Ménière's disease
55994
224763284
2008-07-10T09:06:03Z
84.177.88.4
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{{Refimprove|date=December 2007}}
{{Infobox_Disease |
Name = Ménière's disease |
Image = |
Caption = |
DiseasesDB = 8003 |
ICD10 = {{ICD10|H|81|0|h|80}} |
ICD9 = {{ICD9|386.0}} |
ICDO = |
OMIM = 156000 |
MedlinePlus = 000702 |
eMedicineSubj = emerg |
eMedicineTopic = 308 |
MeshID = D008575 |
}}
'''Ménière's disease''' is a disorder of the [[inner ear]] that can affect [[Hearing (sense)|hearing]] and [[balance]]. It is characterized by episodes of [[dizziness]] and [[tinnitus]] and progressive hearing loss, usually in one ear. It is caused by an increase in volume and pressure of the [[endolymph]] of the inner ear. It is named after the French physician [[Prosper Ménière]], who first reported that [[Vertigo (medical)|vertigo]] was caused by inner ear disorders in an article published in [[1861]].<ref>{{WhoNamedIt|synd|2073|Ménière's disease}}</ref>
==Symptoms==
The symptoms of Ménière's are variable; not all sufferers experience the same symptoms. However, so-called "classic Ménière's" is considered to comprise the following four symptoms:
* Periodic episodes of rotary [[Vertigo (medical)|vertigo]] (the abnormal sensation of movement) or dizziness.
* Fluctuating, progressive, unilateral (in one [[ear]]) or bilateral (in both ears) [[hearing loss]], often initially in the lower frequency ranges.
* Unilateral or bilateral [[tinnitus]] (the perception of noises, often ringing, roaring, or whooshing), sometimes variable.
* A sensation of fullness or pressure in one or both ears.
Ménière's often begins with one symptom, and gradually progresses. However, not all symptoms must be present for a doctor to make a diagnosis of the disease.<ref>{{cite web | url=http://www.tinnitus.org/home/frame/meniere.htm | title = Information on Ménière's Syndrome" | first=Jonathan | last=Hazell | accessdate=2007-02-27}}</ref> Several symptoms at once is more conclusive than different symptoms at separate times.<ref name="Maryland">{{cite web |publisher=Maryland Hearing and Balance Center |title=Meniérè's disease |url=http://www.umm.edu/otolaryngology/menieres_disease.html |accessdate=2008-03-03}}</ref>
Attacks of vertigo can be severe, incapacitating, and unpredictable. Some patients experience vertigo for hours or days, and this combines with an increase in volume of tinnitus and temporary, albeit significant, hearing loss. Hearing may improve after an attack, but often becomes progressively worse. [[Nausea]], [[vomiting]], and [[sweating]] sometimes accompany vertigo.
Some sufferers experience what are informally known as "[[drop attack]]s" — a sudden, severe attack of dizziness or vertigo that causes the sufferer, if not seated, to ''fall''. Patients may also experience the feeling of being pushed or pulled (Pulsion).
Some patients may find it impossible to get up for some time, until the attack passes or medication takes effect.
In addition to hearing loss, sounds can seem tinny or distorted, and patients can experience unusual sensitivity to noises (hyperacusis). Some sufferers also experience [[nystagmus]], or uncontrollable rhythmical and jerky eye movements, usually in the horizontal plane, reflecting the essential role of non-visual balance in coordinating eye movements.
Studies done on both right and left ear sufferers show that patients with their right ear tend to do significantly worse in cognitive performance.<ref>Theilgaard, Laursen, Kjaerby, ''et al''. p. 103</ref> General intelligence was not hindered, and it was concluded that declining performance was related to how long the patient had been suffering from the disease.<ref>Theilgaard, Laursen, Kjaerby, ''et al''. p. 104</ref>
==Cause==
The exact cause of Ménière's disease is not known, but it is believed to be related to ''endolymphatic hydrops'' or excess fluid in the inner ear. It is thought that endolymphatic fluid bursts from its normal channels in the ear and flows into other areas causing damage. 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. The symptoms may occur in the presence of a [[middle ear]] [[infection]], [[head trauma]] or an [[upper respiratory tract infection]], or by using [[aspirin]], smoking [[cigarette]]s or drinking alcohol. They may be further exacerbated by excessive consumption of [[caffeine]] and [[salt]] in some patients.
It has also been proposed that Ménière's symptoms in some patients may be caused by the deleterious effects of a herpes virus.<ref name=" Shichinohe ">{{cite journal | last = Shichinohe | first = Mitsuo | title = Effectiveness of Acyclovir on Meniere's Syndrome III Observation of Clinical Symptoms in 301 cases | journal = Sapporo Medical Journal | volume = 68 | issue = 4/6 | pages = 71-77 | date = December 1999 | url = | doi = | id = | accessdate = 2008-06-26}}</ref><ref name="pmid11464320">{{cite journal |author=Gacek RR, Gacek MR |title=Menière's disease as a manifestation of vestibular ganglionitis |journal=Am J Otolaryngol |volume=22 |issue=4 |pages=241–50 |year=2001 |pmid=11464320 |doi=10.1053/ajot.2001.24822 |url=}}</ref> [[Herpesviridae]] are present in a majority of the population in a dormant state. It is suggested that the virus is reactivated when the immune system is depressed due to a stressor such as trauma, infection or surgery (under general anesthesia). Symptoms then develop as the virus degrades the structure of the inner ear.
==Diagnosis==
Many disorders have symptoms similar to Ménière's. Doctors establish it with complaints and [[medical history]]. However, a detailed [[Otolaryngology|otolaryngological]] examination, [[audiometry]] and head [[magnetic resonance imaging]] (MRI) scan should be performed to exclude a [[tumour]] of the [[cranial nerve VIII|eighth cranial nerve]] (vestibulocochlear nerve) or [[superior canal dehiscence]] which would cause similar symptoms. Because there is no definitive test for Ménière's, it is only diagnosed when all other causes have been ruled out.
Ménière's typically begins between the ages of 30 and 60 and affects men slightly more than women.<ref>[[#Margolis2004|''Johns Hopkins Complete Home Guide to Symptoms & Remedies'']], p.550</ref><ref>[http://www.doctoronline.nhs.uk/masterwebsite1Asp/targetpages/specialts/ent/menieres.asp U.K. National Health Service]</ref>
===History===
Ménière's disease had been recognized prior to 1972, but it 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) made set criteria for diagnosing Ménière's, as well as defining two sub categories of Ménière's: cochlear (without vertigo) and vestibular (without deafness).<ref>Beasley, Jones, p.1111, para. 3</ref>
In 1972, the academy defined criteria for diagnosing Ménière's disease as:<ref>Beasley, Jones, p.1111, para. 2/table I</ref>
#Fluctuating, progressive, sensorineural deafness.
#Episodic, characteristic definitive spells of vertigo lasting 20 minutes to 24 hours with no unconsciousness, vestibular [[nystagmus]] always present.
#Usually tinnitus.
#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.<ref>Beasley, Jones, p.1111, para. 4/table II</ref> Finally in 1995, the list was again altered to allow for degrees of the disease:<ref>Beasley, Jones, p.1112, para. 2/table III</ref>
# Certain - Definite disease with [[Histopathology|histopathological]] confirmation
# Definite - Requires two or more definitive episodes of vertigo with hearing loss plus tinnitus and/or aural fullness
# Probable - Only one definitive episode of vertigo and the other symptoms and signs
# Possible - Definitive vertigo with no associated hearing loss
==Treatment==
Initial treatment is aimed at both dealing with immediate symptoms and preventing recurrence of symptoms, and so will vary from patient to patient. Doctors may recommend vestibular training, methods for dealing with tinnitus, stress reduction, hearing aids to deal with hearing loss, and medication to alleviate nausea and symptoms of vertigo.
Several environmental and dietary changes are thought to reduce the frequency or severity of symptom outbreaks. Most patients are advised to adopt a low-sodium diet, typically one to two grams (1000-2000mg)<ref name="Maryland" /> at first, but diets as low as 400mg are not uncommon.{{fact|date=June 2008}} Patients are advised to avoid caffeine, alcohol and tobacco, all of which can aggravate symptoms of Ménière's. Some recommend avoiding [[Aspartame]]. Patients are often prescribed a mild diuretic (sometimes vitamin B6). Many patients will have allergy testing done to see if they are candidate for allergy desensitization as allergies have been shown to aggravate Ménière's symptoms.<ref name="pmid10652386">{{cite journal |author=Derebery MJ |title=Allergic management of Meniere's disease: an outcome study |journal=Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery |volume=122 |issue=2 |pages=174–82 |year=2000 |pmid=10652386 |doi=}}</ref>
Women may experience increased symptoms during [[pregnancy]] or shortly before [[menstruation]], probably due to increased fluid retention.
Some doctors recommend [[Lipoflavonoid]]s.<ref>{{cite journal |author=Williams HL, Maher FT, Corbin KB, Brown JR, Brown HA, Hedgecock LD |title=Eriodictyol glycoside in the treatment of Meniere’s disease |journal=Ann. Otol. Rhinol. Laryngol. |volume=72 |issue= |pages=1082–101 |year=1963 |month=December |pmid=14088725 |doi= |url=}}</ref>
Many patients consider fluorescent lighting to be a trigger for symptoms.{{Fact|date=March 2008}} The plausibility of this can be explained by how important a part vision plays in the overall mechanism of human balance.
Treatments aimed at lowering the pressure within the inner ear include [[antihistamine]]s, [[anticholinergic]]s, [[steroid]]s, and [[diuretic]]s.<ref name="Maryland" /> Devices that provide transtympanic micropressure pulses (such as the [http://www.meniett.com/ Meniett]) are now showing some promise and is becoming more widely used as a treatment for Ménière's.<ref name="pmid15949105">{{cite journal |author=Rajan GP, Din S, Atlas MD |title=Long-term effects of the Meniett device in Ménière's disease: the Western Australian experience |journal=The Journal of laryngology and otology |volume=119 |issue=5 |pages=391–5 |year=2005 |pmid=15949105 |doi=10.1258/0022215053945868}}</ref> The Meniett, specifically, is proven to be a safe method for reducing vertigo frequency for a majority of users.<ref>{{cite journal |author=Gates GA, Verrall A, Green JD, Tucci DL, Telian SA |title=Meniett clinical trial: long-term follow-up |journal=Arch. Otolaryngol. Head Neck Surg. |volume=132 |issue=12 |pages=1311–6 |year=2006 |month=December |pmid=17178941 |doi=10.1001/archotol.132.12.1311 |url=}}</ref>
[[Surgery]] may be recommended if medical management does not control vertigo. Injection of steroid medication behind the eardrum, or surgery to decompress the endolymphatic sac may relieve symptoms. Permanent surgical destruction of the balance part of the affected ear can be performed for severe cases if only one ear is affected. This can be achieved through [[chemical labyrinthectomy]], in which a drug (such as [[gentamicin]]) that "kills" the vestibular apparatus is injected into the middle ear. Alternatively, surgeons can cut the nerve to the balance portion of the inner ear in a [[Vestibular nerve|vestibular]] [[neurectomy]], or the inner ear itself can be surgically removed (labyrinthectomy). These treatments eliminate vertigo, but because they are destructive, they are a last resort. Typically balance returns to normal after these procedures, but hearing loss may continue to progress.<ref name="Maryland" />
The anti-herpes virus drug [[Aciclovir]] has also been used with some success to treat Ménière's Disease.<ref name=" Shichinohe "/> The likelihood of the effectiveness of the treatment was found to decrease with increasing duration of the disease probably because viral suppression does not reverse damage. Morphological changes to the inner ear of Ménière's sufferers have also been found which it was considered likely to have resulted from attack by a [[herpes simplex virus]].<ref name = "pmid11464320" /> It was considered possible that long term treatment with acyclovir (greater than six months) would be required to produce an appreciable effect on symptoms. Herpes viruses have the ability to remain dormant in nerve cells by a process known as [[HHV Latency Associated Transcript]]. Continued administration of the drug should prevent reactivation of the virus and allow for the possibility of an improvement in symptoms. Another consideration is that different strains of a herpes virus can have different characteristics which may result in differences in the precise effects of the virus. Further confirmation that acyclovir can have a positive effect on Ménière's symptoms has been reported.<ref name="pmid18235200">{{cite journal |author= Gacek RR |title= Evidence for a viral neuropathy in recurrent vertigo |journal=ORL J Otorhinolaryngol Relat Spec. |volume=70 |issue=1 |pages=6–14 |year=2008 |pmid=18235200 }}</ref>
==Progression==
Progression of Ménière's is unpredictable: symptoms may worsen, disappear altogether, or remain the same.
Sufferers whose Ménière's began with one or two of the classic symptoms may develop others with time. Attacks of vertigo can become worse and more frequent over time, resulting in loss of employment, loss of the ability to drive, and inability to travel. Some patients become largely housebound. Hearing loss can become more profound and may become permanent. Some patients become deaf in the affected ear. Tinnitus can also worsen over time. Some patients with unilateral symptoms, as many as fifty percent by some estimates, will develop symptoms in both ears. Some of these will become totally deaf.
Yet the disease may end spontaneously and never repeat again. Some sufferers find that after eight to ten years their vertigo attacks gradually become less frequent and less severe; in some patients they disappear completely. In some patients, symptoms of tinnitus will also disappear, and hearing will stabilize (though usually with some permanent loss).
==Famous sufferers==
*[[Alan Shepard|Alan B. Shepard]], the first [[United States|American]] [[astronaut]], was diagnosed with Ménière’s disease in 1964, grounding him after only one brief spaceflight. Several years later, surgery (which was then at the experimental stage) was performed, allowing Shepard to fly to the [[Moon]] on [[Apollo 14]].<ref>{{cite web | last = Gray
| first = Tara | title = Alan B. Shepard, Jr. | work = 40th Anniversary of Mercury 7 | publisher = NASA | url = http://history.nasa.gov/40thmerc7/shepard.htm | accessdate = 2008-03-03}}</ref>
*The Ménière's Disease Information Center lists poet [[Emily Dickinson]], author [[Jonathan Swift]], [[NBA]] player [[Steve Francis]], and many others as Ménière's disease sufferers.<ref>{{cite web | url=http://www.menieresinfo.com/patients.html | title=Meniere's Disease Information Center -- Famous Patients}}</ref>
*According to his blog, Author and entrepreneur [[Guy Kawasaki]] has the illness.<ref>{{cite web | url=http://blog.guykawasaki.com/2005/12/the_102030_rule.html | title=How to Change the World: The 10/20/30 Rule of PowerPoint}}</ref>
*[[J-pop]] queen [[Ayumi Hamasaki]] recently admitted on her blog the condition affecting her left ear. However, she stated that she will continue to sing.
*Contemporary artist and [[graphic designer]] [[Doc Hammer]], of ''[[The Venture Bros.]]'' fame, has Ménière's syndrome according to his May 16th, 2005 journal entry.<ref>{{cite web | url=http://doc-hammer.deviantart.com/journal/ | title=50 Questions (from MySpace)| date=2005-05-16}}</ref>
*[[Paddy McAloon]], the singer and songwriter for the British pop group [[Prefab Sprout]], was diagnosed with Ménière's in 2004.<ref>{{cite web | url=http://www.prefabsprout.net/about.html | title=sproutnet :: about the band}}</ref>
*A paper by Arenberg et. al, 1990, suggested that [[Vincent Van Gogh]], the Dutch Post-Impressionist, may have suffered from Ménière's,<ref name="pmid2094236">{{cite journal |author=Arenberg IK, Countryman LF, Bernstein LH, Shambaugh GE |title=Van Gogh had Menière's disease and not epilepsy |journal=JAMA |volume=264 |issue=4 |pages=491–3 |year=1990 |pmid=2094236| doi = 10.1001/jama.264.4.491 <!--Retrieved from CrossRef by DOI bot-->}}</ref> though this is now considered conjectural.{{Fact|date=November 2007}} See [[Vincent van Gogh's medical condition]] for a discussion of the range of possible alternative diagnoses. Some believe "[[The Starry Night]]" illustrates his dizziness. It's also speculated that ear pressure and deafness could have inspired him to cut off his own ear.{{Fact|date=June 2007}}
*[[Charles Darwin's illness|Charles Darwin]] may have suffered from Ménière’s disease. This idea is based on a common list of symptoms which were present in Darwin's case, such as tinnitus, vertigo, dizziness, motion sickness, vomiting, continual malaise and tiredness. The absence of hearing loss and 'fullness' of the ear (as far as known) excludes however a diagnosis of typical Ménière’s disease. Darwin himself had the opinion that most of his health problems had an origin in his 4-year bout with sea sickness. Later, he could not stand traveling by carriage, and only horse riding would not affect his health. One of the diagnoses that he received from his physicians at the time was that of "suppressed [[gout]]". The source of Darwin's illness is not known for certain. See ''[[Charles Darwin's illness]]'' for more details.
*Fictional comic book villain [[Count Vertigo]] suffers from Ménière's disease and has the ability to inflict its symptoms on others.
*[[Martin Luther]] wrote in letters about the distresses of vertigo, and suspected Satan was the cause.<ref name="pmid2529669">{{cite journal |author=Feldmann H |title=Martin Luther's seizure disorder |language=German |journal=Sudhoffs Archiv |volume=73 |issue=1 |pages=26–44 |year=1989 |pmid=2529669 |doi=}}</ref><ref>{{cite journal | last = Cawthorne
| first = T | title = Ménière's disease | journal = Annals of Otology | volume = 56 | pages = 18–38 | date = 1947}}</ref>
* [[Julius Caesar]] was known to have suffered from the "falling sickness" as noted in [[Plutarch]]'s [[Parallel Lives]] and has been cited by [[Shakespeare]], noting that Caesar was unable to hear fully in his left ear.<ref>{{cite journal | last = Cawthorne
| first = T | title = Julius Caesar and the falling sickness | journal = Proceedings of the Royal Society of Medicine | volume = 51 | pages = 27–30 | date = 1958}}</ref>
* [[Jonathan Swift]], Anglo-Irish [[satire|satirist]], [[poetry|poet]], and [[cleric]], is known to have suffered from Ménière’s disease.
* [[Marilyn Monroe]], American actress and cultural icon was known to experience the vertigo and compromised hearing associated with Ménière’s.<ref>Brown, Peter and Barham, Patte ''Marilyn: The Last Take''. New York: Dutton, 1992, p. 221 ISBN-10: 0-525-93485-5</ref>
==See also==
* [[Balance disorder]]
* [[Neurectomy]]
* [[Superior canal dehiscence syndrome]]
== References ==
*{{cite journal |author=Theilgaard A, Laursen P, Kjaerby O, ''et al'' |title=Menière's disease. II. A neuropsychological study |journal=ORL J. Otorhinolaryngol. Relat. Spec. |volume=40 |issue=3 |pages=139–46 |year=1978 |pmid=570693 |doi= |url=}}
*{{cite journal |author=Beasley NJ, Jones NS |title=Menière's disease: evolution of a definition |journal=J Laryngol Otol |volume=110 |issue=12 |pages=1107–13 |year=1996 |month=December |pmid=9015421 |doi= |url=}}
* {{cite book |first=Simeon|last=Margolis|title=The Johns Hopkins Complete Home Guide to Symptoms & Remedies|year=2004|publisher=Black Dog & Leventhal Publishers|location=|isbn=978-1579124021 |ref=Margolis2004}}
== Footnotes==
{{reflist|2}}
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{{dmoz|Health/Conditions_and_Diseases/Ear,_Nose_and_Throat/Ear/Meniere's_Disease|Meniere's Disease}}
{{Diseases of the ear and mastoid process}}
{{DEFAULTSORT:Meniere's disease}}
[[Category:Otology]]
[[Category:Neurology]]
[[ca:Vertigen de Ménière]]
[[cs:Ménierova nemoc]]
[[de:Morbus Menière]]
[[et:Ménière'i tõbi]]
[[es:Enfermedad de Ménière]]
[[fr:Maladie de Menière]]
[[hr:Ménièreova bolest]]
[[it:Sindrome di Ménière]]
[[he:מחלת מנייר]]
[[nl:Ziekte van Ménière]]
[[ja:メニエール病]]
[[no:Ménières sykdom]]
[[pl:Choroba Méniére'a]]
[[pt:Síndrome de Ménière]]
[[fi:Ménièren tauti]]
[[sv:Ménières sjukdom]]
[[tr:Ménière hastalığı]]
[[zh:美尼尔氏综合症]]