Timothy C. Hain, MD Page last modified: July 18, 2016
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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. Several committees have attempted to codify the symptoms. We think Meniere's disease should more properly be called Meniere's syndrome, but we will use the two terms as if they were the same. A closely related condition called "delayed endolymphatic hydrops", is probably just Meniere's under another name.
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 or "attack" generally involves severe vertigo (spinning), imbalance, nausea and vomiting as well as acute reduction of hearing. 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 variant is the "invisible hand", where individuals feel as if they are being pushed over. 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
|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, a variant of "drop attacks", 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.
|Age at first visit to Chicago Dizziness and Hearing clinic.|
Meniere's affects roughly 0.2% of the population (click here for more details about the epidemiology). In our clinical practice, where we care for large numbers of Meniere's patients, the most common age of presentation is in the 6th decade. This probably reflects the age distribution of Meniere's in Chicago Illinois, which of course is also a function of the age of the population in Chicago. It is a little older than the typical age of presentation of vestibular migraine which is in the 5th decade, much older than the reported highest prevalence age for non-vestibular migraine (about 35), and similar to the age of presentation of BPPV. The ratio of women to men in our Meniere's population is about 3:2.
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 Meniere's disease is not fatal.
What causes Meniere's Disease? (Click here for a more detailed discussion)
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 hydrops is 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, as might be true in some cases of the closely related condition of "delayed endolymphatic hydrops", or may be narrow from birth. In some cases there may be too much fluid secreted by the stria vascularis.
Besides a stoppage 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 pages on autoimmune inner ear disease and allergy in Meniere's 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.
One can also build a reasonable case for Migraine being the cause of (some) cases of Meniere's disease. Migraine (about 1/6 people) is almost 2 orders of magnitude more common than Meniere's disease (about 1/2000 people), and due to this migraine variants with prominent ear symptoms are more common than Meniere's disease. Patients with Meniere's have migraine also, about 50% of the time. Meniere's disease hearing loss does not follow the expected pattern for an inner ear disease (i.e. loss of high frequencies first, and loss of OAE's). Non-drug treatment of Meniere's (i.e. diet), is almost identical to diets recommended for Migraine.
Thus, the bottom line is that in most patients with Meniere's disease in 2015, 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. Migraine may cause symptoms that overlap with Meniere's disease, or in some, may even be identical to Meniere's disease, at the level that we can resolve disorders in the clinic (i.e. without an autopsy).
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.
3. Some investigators suggest that experimental Meniere's disease kills the cochleovestibular nerve through neurotoxicity in mice and guinea pigs (Megarian et al, 2005). However, the evidence is against this being the main mechanism in human beings (see Kitamura et al, 1997; Nadol et al, 1995). Rather, it seems that Meniere's disease damages both hair cells and nerve fibers in humans. Studies of humans are handicapped by the possibility that Meniere's is the final common pathway of a variety of illnesses of the ear.
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", research studies suggest that this is not likely (Havia and Kentala 2004)
How does the doctor know I have Meniere's disease ?
|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. Several committees have offered diagnostic criteria -- these are reviewed in the link. We continue to favor the AAO 1995 criteria for "definite" Meniere's disease.
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.
Occasionally a "wrong way" (irritative) nystagmus is seen in Meniere's disease. Essentially, the nystagmus jumps towards the bad side. This is a highly specific finding to Meniere's disease. Potential reasons are an exitatory nystagmus, Bechterew's phenomenon (recovery nystagmus), Meniere's involving the opposite ear, and stronger vestibular responses on the side with hydrops due to hydrodynamic factors associated with hydrops.
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 (ECochG) is often helpful, although it remains controversial. As aural fullness can be caused by eustachian tube malfunction, tympanometry is sometimes useful.
The "glycerol test" is an older diagnostic test that depends on detecting improvement of hearing, 4 hours after oral administration of glycerol (Basel and Lutkenhoner, 2012). This test is not commonly used.
Recently the results of high resolution MRI's have been reported in Meniere's disease, with suggestions that contrast enhanced high-resolution "Fiesta" studies using 3T units can document hydrops. At this writing (2016), we feel that this technology is promising and may be useful. Should MRI scanners become even more powerful in the future, this could be an important test. Another new MRI approach involves injecting gadolinium through the ear drum, and then a MRI scan (Gu et al, 2014). This technique is plausible, but we are doubtful that the diagnostic gain justifies injecting gadolinium dye into the middle ear.
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. Oddly, there is sometimes a discrepency between OAE tests and audiometry in the low-frequency SNHL of Meniere's. Perhaps this means that the low-frequency SNHL is not due to cochlear damage. Recently, it has become possible to test ones hearing with cell-phone apps. This may help with the diagnosis process.
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 authors have suggested that the pattern and severity of the hearing deficit does not correlate with the duration of illness (Mateijsen et al, 2001), but this does not match are very large our experience. It seems more likely that the Meniere's hearing pattern is variable and that these authors simply did not study enough patients. Those who present to the doctor with more severe hearing impairments at onset, usually do worse than those who have milder hearing impairments (Sato et al, 2014).
A set of tests from the same patient showing classic Meniere's is shown here.
A longer discussion of the information to be gained in testing of persons with Meniere's disease is found here.
This is the way that we manage acute attacks at Chicago Dizziness and Hearing
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/She 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, Phenergan or Ondansetron are commonly used medications for nausea. In our practice in Chicago, we commonly prescribe an "emergency kit", consisting of a small prescription of lorazepam and ondansetron, to be taken sublingually for an acute attack.
In the United States, Dyazide (triamterine/HCTZ) is prescribed almost universally for Menieres. Maxide is used when a smaller dose than found in Dyazide is needed (it is scored). Van Deelen and Huizing studied the use of diuretics in Meniere's disease in a double-blind, placebo controlled trial, and reported that it reduces vestibular complaints, but has no significant effect on hearing (1986). Thirlwall and S. Kundu (2006) were unable to come to a conclusion as to efficacy as no papers published up to 2006 were adequate for meta-analysis. Crowson et al (2016) reported that all studies were of "low evidence level" and that they consistently report reduction in vertigo. While these authors appear to be "bashing" diuretics, it is important to remember that lack of evidence is not the same as evidence of lack. Our observation in our large clinic Menieres population is that about 1/3 of Meniere's patients report a good response, and the remaining aren't sure whether it is doing them any good at all.
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 diuretics have sulfa in them, which persons with sulfa allergies may be unable to tolerate.
Diuretics that do not contain sulfa (Ponka, 2006):
- ethacrynic acid
When there is sulfa allergy, one may try amiloride by itself, or ethacrinic acid (edecrin). Loop diuretics such as Edecrin should be used in low doses and with caution because they are ototoxic. Note that the diuretics listed are mainly ones that increase serum potassium. (Ponka, 2006).
Vestibular Suppressants (click here for more details on drug treatments)
Note that antithistamines that do not cross into the brain are not used because they don't work -- i.e. loratidine, cetirizine, fexofenadine
Calcium Channel Blockers
Steroids (commonly for severe bouts)
Agents that are controversial
This is the way that we attempt to prevent Meniere's attacks at Chicago Dizziness and Hearing:
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:
Not so standard treatments
What the author recommends in his practice in Chicago 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 high dose steroids. If there is a significant improvement in hearing, then there is an attempt to switch to Enbrel (an immunosuppressant with less side effects than steroids), with the thought that in this situation an autoimmune disorder is more probable. Of course this strategy should rationally be adjusted to the variability in hearing, as in any undertaking, one's confidence that a particular result has been obtained depends both on the size of the effect as well as the underlying variability in the effect. Much room for improvement in these algorithms is needed -perhaps involving daily hearing monitoring using an internet protocol.
In persons who do not respond to medical management, we move on to low-dose gentamicin (see flowchart and "last resort" section), and then definitive destructive treatment.
An emerging treatment that may eventually end up as a standard treatment is multiple injections of steroids into the middle ear. We have encountered individuals who have had as many as 40 of these injections. There is intrinsic risk of TM perforation and infection.
(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 intrinsically 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 Meniett device's status at this writing (2010) 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 Meniett told me.
There is a separate page on this topic, but briefly, for intractable unilateral Menieres disease cases, we often presently advise a low dose gentamicin protocol. Gentamicin is administered 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).
Persons who have intractable bilateral Meniere's disease are offered a trial of steroids with a safer immunosuppressant should this be effective, a modified gentamicin protocol, and very occasionally systemic aminoglycosides to deaden both ears.
In our opinion, medical treatments, including a reasonable trial of anti-migraine drugs such as verapamil or venlafaxine, should precede use of any last resort treatment.
Pregnancy is thought to have no net effect on Meniere's disease. The difficulty in pregnancy is that there should be an effort to avoid medications that might cause birth defects or otherwise injure the unborn child.
In persons who are unfortunate enough to have a flare during pregnancy, we attempt to manage them with salt restriction, minimal use of meclizine and/or ondansetron. In persons with severe symptoms, we suggest use of intratympanic steroids (dexamethasone).
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 30 years or more. It is generally a chronic disease (Havia et al, 2004).
Hearing is thought to gradually decline throughout life, with roughly 50 dB loss in 10 years. Vertigo spells are generally thought to persist for decades, although with a gradual decline after roughly the first decade (Huppert et al, 2010). Bilaterality of the condition gradually increases with time, but in most the symptoms in the opposite ear are minor.
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). Disability is mainly correlated with vertigo (Soto-Varela et al, 2015). 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 that is important in avoiding attacks of dizziness. 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. This web site: http://www.myfitnesspal.com has free software that can help tracking sodium.
Written By: Timothy C. Hain, MD of Chicago Dizziness and Hearing.Return to Index