Ménière's disease

Ménière's disease
Inner ear
Classification and external resources
Specialty	otolaryngology
ICD-10	H81.0
ICD-9-CM	386.0
OMIM	156000
DiseasesDB	8003
MedlinePlus	000702
eMedicine	emerg/308
MeSH	D008575
Orphanet	45360

Ménière's disease /meɪnˈjɛərz/,^[1] is a disorder of the inner ear that usually affects both hearing and balance. It is characterized by episodes of vertigo and by fluctuating or permanent tinnitus and hearing loss. The condition affects people differently. It can range in intensity from being a mild annoyance to a disabling disease.

The condition is named after the French physician Prosper Ménière, who in an article from 1861 described the main symptoms and was the first to suggest as a single cause for all of them a disorder in the combined organ of balance and hearing in the inner ear.^[2]

The immediate cause of Ménière's disease is endolymphatic hydrops, an excess of fluid in the inner ear. The possible causes of endolymphatic hydrops, in turn, are not well understood. For this reason a causal treatment of endolymphatic hydrops - and thus also for Ménière's disease - does not exist.

However, episodes of vertigo usually subside as the illness progresses or stabilizes, and most patients learn to manage tinnitus and hearing loss. This even applies to patients who are affected in both ears from some point in the course of their lives (ca. 30%).

Signs and symptoms

Audiograms illustrating normal hearing (left) and unilateral low-pitch hearing loss associated with Ménière's disease (right).

Ménière's is characterized by recurrent episodes of vertigo, hearing loss and tinnitus. Though it often begins with a single symptom, the disease gradually progresses. Not all symptoms must be present to confirm the diagnosis,^[3] but experiencing several of the typical symptoms at once greatly increases the likelihood that a diagnosis of Ménière's is correct. The diagnosis of Ménière's disease is made only if patients complain of both episodic vertigo and episodic sensorineural hearing loss. While these symptoms could be related to a variety of ear-related illnesses, Ménière's disease is characterized by the occurrence of 2-3 symptoms at the same time, in discrete "episodes".^[4] Conditions with partly similar symptoms - but no connection to Ménière's disease - include syphilis, Cogan's syndrome, autoimmune inner ear disease, dysautonomia, perilymph fistula, multiple sclerosis, acoustic neuroma, and both hypo- and hyperthyroidism.^[5]

Ménière's symptoms vary. Not all sufferers experience the same symptoms. However the "classic" presentation of Ménière's has the following three symptoms:^[6]

• vertigo Attacks of rotational vertigo can be severe, incapacitating, unpredictable, and last anywhere from minutes to hours.^[7] Generally the vertigo lasts no longer than 24 hours. For some, prolonged attacks can occur, lasting from several days to several weeks, often severely incapacitating the sufferer.^[8] This typically combines with increased tinnitus and temporary, albeit significant, hearing loss. The hearing loss may improve once the attack has subsided, but often becomes progressively worse and permanent over time.^[9]
• hearing loss Fluctuating, progressive, unilateral (in one ear) or bilateral (both ears) hearing loss, usually of lower frequency sound.^[10] For some, sounds may seem tinny or distorted, and patients may experience unusual sensitivity to sound (hyperacusis).^[11]
• tinnitus Unilateral or bilateral tinnitus or the perception of a persisting ringing sound.

Some patients may also experience a sensation of fullness or pressure to one or both ears. They may also experience additional symptoms related to irregular reactions of the autonomic nervous system. These symptoms are not symptoms of Meniere's disease per se, but rather are side effects resulting from failure of the organ of hearing and balance, and include nausea, vomiting, and sweating—which are typically symptoms of vertigo, and not of Ménière's. Vertigo may induce nystagmus, or uncontrollable rhythmical and jerky eye movements, usually in the horizontal plane, reflecting the essential role of the non-visual input by the organ of balance in coordinating eye movements.^[12]

Sudden falls without loss of consciousness (drop attacks, also known as Tumarkin attacks) may be experienced by some people, usually in the later stages of the disease.^[13][14] Less than 10% of people with Ménière's disease tend to experience such attacks. There is typically a sensation of being pushed sharply to the floor from behind (this is thought to be triggered by a sudden mechanical disturbance of the otolithic membrane that activates motoneurons in the vestibulospinal tract).^[15] The affected person is able to get up again immediately afterwards.^[15]

Cause

Ménière's disease is linked to endolymphatic hydrops, an excess of fluid in the inner ear.^[16] The membranous labyrinth, a system of membranes in the ear, contains a fluid called endolymph. In Ménière's disease, endolymph bursts from its normal channels in the ear and flows into other areas, causing damage. This accumulation of fluid is referred to as "hydrops". The membranes become dilated (stretched thin, like a balloon) when pressure increases and drainage is blocked.^[17] This may be related to swelling of the endolymphatic sac or other tissues in the vestibular system of the inner ear, which is responsible for the body's sense of balance.

Ménière's disease affects about 190 people per 100,000.^[18] Recent gender predominance studies show that Ménière's tends to affect women more often than men.^[18] In three nationwide surveys in Japan on Ménière's disease between 1975 and 1990 the data of 958 definite Ménière cases were analyzed. Onset of the disease peaked in the forties for males and thirties for females. A higher incidence was noted "in people with a nervous and precise character".^[19]

Diagnosis

Doctors establish a diagnosis with complaints and medical history. However, a detailed otolaryngological examination, audiometry, and head MRI scan should be performed to exclude a vestibular schwannoma or superior canal dehiscence, which would cause similar symptoms. Some of the same symptoms also occur with benign paroxysmal positional vertigo (BPPV), and with cervical spondylosis (which can affect blood supply to the brain and cause vertigo).

Ménière's disease is idiopathic and therefore a diagnosis of exclusion, meaning there is no definitive test for Ménière's; it is only diagnosed when all other possible causes of the patient's symptom have been ruled out.^[20]

History

Ménière's disease had been recognized as early as the 1860s, but the definition was still relatively vague and broad at the time. The American Academy of Otolaryngology-Head and Neck Surgery Committee on Hearing and Equilibrium (AAO HNS CHE) set criteria for diagnosing Ménière's, as well as defining two sub categories of Ménière's: cochlear (without vertigo) and vestibular (without deafness).^[21]

In 1972, the academy defined criteria for diagnosing Ménière's disease as:^[22]

1. Fluctuating, progressive, sensorineural deafness.
2. Episodic, characteristic definitive spells of vertigo lasting 20 minutes to 24 hours with no unconsciousness, vestibular nystagmus always present.
3. Tinnitus (ringing in the ears, from mild to severe) Often the tinnitus is accompanied by ear pain and a feeling of fullness in the affected ear. Usually the tinnitus is more severe before a spell of vertigo and lessens after the vertigo attack.
4. Attacks are characterized by periods of remission and exacerbation.

In 1985, this list changed to alter wording, such as changing "deafness" to "hearing loss associated with tinnitus, characteristically of low frequencies" and requiring more than one attack of vertigo to diagnose.^[23] Finally in 1995, the list was again altered to allow for degrees of the disease:^[24]

1. Certain - Definite disease with histopathological confirmation
2. Definite - Requires two or more definitive episodes of vertigo with hearing loss plus tinnitus and/or aural fullness
3. Probable - Only one definitive episode of vertigo and the other symptoms and signs
4. Possible - Definitive vertigo with no associated hearing loss

Management

Several environmental and dietary changes are thought to reduce the frequency or severity of symptom outbreaks. It is believed that since high sodium intake causes water retention, a diet high in salt can lead to an increase (or at least prevent the decrease) of fluid within the inner ear, although the relationship between salt and the inner ear is not fully understood.^[25] Thus, a low sodium diet is often prescribed, with sodium intake reduced to one to two grams of sodium per day^[25] (equivalent to approximately 2.5 to 5 grams of table salt, or a little more than one third to two thirds of a teaspoon). By comparison, the recommended Upper Limit (UL) for sodium intake is 2.3 grams per day,^[26] and most people are recommended to consume less than 1.5 grams,^[26] but on average people in the United States consume 3.4 grams per day.^[27]

Low-sodium, high-potassium dietary intake has been recommended on the basis of physiological experiments.^[28] The WHO generally recommends for adults a daily potassium intake of 3.5 g.^[29]

Getting adequate sleep and avoiding stress is an important aspect of managing the symptoms of Ménière's disease. Additionally, patients are advised to avoid alcohol, caffeine, and tobacco, as well as large quantities of chocolate or salt, all of which can aggravate Ménière's symptoms.^[30]

Although a causal relation between allergy and Menière's disease is uncertain, it has been recommended that allergy control is part of the treatment for Menière's disease in patients with a history of seasonal or food allergy, childhood or family history of allergy, or a development of symptoms within a short time after exposure to food or inhaled allergen.^[31]

In order to reduce nausea and vomiting during an episode, antihistamines such as meclizine are used.^[32]

Equalizing pressure in middle and inner ear

A man performs the Valsalva maneuver, while the eardrum of his right ear is examined with an otoscope.

The systems of pressure equalization of the inner ear are coupled in a highly complex way to the permanently ongoing variations of static pressure in the air chamber in the middle ear. Importantly, the inner ear pressure equalization is activated by these middle ear pressure changes. The details of these mechanisms have been described both physiologically^[33] and anatomically.^[34]

Middle ear pressure is determined primarily by a slow mechanism, the gas exchange with middle ear tissues, and secondarily by a fast mechanism, the transient opening of the Eustachian tube (auditory tube) that links the nasal spaces (nasopharynx) to the middle ear. The fast mechanism is normally triggered automatically, e.g., when chewing or yawning, but it can also be started voluntarily, e.g., when flying or diving (ear clearing).

In Ménière's patients the natural pressure regulation of the middle ear is clearly worse than normal^[35] and already in 1988 it was shown that additional ventilation of the middle ear could prevent Ménière's attacks.^[36] In 1997 it could even be established experimentally that additional ventilation of the middle ear indeed is effective against the development and progression of endolymphatic hydrops, the pathology preceding and immediately causing Ménière's disease.^[37]

The method of choice for voluntary ventilation of the middle ear is the Valsalva maneuver.^[38] It can be applied nearly anywhere spontaneously and without tools and is preferably combined with a following equalization by yawning or chewing (soft plopping sound per ear). The Valsalva maneuver, which always needs to be carried out gently, leads to a short-time increase in middle ear pressure that then disappears during the following equalization. Because the procedure activates – secondarily – also the natural pressure equalization in the inner ear, as described above, it generally has positive effects against the symptoms of Ménière's disease.

Coping

Sufferers tend to have high stress and anxiety, which may be caused directly by the disease and not merely a secondary effect.^[39] Vestibular injuries are known to increase levels of anxiety directly by affecting signal processing in the brain, and vice versa, i.e. anxiety negatively affects vestibular signal processing.^[40][41] Some patients benefit from non-specific yoga, t'ai chi,^[42] and meditation. Greenberg and Nedzelski recommend education to alleviate feelings of depression or helplessness.^[25]

Surgery

If symptoms do not improve with typical treatment, more permanent surgery is considered.^[43] However, because the inner ear deals with both balance and hearing, few surgeries guarantee no hearing loss.

Nondestructive surgeries include procedures that don't actively remove any functionality, but rather aim to improve the way the ear works.^[44] Surgery to decompress the endolymphatic sac has shown effective for relief from symptoms. Most patients see a decrease in vertigo occurrence, while their hearing may be unaffected. In a systematic review and meta-analysis from 2014 it was reported that in at least 75% of patients with Ménière's disease it was effective at controlling vertigo in the short term (>1 yearr of follow-up) and long term (>24 months).^[45]

Conversely, destructive surgeries are irreversible and involve removing entire functionality of most, if not all, of the affected ear.^[46] The inner ear itself can be surgically removed via labyrinthectomy although hearing is always completely lost in the affected ear with this operation.^[4] Alternatively, a chemical labyrinthectomy, in which a drug (such as gentamicin) that "kills" parts or most of the vestibular apparatus is injected into the middle ear, can accomplish the same results with a reasonable chance of retaining hearing, at least partly.^[47][48]

In more serious cases surgeons can cut the nerve to the balance portion of the inner ear in a vestibular neurectomy. Hearing is often mostly preserved, however the surgery involves cutting open into the lining of the brain, and a hospital stay of a few days for monitoring would be required.^[49] Vertigo (and the associated nausea and vomiting) typically accompany the recovery from destructive surgeries as the brain learns to compensate.^[49]

Physical exercise

Retraining of the balance system after loss of sensory input from the vestibular system in the inner ear is essential. Almost any kind of physical activity is recommended, as soon as the immediate effects of vertigo attacks have subsided.^[50]

Applications without evidence of positive effect

• Betahistine was investigated in both a systematic review and a large-scale placebo-controlled longitudinal study. Neither found evidence to justify its use in Ménière's disease.^[51][52]
• Transtympanic micropressure pulses were investigated in two systematic reviews. Neither found evidence to justify this technique.^[53][54]
• Intratympanic steroids were investigated in a systematic review of clinical trials. It was concluded that the data were insufficient to decide if this therapy has positive effects.^[55]

Prognosis

Ménière's disease usually starts confined to one ear, but it often extends to involve both ears over time. The number of patients who end up with bilaterial Ménière's was estimated on the basis of temporal bone autopsies to be ca. 30%.^[56]

The impact of vertigo on the ability to work has been described as moderate. According to a Swedish study the loss of annual working days was between none and four.^[57]

Hearing loss usually fluctuates in the beginning stages and becomes more permanent in later stages, although hearing aids and cochlear implants can help remedy damage.^[58] Tinnitus can be unpredictable, but patients usually get used to it over time.^[58]

The course of progression of Ménière's disease varies. Attacks can come more frequently and more severely, less frequently and less severely, and anywhere in between.^[59] However, Ménière's has a tendency to "burn out". Once much or all of vestibular function in an ear has disappeared, distorted sensory input to the brain is no longer generated and vertigo attacks cease.

While Ménière's disease in general has no known effects on cognitive performance, vertigo is associated with a clearly increased risk of impairment in concentration and memory.^[60]

Notable cases

• Ryan Adams, an American musician, had to take a two-year break from music due to severe symptoms of the disease and his resulting psychological distress.^[61]
• Kristin Chenoweth, Broadway, film, TV actress, and singer.^[62]
• Brent Crosswell, former Australian Rules football player.
• Mamie Eisenhower, wife of Dwight D. Eisenhower, 34th President of the United States.
• Rev. Miguel d’Escoto Brockmann, Nicaraguan diplomat, politician and Catholic priest of the Maryknoll Society of Missionaries.
• Steve Francis, American Pro Basketball Player, is known to have suffered from Ménière's disease.
• It has been suggested that Vincent van Gogh may have suffered from Ménière's,^[63] though this is now considered conjectural.^[64] See Vincent van Gogh's medical condition for a discussion of the range of possible alternative diagnoses.
• Goya the famous Spanish court painter also became deaf after a series of episodes of illness.
• Abdullah Gül, the 11th President of the Republic of Turkey.^[65]
• Henry Solon Graves, American forester. Co-founder and first director of the Yale School of Forestry in New Haven, CT. Second chief of the US Forest Service. Dean of the Yale School of Forestry and also served as provost of the University. Due to the symptoms of 'Ménière's Symbole', he resigned as Chief of the US Forest Service following his return to the US from France (during WWI, he was a Lieutenant Colonel with the Army Corps of Engineers). It appears that the onset of Ménière's occurred during this time. Henry disclosed his diagnosis of "Ménière's Symbole" in a 1919 letter to his good friend, George Dudley Seymour.^[66]
• Doc Hammer, painter and co-creator of The Venture Bros. He has stated repeatedly that he has Ménière's disease.^[67]
• Shawnae Jebbia Miss USA 1998; After experiencing a hearing impairment caused by Ménière's disease she moved out of the entertainment industry and is studying towards a Master's degree in nursing. She has acted as the spokeswoman for the Siemens Pure 700 hearing aid.
• Katie Leclerc, an American actress and star in the ABC Family television series Switched at Birth, is known to suffer from vertigo and was diagnosed as having Ménière's disease.
• Martin Luther wrote in letters about the distresses of vertigo, and suspected Satan was the cause.^[68][69]
• Paddy McAloon, English singer-songwriter and member of the band Prefab Sprout, hailed as one of the great songwriters of his era.
• Marilyn Monroe, American actress and cultural icon was known to experience the vertigo and compromised hearing associated with Ménière’s.^[70]
• Chris Packham, British wildlife photographer and television presenter.^[71] He has suffered from the condition since he was 37, but has vowed to continue with his work regardless.
• Les Paul, American musician, innovator of early electric guitar and recording technology, prolific songwriter, performer.
• Varlam Shalamov, a Russian writer, was affected.^[72]
• Alan B. Shepard, the first American astronaut and fifth man on the Moon, was diagnosed with Ménière’s disease in 1964, grounding him after only one brief spaceflight. Several years later, an endolymphatic shunt surgery (which was then at the experimental stage) was performed, allowing Shepard to fly to the Moon on Apollo 14.^[73]
• Jonathan Swift, Anglo-Irish satirist, poet, and cleric, is known to have suffered from Ménière’s disease.^[74]
• David Terrell; UFC president Dana White mentioned in an interview that Terrell underwent the same surgery as White himself.
• Dana White, president and minority owner of the Ultimate Fighting Championship (UFC). White had surgery on the condition but the procedure was a failure.^[75] After the failed surgery White had another procedure involving the use of stem cells, this time the procedure was successful.^[76]
• Su Yu, PLA General who achieved many victories for the communists during the Chinese Civil War was hospitalized in 1949. This prevented him from taking command in the Korean War, and Mao Zedong selected Peng Dehuai instead.^[77]

Notes

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2. Méniere (1861) "Sur une forme de surdité grave dépendant d’une lésion de l’oreille interne" (On a form of severe deafness dependent on a lesion of the inner ear), Bulletin de l'Académie impériale de médecine, 26 : 241.
3. Hazell, Jonathan. "Information on Ménière's Syndrome". Retrieved 27 February 2007.
4. "Meniérè's disease". Maryland Hearing and Balance Center. Retrieved 18 December 2015.
5. Haybach, pg. 55
6. "Meniere's disease symptoms". Mayo Clinic. 18 June 2008. Retrieved 17 October 2008.
7. Haybach, pg. 70
8. Lempert, T.; Neuhauser, H. (November 2008). "Epidemiology of vertigo, migraine and vestibular migraine". Journal of Neurology 256 (3): 333–338. doi:10.1007/s00415-009-0149-2. PMID 19225823.
9. Haybach, p. 72
10. Haybach, p. 71
11. Haybach, pg. 79
12. Haybach, pg. 46
13. Ruckenstein, MJ; Shea, JJ Jr (1999). Harris, JP, ed. Meniere's Disease. Kugler Publications. p. 266. ISBN 978-90-6299-162-4.
14. Hayback, PJ. "Mèniére's Disease". vestibular.org. Vestibular Disorders Association. Retrieved 22 September 2015.
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16. Haybach, pg. 8
17. Menieres Causes at the American Hearing Research Foundation Chicago, Illinois 2008.
18. Thomas, Alexander; Jeffrey Harris (October 2010). "Current Epidemiology of Menière's Syndrome". Otolaryngologic Clinics of North America 43 (5): 965–970. doi:10.1016/j.otc.2010.05.001. PMID 20713236.
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21. Beasley, Jones, p.1111, para.elsei 3
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29. WHO-Guideline: Potassium Intake for Adults and Children, 2012.
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51. A. L. James, M. J. Burton: Betahistine for Menière's disease or syndrome. In: The Cochrane database of systematic reviews. Nr 1, 2001, ISSN 1469-493X, pp. CD001873, doi:10.1002/14651858.CD001873, PMID 11279734 (Review).
52. C. Adrion, C. S. Fischer, J. Wagner, R. Gürkov, U. Mansmann, M. Strupp: Efficacy and safety of betahistine treatment in patients with Meniere's disease: primary results of a long term, multicentre, double blind, randomised, placebo controlled, dose defining trial (BEMED trial). In: BMJ (Clinical research ed.). Vol 352, 2016, pp. h6816, PMID 26797774, PMC 4721211.
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61. Drew, Ian (21 October 2011). "Ryan Adams Opens Up About Tragic Ear Disease". US Weekly. Retrieved 25 May 2012. Having your first album of new material immediately break into the Billboard Top 200 albums chart on release week is usually cause for celebration for most rock stars. But Ryan Adams isn't smiling just because his brilliantly subdued new disc, Ashes & Fire, slid right in at #7 this week. That's because the alternative singer, 36, is still grappling with Ménière's disease, a debilitating and incurable inner ear condition that forced him to take a break from music for over two years.
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