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Meniere’s disease: symptoms, therapy, causes

by Josephine Andrews
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Meniere’s disease (Ménière’s disease) is a disease of the inner ear, thought to be caused by excess pressure in the inner ear. The three most important symptoms are unpredictable attacks of sudden vertigo, ringing in the ears (tinnitus) and a reduction in hearing. In principle, Meniere’s disease cannot be cured, but the severity and frequency of the attacks can be reduced with medication. Find out more about the symptoms and treatment options for Meniere’s disease here.

ICD codes for this disease:

ICD codes are internationally valid codes for medical diagnoses. They can be found, for example, in doctor’s letters or on certificates of incapacity for work.

H81

quick overview

  • Symptoms: Combination of suddenly occurring vertigo with ringing in the ears and one-sided hearing loss, the attacks last from minutes to hours
  • Therapy: In acute cases: medication for dizziness; other medications serve to prevent seizures. In very severe cases, the vestibular system is switched off by medication or an operation.
  • Causes: Imbalance in the fluid balance of the inner ear of unknown cause
  • Diagnosis: Examination of the eardrum and auditory nerve, various hearing tests, imaging procedures to rule out other diseases with similar symptoms
  • Course of the disease: Usually only one ear is affected at first, later often both. Symptoms sometimes resolve completely after an attack, but permanent damage to hearing and balance is also possible.
  • Prevention: The disease cannot be prevented, but if those affected follow medical prophylaxis recommendations, the attacks usually occur less frequently.

What is Meniere’s disease?

Meniere’s disease (Meniere’s disease) is a disease of the inner ear. It is named after the French doctor Prosper Menière, who described it in 1861.

The inner ear contains the cochlea and the organ of equilibrium as well as two different fluids – including the so-called endolymph. According to the current state of knowledge, the reabsorption of this fluid in the inner ear is disturbed in Meniere’s disease. As a result, too much endolymph accumulates.

Because the inner ear is lined by a membranous membrane, fluid has limited room to expand. The result is an increase in pressure in the inner ear, which damages the cochlea (see causes and risk factors).

Meniere’s disease often occurs between the ages of 40 and 60. But people in young adulthood also become ill. Women are affected more often than men. In Europe, around 13 out of 100,000 people develop Meniere’s disease every year.

Popularly Meniere’s disease is sometimes known under the name Meniere’s syndrome or Menièr’s disease.

What are the symptoms of Meniere’s disease?

Typical of Meniere’s disease is the vertigo that occurs during attacks in combination with ringing in the ears (tinnitus) and one-sided hearing loss. Spinning vertigo causes those affected to have the feeling that the environment is spinning around them very quickly (similar to a ride on a carousel).

The dizziness is sometimes so severe that those affected have to lie down. Falls caused by dizziness and nausea with vomiting also occur. Those affected describe rotary vertigo as the most distressing of the Meniere’s disease symptoms, as it occurs without warning and can last for hours or days.

In addition, there is tinnitus and hearing loss, which mainly affects low tones. Those affected often feel pressure on the ear during an attack of Meniere’s disease. While at the beginning of the disease usually only one ear is affected, Meniere’s  disease may spread to the second ear as it progresses.

In addition to these main characteristics of Meniere’s disease, sufferers often turn pale and sweat. Some people’s eyes tremble (nystagmus) during an attack.

The attacks of Meniere’s disease come on suddenly and unexpectedly. They usually last between ten and twenty minutes, but sometimes last for hours. Then the attacks usually stop by themselves.

Since Meniere’s disease attacks are extremely stressful due to the rotary vertigo and occur completely unpredictably, some of those affected develop psychological problems such as anxiety disorders and depression. Under certain circumstances, this creates a vicious circle between the seizures and the psychological situation of the person concerned, in which the problems reinforce each other.

treatment

Meniere’s disease cannot be cured, but the symptoms can be curbed with various therapeutic measures. Meniere’s disease therapy has two main goals: On the one hand, this includes reducing the severity of the symptoms in an acute attack. On the other hand, the doctor will try to prevent further attacks (prophylaxis) with preventive measures.

acute therapy

Various medications (antivertebral drugs) are available to combat the dizziness and vomiting caused by Meniere’s disease. The standard drug is the active ingredient dimenhydrinate. It is advisable for those affected by Meniere’s disease to always carry this active ingredient with them so that they can react immediately in the event of a sudden attack. If the symptoms are severe, it is also possible to have the appropriate medication administered intravenously by the doctor.

Prevention (prophylaxis)

The most important goal, apart from the acute therapy, is to reduce the number of attacks. There are a number of therapy options. The current recommendation for seizure prophylaxis in Meniere’s disease is the administration of betahistine. It stimulates the docking points (receptors) for a specific messenger substance (histamine) and thereby improves the blood circulation in the inner ear. Experts suspect that the excess endolymph is transported away more quickly due to the higher blood flow in the inner ear.

Diuretics, on the other hand, are drugs that promote water excretion. They are also used in Meniere’s disease to reduce the accumulation of fluid in the inner ear.

The anti-inflammatory cortisone also serves to prevent Meniere’s disease attacks. The doctor injects it under local anesthetic through the eardrum into the middle ear (intratympanic corticosteroid therapy). From there, the cortisone diffuses into the inner ear, where it has an anti-inflammatory and vessel-sealing effect. This therapy has been used for several years, mainly in the therapy of sudden hearing loss.

Enlargement of the endolymphatic sac

Depending on the severity of the symptoms, the doctor recommends a saccotomy for Meniere’s disease. It is a surgical procedure in which the surgeon widens the endolymphatic sac. The endolymphatic sac is part of the balance system and is involved in regulating the amount of endolymph. The intervention enables him to expand more.

elimination of the organ of balance

If the above therapies are unsuccessful in Meniere’s disease patients, there are various methods that completely and permanently switch off the affected organ of balance: This can be done with medication or as part of an operation. In both cases, the result is hearing loss or deafness and a loss of balance.

In addition, the corresponding measures can no longer be undone. Although the healthy side is able to take over some of these functions, it often suffers as the disease progresses. Appropriate measures are therefore only used in severe cases

In order to switch off the inner ear with medication in Meniere’s disease, the doctor injects the antibiotic gentamicin into the middle ear. It damages the sensory cells and thus renders the balance organ in the inner ear inoperable.

It is possible to repeat the gentamicin administration at intervals of several weeks. The large distance is necessary to avoid as much as possible unwanted damage to the cochlea, which is also located in the inner ear. Since hearing loss is still possible, gentamicin is used primarily in those affected who already have significant hearing impairment before the therapy.

If the measure does not achieve the hoped-for success, it is possible to remove a part of the inner ear, the so-called labyrinth (labyrinthectomy), in Meniere’s disease. However, this therapy is controversial.

Psychological care

In addition to drug therapy, psychological support is very important in Meniere’s disease. The sudden and unpredictable attacks represent a significant psychological burden for some of those affected.

alternative therapies

Apart from conventional medicine, there are alternative therapeutic approaches for treating Meniere’s disease. Homeopathy can help, especially to reduce the often tormenting dizziness. It is recommended to take Cocculus D6 three times a day for several weeks. Tabacum D12 is used to interrupt seizures.

The concept of homeopathy and its specific effectiveness are controversial in science and not clearly proven by studies.

Certain diets (especially salt-free food) also relieve the symptoms in some sufferers of Meniere’s disease. Other supportive therapies are acupuncture, Feldenkrais or balance training. In any case, discuss with your doctor whether alternative medical approaches are effective in your case.

Causes and risk factors

According to current knowledge, the cause of Meniere’s disease is a disorder in the inner ear. This assumption is based on the fact that the majority of those affected have enlarged spaces in the inner ear. Nevertheless, it is still unclear whether this finding is also causally related to Meniere’s disease.

The inner ear is responsible for hearing and balance. It consists of an intricate duct system filled with two different fluids (endolymph and perilymph). These are in a sensitive balance and are indispensable for the function of the organ.

Doctors assume that Meniere’s disease is caused by excess fluid (hydrops) in the endolymph. This can be demonstrated in almost all those affected. Possible causes include the rare inflammation of the inner ear (labyrinthitis) or a concussion. In most cases, however, the cause remains unclear. The condition is genetic in up to 15 percent of those affected. Especially when the symptoms appear in childhood, there are often other cases of Meniere’s disease in the family.

The currently most common but controversial assumption is that a seizure causes a tear in the fine membranes that separate different areas of the inner ear. When these membranes rupture, endo- and perilymph mix, disrupting the delicate balance of salts (electrolytes) in these fluids. There is also a sudden change in pressure conditions. Overall, this results in a malfunction of the sensory cells, which doctors use to explain the symptoms of Meniere’s disease.

investigations and diagnosis

The first point of contact if you suspect Meniere’s disease is your general practitioner. If necessary, he will refer the person concerned to the ENT doctor or neurologist. Many clinics also have special “vertigo centers” that are the point of contact, especially in severe cases.

During the consultation with the doctor, the doctor first inquires about the symptoms and any previous illnesses. For example, to diagnose Meniere’s disease, it is important

  • exactly how the vertigo attack works
  • whether the dizziness is accompanied by tinnitus and hearing loss in that ear
  • how long the vertigo attack lasts
  • whether the vertigo attack can be provoked by a certain movement, for example by turning your neck? (This would argue against Meniere’s disease.)
  • whether the patient is taking medication

Physical examination

During the physical examination, the doctor uses an otoscope to look at the eardrum in the ear. The damage in Menière’s disease is in the inner ear and is therefore not visible from the outside. However, it is important for the doctor to rule out concomitant diseases of the eardrum and the middle ear by inspection with the otoscope.

The standard examinations in ear, nose and throat medicine also include the Weber and Rinne tuning fork test. The doctor places a vibrating tuning fork on the crown of the head or behind the ear. The affected person must indicate when they can no longer hear the sound of the tuning fork or whether they can hear it again when the tuning fork is directly in front of their ear (Rinne test). In addition, he should state whether the sound of the tuning fork placed on the crown of the head appears louder in one of the two ears (Weber test).

These tests enable the doctor to draw conclusions as to whether the symptoms are caused by damage to the inner or middle ear.

The doctor also uses various tests to check the functioning of the sense of balance. This includes, for example, the Unterberger stepping test, in which the person concerned steps on the spot 50 times with their eyes closed. If it unconsciously turns around its own axis, this may indicate damage to the vestibular system.

As part of the clarification of Meniere’s disease, the doctor may also check whether there are any involuntary eye movements (nystagmus). Twitching eye movements to one side (horizontal nystagmus) are typical of Meniere’s disease, but these usually only occur during the attack.

Further investigations

A hearing test (tone threshold audiometry) is required to assess an existing hearing loss more precisely. The ability to hear in one ear is significantly reduced in those affected by Meniere’s disease. In addition, the hearing performance for low frequencies in particular is reduced. Hearing recovers after the attack in many cases, but permanent hearing loss sometimes remains.

Under certain circumstances, the doctor analyzes the brain waves that occur after a sound signal (= acoustically evoked potentials). In this way, the connection between the auditory pathway and the brain can be checked. It is not affected by Meniere’s disease.

Symptoms similar to those seen in Meniere’s disease are seen in other diseases in a similar way. It is therefore necessary to exclude these alternative causes of Meniere’s disease symptoms in the diagnosis. For example, the doctor examines the auditory nerve to make sure it is not damaged.

Computer tomography (CT) or magnetic resonance imaging (MRI) can be used to create images of the head and inner ear. This makes it possible, for example, to rule out tumor and inflammatory processes.

Diagnosis of Meniere’s disease

The diagnosis of Meniere’s disease is confirmed when the patient meets the following criteria:

  • Two or more attacks of vertigo, each lasting at least 20 minutes
  • Deterioration of hearing confirmed by examination
  • Ringing in the ears (tinnitus) or ear pressure in the affected ear
  • exclusion of other causes

Course of the disease and prognosis

The course of Meniere’s disease varies greatly from person to person. It is possible that it will remain in a single attack. Most of the time, however, the attacks are repeated. But even after several years, it is still possible that the symptoms of Meniere’s disease will end spontaneously and never reappear. In this case, however, damage to hearing and balance that has occurred up to that point usually remains permanent.

The consequential damage of Meniere’s disease attacks ranges to complete deafness on the affected side. After five years of illness, symptoms affect both sides in 50 percent of cases. After about ten years, in the late stage of Meniere’s disease, the symptoms in many of those affected subside significantly, and therapy is then often no longer necessary.

Sometimes Meniere’s disease leads to incapacity to work because certain jobs that place high demands on the sense of balance can no longer be carried out (e.g. roofer or diver). It is possible for those affected to have Meniere’s disease recognized as a disability. However, doctors only certify a severe disability in serious cases in the case of very numerous and/or severe illness attacks.

The ability to drive a vehicle may also be impaired in Meniere’s disease. According to current legislation, driving suitability for single-track vehicles (e.g. a motorcycle) is generally not given. Whether those affected are allowed to drive a car depends on the result of a specialist medical examination.

Last but not least, the extent of the influence of the symptoms on the psyche of an affected person is decisive for the prognosis of Meniere’s disease. The fear of new attacks is very stressful and in turn encourages new attacks. Breaking this vicious circle, if necessary with therapeutic support, is an important therapeutic goal in Meniere’s disease.

Prevent

It is not possible to prevent Meniere’s disease in general because the causes are not known. However, it may be possible for those affected to prevent the seizures with various measures. In addition to the medicinal prophylaxis prescribed by the doctor, this includes a low-salt diet and abstaining from coffee, alcohol and nicotine. However, their effectiveness has not been scientifically proven.

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