Ménière's disease

In Ménière 's disease ( Meniere's disease ) is a disorder of the inner ear, which is characterized by bouts of vertigo, unilateral hearing loss and ringing in the ears ( tinnitus). If these three symptoms in common, it is called by the Meniere's triad. The exact cause of Meniere's disease is not known. There are a large number of treatment methods that can influence the course of the disease favorably, but are discussed controversially.

The disease usually occurs between the ages of 40 and 60 and affects women slightly more often than men.

The name comes from the French physician Prosper Ménière (Paris, 1799-1862 ) back.

• 3.1 hearing tests
• 3.2 Imaging Methods

• 4.1 acute cases treatment
• 4.2 Surgical procedures
• 4.3 seizure prophylaxis

Symptoms

Meniere's attacks occur intermittently and are repeated usually at different distances. Several years long intervals may elapse between the individual seizures. It is also possible that only one or two attacks in a patient's life may occur. Not infrequently, the disease starts without complete Meniere's triad, ie occur only hearing loss and tinnitus or vertigo on their own, a classic Menière'scher attack occurs later. In these cases, if need be suspected of having a so-called " monosymptomatic Meniere's disease " are expressed, for example in recurrent Hörstürzen. Only when then a classic Meniere's attack occurs, the diagnosis can be considered certain.

Dizziness

Typical of a Meniere's attack is severe vertigo with nausea and vomiting. The vertigo lasts for minutes to hours and may be so severe that the patient can no longer stand. The dizziness is worse when moving, but is present even at rest. Patients, therefore, to keep your head perfectly still endeavor.

No balance problems are perceived Between attacks. Much longer or much shorter dizziness times speak for other conditions as Meniere's disease (about positional vertigo, lack of blood supply to the inner ear, inflammation ). If the time between attacks is a constant dizziness without other features of Meniere's attacks on, it is in this " additional fraud," often to psychogenic dizziness, which can be explained inter alia by principles of classical and operant conditioning and stimulus generalization.

As Tumarkinsche Otolithenkrise or vestibular drop attacks is called sudden falls without loss of consciousness in patients with Meniere's disease. Approximately 5 % of patients with Meniere's disease suffer from it. The disease was first described in 1936 by A. Tumarkin.

Hearing loss

In the attack, the hearing of the affected ear, connected to a noise in the ear (tinnitus ), and a feeling of pressure deteriorated. Hearing loss and tinnitus can disappear at more frequent seizures and deafness remain mostly consist of tinnitus after the first attacks. Hearing loss may precede the frame of Meniere's disease years. Hearing loss affects Menière's disease usually particularly bass frequencies, remarkably often are complaints about a distorted hearing. The degree of hearing impairment is not related to the severity and frequency of seizures.

If during a Meniere's attack to an improvement of a pre-existing hearing loss, one speaks of the ( rare ) Lermoyez syndrome. Whether it is a special form of Meniere's disease or a stand-alone disease is still unclear.

Tinnitus

Simultaneously with the hearing loss occurs tinnitus or worsening. In Meniere's disease, tinnitus is often low frequency. The impairment by tinnitus is often pronounced only moderate in strength or low Menière's disease.

Aura

Not infrequently, a feeling of pressure in the ear and a gain of tinnitus occurs already shortly before the attack, making the attack the patient may announce itself.

Causes

The cause of this disorder endolymphatic hydrops is a ( cochlear hydrops, excess pressure in the cochlea ) suspected. The exact cause of this Endolymphstaus is not released. Some studies estimate that a Endolymphstau can have several causes. A chronic or recurrent Endolymphstau but on the other hand probably a single, specific cause. Thus, not every cochlear hydrops automatically lead to Crohn's Meniere's disease, which develops from the first sign of dizziness and hearing impairments to full training in an average of one year. Conversely, however, to assume that every Crohn's Meniere's disease is associated with a Endolymphschwankung.

The exact pathogenesis has not been elucidated. A common theory is that too high a pressure on the Reissner membrane to rupture of the membrane or increased permeability of the endolymphatic leads. Thus, the potassium-rich endolymph can mix with the sodium-rich perilymph. However, the separation of the ions is important for the electrical Zell-/Nervenebene processes in the inner ear, since it maintains an electrical potential difference. Therefore, by the mixture of the two fluids, there is a wrong signal transmission to the brain. This is by the patient as long-lasting vertigo perceived. By physiological processes the Reissner membrane is closed again and restore the potential difference. Due to the scarring of the Reissner membrane by these attacks the hearing of the persons concerned is getting worse, which can lead to a complete loss of hearing over time. Recent research results suggest a multifactorial pathogenesis appear to be possible, for example, can cause a significant change in stimulus transmission pressure-sensitive ion channels of the hair cells.

Diagnosis

Typically, patients report a sudden onset of rotational vertigo with nausea, tinnitus and hearing loss, which is repeated at regular intervals. A particularly important part of the diagnostic process is therefore questioning the patient ( history ). The " Meniere's triad ", however, is not always present in the complete expression; especially at the beginning, the disease can also run monosymptomatic, which makes evaluation difficult.

To further confirm the diagnosis ( differential diagnoses ) studies of the middle ear (otitis media), the inner ear ( sudden hearing loss ), the hearing and balance nerve ( acoustic neuroma, vestibular neuritis ) and the brain are performed (multiple sclerosis, migraine from Basiliaristyp ) and made ​​an equilibrium test to to identify diseases with similar symptoms.

Hearing tests

• Audiogram: With the hearing test, the hearing of the ears via air and bone conduction is checked. The examination of the bone conduction is important, in order to distinguish from those of the middle ear hearing loss in the inner ear can. In Meniere's patients an inner ear hearing loss for low frequencies is often found.
• Otoacoustic emissions: The otoacoustic emissions measure the sound that the ear emits a occurring outside stimulus. The otoacoustic emissions are the byproduct of the active sound reinforcement, which takes place in the inner ear. By measurement of the otoacoustic emissions the functionality of the outer hair cells may be checked.
• BERA: The BERA ( Brainstem Evoked Response Audiometry ) the ear is stimulated with sound pulses. Electrodes on the head measure the currents it generated by the brain stem. The study allows conclusions about whether the sound is forwarded without problems via the auditory nerve. For unique measurement results to an acoustic neuroma can be so largely exclude.
• Glycerolbelastungsprobe / glycerol test: With the Glycerolbelastungsprobe a endolymphatisches happening in the inner ear and thus a Meniere's disease can be demonstrated. For this purpose, the subject drinks a glass with the high sweet drink. If the substance of the gastric mucosa enters the bloodstream, there is a concentration gradient between blood and endolymph. To compensate for the concentration gradient, diffuses the endolymph fluid into the blood. Thereby, the pressure is reduced in the inner ear. Then, when at least three adjacent frequencies an improvement of 10-15 dB is detectable in Tonschwellenaudiogramm, a endolymphatisches happening is proven.
• Induced air line (AC, air Conducted ) cervical and ocular vestibular evoked myogenic potentials (AC cVEMP, AC oVEMP ): Using the cVEMP ( Sakkulusfunktionsprüfung ) and oVEMP (analysis of overriding Utrikulusanteils ) may contain statements about the function of the otolith organs are made. Especially the Sakkulusfunktion is often impaired early due to its central location in the organ of balance.

Imaging methods

To exclude an acoustic neuroma, a magnetic resonance examination should be performed ( under administration of contrast medium and T2 -weighted images ). Since the endolymphatic hydrops in Japan could be shown even by contrast-enhanced magnetic resonance imaging for the first time in 2007, this method is also used in Europe are increasingly used. First study centers in Germany are the Hospital of the University of Munich and the University Hospital of Münster.

Treatment

The Meniere's disease itself is not curable, but many effects can be compensated or low influenced.

Acute cases treatment

In acute cases often help with medication dimenhydrinate ( Vomex ) for the treatment of nausea. For permanent treatment of elevated Endolymphdrucks betahistine is currently recommended by the professional associations agent, with positive impact studies are made on the basis of systematic errors in question. In the first few weeks of taking the medication may cause diarrhea, a slight or an increased sensitivity of the skin to sun rays. In severe and frequent attacks ( > 2 per quarter ) Doctors sometimes recommend a therapeutic trial with a low dose loop diuretic ( eg, furosemide). Again, the unproven effect to emphasize.

Circulation-promoting measures in the form of medication or the compressed air chamber are indeed frequently administered in inner ear disorders such as sudden hearing loss or tinnitus, but have with a confirmed diagnosis of Meniere's disease no sense. Other highly sought-after procedures such as low-level laser therapy, in which the outer ear is irradiated with a red light laser, are more questionable, since the laser light - is the attributed to a beneficial effect on the sensory cells in the inner ear - not physically get there.

Surgical procedures

A current also historically reclaimed overview of the methods of therapy Menière's disease is found in Leif Erik Walther. Here it is pointed inter alia to the levels therapy in Meniere's disease. For very frequent dizziness, an engagement at the endolymphatic sac ( Sakkotomie ) can be made. In this operation, the skull bone is drilled away to the saccule in the sense of decompression ( pressure relief ). The intervention aims that the sacculus expand better and thus the endolymph can be better absorbed. In addition, the endolymphatic sac may be opened permanently to allow a flow of endolymph ( eg via a silicone rail). It often forms a Neo - sac. Frequently, the vertigo attacks by this operation in their numbers from, but the procedure is not always successful and success is not always consistent ( about 50-60% of patients experience an improvement in the vertigo attacks ). The fact that these numbers are more than a placebo effect could not be demonstrated so far.

Another way to turn off the dizziness, is the partial or total elimination of the vestibular system with gentamicin. This treatment is evidence-based. With treatment for years lasting vertigo attacks the patient should be avoided. The effect of gentamicin in the inner ear is not completely elucidated. The function of the five equilibrium receptors must not be compromised in any case. The failure of the vestibular system and a partial loss of hearing in this treatment is accepted because it already belongs to the typical image of Meniere's disease. A only one-sided equilibrium loss is compensated for in the rule. This agent is a last resort and should only be used in very severe impairment and even then only when sure it is clear that the vestibular system is responsible for the dizziness (and not disturbances in the brain ). According to a recent study, the intratympanic gentamicin therapy of saccotomy is superior in some respects. With the help of cervical and ocular vestibular evoked myogenic potentials and the video head impulse test monitoring of receptor function is now possible but also necessary in the context of intratympanic gentamicin therapy.

A new treatment method is the labyrinth anesthesia. In this case, an anesthetic is introduced through a small incision in the eardrum into the middle ear. From there, the anesthetic diffuses into the organ of balance and calm there balance and stunned by this. The vertigo can be reduced by, or even turn off for years. The method can be repeated investigations revealed regarding the potential risk of hearing impairment different results.

A final treatment option consists of surgical transection of the hearing and balance nerve ( vestibulocochlear nerve ) with loss of hearing of the ear. Certain movements must then be re-learned.

For all operational ( Sakkotomie, transection of the eighth cranial nerve ) and non- operational ( gentamicin administration, labyrinth anesthesia) operations on the inner ear is important to note that the disease in the course can affect both organs of equilibrium. A hasty elimination of a page can therefore lead eventually to a complete loss of balance or hearing.

Seizure prophylaxis

For the treatment between attacks, various methods are available. These are more aimed to strengthen the people as a whole, as against the disease ( relaxation techniques, psychotherapy, balance tests ). Any kind of physical activity is recommended because over here after a vertigo attack again confidence in one's own equilibrium system can be recovered. Voted applies particularly rich in potassium and sodium diet. The required daily amount of potassium is approximately equal to the amount in two deciliters soy drink (= 2 g potassium). Likewise, noise, alcohol, nicotine and negative stress is to be avoided.

Spelling of the name

Prosper Ménière himself wrote only with a grave accent on the second "e", which is confirmed by several handwritten letters with his signature. In the literature, there are often other spellings ( Ménière, Menier ), some of which stem from the irritating lettering on the grave chapel of Ménière family on the Montparnasse Cemetery in Paris.