Ménière’s disease

Synonyms

1

Overview

Ménière's disease , 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.

Symptoms

Ménière's often begins with one symptom, and gradually progresses. However, not all symptoms must be present to confirm the diagnosis[4] 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, severe attacks of dizziness or vertigo—called Tumarkin's otolithic crises or, informally, as "drop attacks"—can make someone who is standing suddenly fall. Drop attacks are likely to occur later in the disease, but can occur at any time.

Migraine

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.

Causes

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.

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.

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 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.

History

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:

  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. Usually tinnitus.
  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. Finally in 1995, the list was again altered to allow for degrees of the disease:

  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

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 is debated, with ranges spanning from 17% to 75%.

Some Ménière's disease sufferers, in severe cases, find themselves unable to work. However, a majority (60-80%) of sufferers recover with or without medical help.

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

Treatment

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.

Coping

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.

Surgery

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.

Physiotherapy

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.