4 injections/year) suggest that their results are very good (Sennaroglu et al, 2001; Boleas et al, 2007). This makes more sense to us. If this methodology does indeed prove out, then the main issue would be to determine where this methodology belongs in the staged management of Menieres. Perhaps, after medical and prior to gentamicin treatment.
Other variant methods of administration of steroids.
Intratympanic steroids combined with a viscous osmotic agent (hyaluronic acid) that reduces endolymph volume, has also been recently reported in an uncontrolled study to be effective (Selivanova et al, 2005) in persons who have failed intravenous steroids and vasodilators. They used a protocol of every other day injections, with a maximum of 7 total, combining 8mg of dexamethasone and a 0.2 mg/ml solution of hyaluronic acid. In our view, this is simply the combination of two short-lived treatments, but because hyaluronic acid is very viscous, the combination may keep the steroids in the ear for longer periods than simple injections of an aqueous solution, and might have better results due to this effect. A comparison between steroids injected with and without hyaluronic acid would be a reasonable next step.
Bottom line: At the present writing (2007), we feel that
(we have moved most of the (long) placebo discussion to a separate page).
The big problem with this section is separating placebo's from slightly effective treatments. This intrinsicially involves a cost-benefit comparison.
There are an immense number of placebo and probable or nearly placebo treatments for Meniere's disease. There is nothing wrong with a placebo, if it provides some benefit (presumably psychological), and you can afford to do this. However, we don't think that placebos that are billed to health care insurance providers should be supported. The treatments briefly discussed below are (probably) not placebos, but they are high in cost (because they are surgical), and low in benefit (because the literature suggests low or no efficacy).
The endolymphatic shunt is probably a placebo treatment, although it has continues to be recommended by those who put them in (otologic surgeons). We have never recommended a shunt in our (non-surgical) practice -- we prefer low-dose gentamicin. There is a large literature about the effect (or lack of effect) of this surgical treatment. Again, it seems to be a "slightly effective" procedure.
The Meniett device's status at this writing (2006) it again seems most likely to be a slightly effective treatment. This treatment requires a surgical procedure (insertion of a ventilation tube) and purchase of a very expensive device (the pressure machine). We do not recommend Meniett treatment in our practice. We prefer low-dose gentamicin after medication fails.
Here is what a patient who had a good experience with the Menniet told me.
For intractable cases, we often presently advise a low dose gentamicin protocol. Gentamicin is adminstered through the ear drum every month for a total of one or two administrations. We have had very good results with this procedure, and no hearing loss as yet. If this fails, then if symptoms are severe we may follow with a labyrinthectomy/BAHA combination, or in patients with good hearing and good health, vestibular neurectomy (see "well accepted surgical treatment section above).
In our opinion, medical treatments, including a reasonable trial of anti-migraine drug, should come first.
Meniere's disease has a severe impact on people's lives. In acute episodes, Meniere's disease is one of the most debilitating disease experienced by people who survive any illness (Anderson and Harris, 2001). Meniere's may persist for 20 years or more. It is generally a chronic disease (Havia et al, 2004).
Since the acute symptoms of Meniere'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 Meniere's disease tend to have more psychological disability than the normal population, possibly including depression and/or anxiety. This is generally a reaction to their disease (Savastano et al, 2007). 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.
Note that it is not the overall level of sodium that is important, but whether or not it fluctuates. It is not necessary or wise to lower your salt intake to amounts barely able to sustain life. We do not encourage use of 1 gram sodium diets. Rather the goal is to keep sodium levels from fluctuation. A 2 gram diet is usually possible.
Self-help groups.
Written By: Timothy C. Hain, MD of Chicago Dizziness and Hearing.
(acknowledgement to source of graphics)
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