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Ménière's disease //, also called endolymphatic hydrops, is a disorder of the inner ear that can affect hearing and balance. It is characterized by episodes of vertigo, tinnitus, and hearing loss. The hearing loss comes and goes for some time, alternating between ears, then becomes permanent.
The condition is named after the French physician Prosper Ménière, who, in an 1861 article, first reported that vertigo was caused by inner ear disorders. The condition affects people differently; it can range in intensity from being a mild annoyance to a lifelong condition.
Signs and symptoms
Ménière's often begins with one symptom, and gradually progresses. However, not all symptoms must be present to confirm the diagnosis although several symptoms at once is more conclusive than different symptoms at separate times. Other conditions can present themselves with Ménière's-like symptoms, such as syphilis, Cogan's syndrome, autoimmune inner ear disease, dysautonomia, perilymph fistula, multiple sclerosis, acoustic neuroma, and both hypo- and hyperthyroidism.
Ménière's symptoms vary. Not all sufferers experience the same symptoms. However, so-called "classic" Ménière's has the following four symptoms:
- Attacks of rotational vertigo that can be severe, incapacitating, unpredictable, and last anywhere from minutes to hours, but generally no longer than 24 hours. For some, prolonged attacks can occur, lasting from several days to several weeks, often severely incapacitating the sufferer. 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, but are symptoms of vertigo, and not of Ménière's.
- Fluctuating, progressive, unilateral (in one ear) or bilateral (in both ears) hearing loss, usually in lower frequencies. For some, sounds can appear tinny or distorted, and patients can experience unusual sensitivity to noises.
- Unilateral or bilateral tinnitus.
- A sensation of fullness or pressure in one or both ears.
Some patients may have parasympathetic symptoms, which aren't necessarily symptoms of Ménière's, but rather side effects of other symptoms. These 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 non-visual balance in coordinating eye movements.
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. 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). The affected person is able to get up again immediately afterwards.
There is an increased prevalence of migraine in patients with Ménière’s disease. Some clinical samples show about one third of patients experiencing migraines. An association with familial history of vestibular migraine has also been demonstrated. It is likely that a pathophysiologic continuum (spectrum) exists between Ménière’s disease and vestibular migraine.
Ménière's disease is linked to endolymphatic hydrops, an excess of fluid in the inner ear. 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 is called "hydrops." The membranes can become dilated like a balloon when pressure increases and drainage is blocked. 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.
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. 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 cigarettes, or drinking alcohol. They may be further exacerbated by excessive consumption of salt in some patients. It has also been proposed that in some patients Ménière's disease could be caused by the harmful effects of a herpes virus.[medical citation needed]
Ménière's disease affects about 190 people per 100,000. Recent gender predominance studies show that Ménière's tends to affect women more often than men. Age of onset typically occurs in adult years, with prevalence increasing with age.
Recent research has found that Ménière's disease may potentially be influenced and worsened by obstructive sleep apnea, and that risk factors for reduced vascular function in the brain such as smoking, migraines, and atherosclerosis may play an important role in triggering attacks.
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 an 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.
Ménière's disease had been recognized as early as the 1860s, 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) set criteria for diagnosing Ménière's, as well as defining two sub categories of Ménière's: cochlear (without vertigo) and vestibular (without deafness).
In 1972, the academy defined criteria for diagnosing Ménière's disease as:
- 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. Finally in 1995, the list was again altered to allow for degrees of the disease:
- Certain - Definite disease with 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
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. Thus, a low sodium diet is often prescribed, with sodium intake reduced to one to two grams of sodium per day (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, and most people are recommended to consume less than 1.5 grams, but on average people in the United States consume 3.4 grams per day.
Diuretics have traditionally been prescribed to increase sodium excretion through the urine and thus (it is thought) enhance the effect of sodium restriction, although there is no definite supportive evidence. Some sources recommend taking two grams of potassium or more daily for similar reasons.
Additionally, patients may be advised to avoid alcohol, caffeine, and tobacco, all of which can aggravate Ménière's symptoms. Many patients have allergy testing to see if they are candidates for allergy desensitization, as allergies have been shown to aggravate Ménière's symptoms.
Prescription and over-the-counter medicine can reduce nausea and vomiting during an episode. Possibly effective medicines include antihistamines such as meclozine or dimenhydrinate, trimethobenzamide and other antiemetics, betahistine, diazepam, and ginger root. Betahistine, specifically, is of note because it is the only drug listed that has been proposed to prevent symptoms due to its vasodilation effect on the inner ear.
Another consideration is that different strains of a herpes virus can have different characteristics that produce differences in the precise effects of the virus. Further confirmation that acyclovir can have a positive effect on Ménière's symptoms has been reported.
Studies done over the use of transtympanic micropressure pulses have indicated promise with patients who had not been previously treated by gentamicin or surgery. Other studies suggest less clear results and propose that micropressure devices are simply placebos.
Sufferers tend to have high stress and anxiety, which may be caused directly by the disease and not merely a secondary effect. 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. Some patients benefit from non-specific yoga, t'ai chi, and meditation. Greenberg and Nedzelski recommend education to alleviate feelings of depression or helplessness.
If symptoms do not improve with typical treatment, more permanent surgery is considered. Unfortunately, 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. Intratympanic steroid treatments involve injecting steroids (commonly dexamethasone) into the middle ear to reduce inflammation and alter inner ear circulation. Surgery to decompress the endolymphatic sac has shown effective for temporary relief from symptoms. Most patients see a decrease in vertigo occurrence, while their hearing may be unaffected. This treatment, however, does not address the long-term course of vertigo in Ménière's disease and may require repeated surgery. Danish studies even link this surgery to a very strong placebo effect, and that very little difference occurred in a 9-year followup, but could not deny the efficacy of the treatment.
Conversely, destructive surgeries are irreversible and involve removing entire functionality of most, if not all, of the affected ear. The inner ear itself can be surgically removed via labyrinthectomy although hearing is always completely lost in the affected ear with this operation. Alternatively, a chemical labyrinthectomy, in which a drug (such as gentamicin) that "kills" the vestibular apparatus is injected into the middle ear can accomplish the same results while retaining hearing. 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. Vertigo (and the associated nausea and vomiting) typically accompany the recovery from destructive surgeries as the brain learns to compensate.
Intratympanic Gentamycin Treatment Protocol
A more recent development in the treatment of Meniere's disease is the intra-tympanic (or transtympanic) Gentamicin treatment, commonly referred to as ITG or TTG. Gentamicin, a broad spectrum antibiotic of the aminoglycoside group, is a known vestibulotoxic drug. This means that, although useful in treating many types of infections, it has the side-effect of affecting the vestibular system of the inner ear, and thus affecting balance, and, to a lesser extent, hearing. However, this particular adverse action is made use of in a Meniere's patient whose primary symptom of recurrent attacks of severe vertigo have not been controlled by conservative methods. This is a minimally invasive treatment protocol where, the drug is used as an in situ poison for destroying the vestibular balancing function of the affected ear. The treatment involves injecting a low dose (normally 40 mg/ml buffered) of the drug into the middle ear, through the ear drum, 4 to 6 serial titration injections, each generally a week apart, while monitoring the progress of the patient. The drug gradually destroys the Type I hair cells inside the ear and thus the balancing function of the vestibule, thereby minimising or eliminating the effects of endolymphatic hydrops. The healthy ear, then, takes over the balancing function and the patient may also develop physical traits such as walking with wider steps to keep balance. The treatment is low cost and about 90 percent success rate has been reported in studies. The significant advantage of this treatment is that it helps to control the handicapping vertigo attacks, without unduly worsening the hearing. The option to this method would be ear surgery to destroy the vestibule or its nerve connections. Most studies over the last decade have shown similar results, with the obvious lack of morbidity with the Gentamicin injection. The patient may develop signs of vertigo after a year which may require another series of injections.
Physiotherapists also have a role in the management of Ménière's disease. In vestibular rehabilitation, physiotherapists use interventions aimed at stabilizing gait, reducing dizziness and increasing postural balance within the context of activities of daily living. After a vestibular assessment is conducted, the physiotherapist tailors the treatment plan to the needs of that specific patient.
The central nervous system (CNS) can be re-trained because of its plasticity, or alterability, as well as its repetitious pathways. During vestibular rehabilitation, physiotherapists take advantage of this characteristic of the CNS by provoking symptoms of dizziness or unsteadiness with head movements while allowing the visual, somatosensory and vestibular systems to interpret the information. This leads to a continuous decrease in symptoms.
Although a significant amount of research has been done regarding vestibular rehabilitation in other disorders, substantially less has been done specifically on Ménière's disease. However, vestibular physiotherapy is currently accepted as part of best practices in the management of this condition.
The Merck Manual has added head trauma as a risk factor due to the research on 300 Ménière's patients over the past fourteen years. Michael Burcon, BPh, DC has established a link between whiplash as a result of vehicular accidents or falling on one's head and Ménière's disease. It takes an average of fifteen years after the trauma before the onset of symptoms. Case history, thermography, MRI, CScan[clarification needed], and/or cervical x-ray and modified Prill relative leg length tests are used for diagnosis and upper cervical specific adjustments are performed for treatment to reduce or eliminate vertigo.
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 is debated, with ranges spanning from 17% to 75%.
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. Tinnitus can be unpredictable, but patients usually get used to it over time.
Ménière's disease, being unpredictable, has a variable prognosis. Attacks could come more frequently and more severely, less frequently and less severely, and anywhere in between. However, Ménière's is known to "burn out" when vestibular function has been destroyed to a stage where vertigo attacks cease.
Studies done on both right and left ear sufferers show that patients with their right ear affected tend to do significantly worse in cognitive performance. 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.
- Ryan Adams, an American musician, had to take a two-year break from music because the disease became so degenerative to him, and needed to undergo therapy to get back on stage to overcome the anxiety the disease caused him.
- 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.
- Kristin Chenoweth, Broadway, film, TV actress, and singer.
- Brent Crosswell, former Australian Rules football player.
- Charles Darwin may have suffered from Ménière’s disease. This idea is based on a common list of symptoms 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 health.
- 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, though this is now considered conjectural. See Vincent van Gogh's medical condition for a discussion of the range of possible alternative diagnoses.
- Abdullah Gül, the 11th President of the Republic of Turkey.
- 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.
- Doc Hammer, painter and co-creator of The Venture Bros. He has stated repeatedly that he has Ménière's disease.
- 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 spokesperson 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.
- 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.
- Chris Packham, British wildlife photographer and television presenter. Has suffered with 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.
- 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.
- Jonathan Swift, Anglo-Irish satirist, poet, and cleric, is known to have suffered from Ménière’s disease.
- 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. After the failed surgery White had another procedure involving the use of stem cells, this time the procedure was successful.
- Su Yu, PLA General who achieved many victories for the communists during the Chinese Civil War was hospitalized in 1949 and that prevented him from taking command in the Korean War, and Mao Zedong selected Peng Dehuai instead.
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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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Meanwhile, Shalamov's health kept deteriorating—the Kolyma twig would have but a short time before wilting in Moscow. The body of a Kolyma survivor bore witness of its own: blindness, deafness, frostbitten skin, Ménière’s disease, chronic congestion, and apparently also minor strokes, angina pectoris, Parkinson's disease, and incipient dementia. Iulii Shreider found a woman to cook and clean for him, yet eventually Shalamov broke with her too. Unable to take care of himself, in 1979 he was placed in a nursing home (see Isaev 1996).
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