Menyer's disease — the disease of an inner ear of not inflammatory character which is shown the repeating attacks of labyrinth dizziness, noise in the affected ear and the progressing decrease in hearing. The list of diagnostic actions at Menyer's disease includes an otoskopiya, researches of the acoustic analyzer (an audiometriya, an elektrokokhleografiya, an acoustic impedansometriya, the promontorialny test, otoakustichesky issue) and vestibular function (a vestibulometriya, a stabilografiya, an indirect otolitometriya, an elektronistagmografiya), MRT of a brain, EEG, EHO-EG, REG, UZDG of vessels of a brain. Treatment of a disease of Menyer consists in complex medicamentous therapy, at its inefficiency resort to surgical methods of treatment, hearing aid.
Menyer's disease carries the name by last name the French physician, in 1861 for the first time the disease which described symptoms. Dizziness attacks similar with described by Menyer, can be observed also at vegeto-vascular dystonia, insufficiency of blood circulation of a brain in the vertebro-bazilyarny pool, violation of venous outflow, a craniocereberal trauma and so forth diseases. In such cases speak about Menyer's syndrome.
The greatest incidence of Menyer's disease is noted among persons of 30-50 years though the age of the diseased can be in range from 17 to 70 years. In children's otolaryngology the disease meets extremely seldom. In most cases Menyer's diseases process has unilateral character, only at 10-15% of patients bilateral defeat is observed. However over time unilateral process at Menyer's disease can be transformed to bilateral.
Causes of a disease of Menyer
In spite of the fact that after the first description of a disease of Menyer there passed more than 150 years, the question of its causal factors and the mechanism of development still remains open. There are several assumptions concerning the factors leading to developing of a disease of Menyer. The virus theory assumes provocative influence of a viral infection (for example, a cytomegalovirus and a virus of simple herpes) which can start the autoimmune mechanism leading to a disease. The hereditary theory is spoken well by the family cases of a disease of Menyer demonstrating autosono-prepotent inheritance of a disease. Some authors point to communication of a disease of Menyer with an allergy. Distinguish from other trigger factors vascular disorders, ear injuries, a lack of estragenes, violations of water-salt exchange.
Lately the greatest distribution was gained by the theory about developing of a disease of Menyer as a result of violation of a vegetative innervation of vessels of an inner ear. It is not excluded that change of sekretorny activity of cages of a labyrinth which produce adrenaline, serotonin, noradrenaline is the reason of vascular disorders.
Most of the researchers studying Menyer's disease consider that increase in intra labyrinth pressure at the expense of a congestion in a labyrinth of excess quantity of an endolymph is its cornerstone. Surplus of an endolymph can be caused by its raised production, violation of its absorption or circulation. In the conditions of the elevated pressure of an endolymph carrying out sound vibrations is at a loss, and also trophic processes in touch cages of a labyrinth worsen. Sharply arising increase in intra labyrinth pressure causes an attack of a disease of Menyer.
Classification of a disease of Menyer
On the clinical symptoms prevailing at the beginning of a disease, the otolaryngology allocates 3 forms of a disease of Menyer. About a half of cases of a disease of Menyer is the share of the cochlear form beginning acoustical frustration. The vestibular form begins according to vestibular violations and makes about 20%. If the onset of the illness of Menyer is shown by a combination of acoustical and vestibular violations, then it is carried to the classical form of a disease making 30% of all cases of a disease.
During Menyer's disease distinguish an aggravation phase in which there is a repetition of attacks, and a remission phase — the period of lack of attacks.
Depending on duration of attacks and temporary intervals between them Menyer's disease is classified by severity. Easy degree is characterized by short frequent attacks which alternate with long breaks in several months or even years, during the mezhpristupny period efficiency of patients completely remains. Menyer's disease of moderate severity is shown by frequent attacks lasting up to 5 watch after which patients for several days lose working capacity. At heavy degree of a disease of Menyer the attack lasts more than 5 hours and arises with a frequency from 1 time a day to 1 time a week, working ability of patients is not restored.
Many domestic clinical physicians use also classification of a disease of Menyer which was offered by I. B. Soldatov. According to this classification during a disease allocate a reversible and irreversible stage. At a reversible stage of a disease of Menyer light intervals between attacks take place, decrease in hearing is caused mainly by violation of the sound carrying out mechanism, vestibular violations have passing character. The irreversible stage of a disease of Menyer is expressed by increase in frequency and duration of attacks, reduction and total disappearance of light intervals, permanent vestibular disorders, considerable and continuous decrease in hearing due to defeat not only sound carrying out, but also sound perceiving ear device.
Symptoms of a disease of Menyer
The main display of a disease of Menyer is the attack of the expressed system dizziness which is followed by nausea and numerous vomiting. During this period patients feel feeling of shift or rotation of the objects surrounding them or feeling of fall or rotation of own body. Dizziness at an attack of a disease of Menyer so strong that the patient cannot stand and even to sit. Most often he tries to lay down and close eyes. In attempt to change position of a body the state worsens, strengthening of nausea and vomiting is noted.
During an attack of a disease of Menyer the congestion, a raspiraniye and noise in an ear, an incoordination and balances, decrease in hearing, short wind, tachycardia, pobledneny persons, the increased sweating is also noted. Objectively in the period of an attack it is observed rotatorny . It is more expressed when the patient with a disease Menyera lies on the affected ear.
Duration of an attack can vary from 2-3 minutes to several days, but most often is in range from 2 to 8 watch. Can provoke emergence of the next attack at Menyer's disease overfatigue, a stressful situation, an overeating, tobacco smoke, alcohol intake, rise in body temperature, noise, carrying out medical manipulations in an ear. In some cases patients with Menyer's disease feel approach of an attack on the aura preceding it which is shown in emergence of small violation of balance or strengthening of noise in an ear. Sometimes before an attack patients note hearing improvement.
After an attack of a disease of Menyer at patients some time remains relative deafness, noise in an ear, weight in the head, a small incoordination, feeling of instability, gait change, the general weakness. Over time as a result of progressing of a disease of Menyer these phenomena become more expressed and long. Eventually they remain throughout the entire period between attacks.
Hearing disorder at Menyer's disease has steadily progressing character. At the beginning of a disease deterioration in perception of sounds of low frequency, then all sound range is observed. Relative deafness increases with each new attack of a disease of Menyer and gradually passes into full deafness. With approach of deafness dizziness attacks, as a rule, stop.
At the beginning of a disease at a slight and medium-weight disease of Menyer at patients staging of process is well traced: alternation of aggravations with the remission periods in which the condition of patients is completely normalized and their working capacity is restored. Further the clinical picture of a disease of Menyer is often aggravated, during remission at patients weight in the head, the general weakness, vestibular violations, decrease in working capacity remain.
Diagnosis of a disease of Menyer
The characteristic picture of attacks of system dizziness in combination with noise in an ear and relative deafness usually allows the otolaryngologist to diagnose Menyer's disease without difficulties. For the purpose of definition of degree of a hearing disorder functional researches of the acoustic analyzer are conducted: audiometriya, research tuning fork, acoustic impedansometriya, elektrokokhleografiya, otoakustichesky issue, promontorialny test.
During an audiometriya the mixed nature of decrease in hearing is diagnosed for patients with Menyer's disease. The voice-frequency threshold audiometriya in initial stages of a disease of Menyer notes a hearing disorder in the range of low frequencies, at frequencies of 125-1000 Hz the bone and air interval comes to light. When progressing a disease the touch type of increase in voice-frequency thresholds of audibility at all studied frequencies is noted.
The acoustic impedansometriya allows to estimate mobility of acoustical stones and a functional condition of intra ear muscles. The Promontorialny test is directed to detection of pathology of an acoustical nerve. Besides, for an exception of a nevrinoma of an acoustical nerve all patients with Menyer's disease need carrying out MRT of a brain. When carrying out an otoskopiya and mikrootoskopiya at patients with Menyer's disease lack of changes of external acoustical pass and an eardrum is noted that allows to exclude inflammatory diseases of an ear.
Diagnostics of vestibular violations at Menyer's disease is carried out by means of a vestibulometriya, an indirect otolitometriya, a stabilografiya. At researches of the vestibular analyzer the hyporeflection, in the period of an attack — a hyper reflection is observed. Researches of a spontaneous nistagm (a videookulografiya, an elektronistagmografiya) reveal its horizontally look. During the period between attacks of a disease of Menyer the bystry component of a nistagm is noted in the healthy party, and at an attack — towards defeat.
The cases of system dizziness which are not followed by a hearing impairment carry to Menyer's syndrome. At the same time consultation of the neurologist, carrying out neurologic inspection, an electroencephalography, measurement of intra cranial pressure by means of EHO-EG, a research of vessels of a brain (REG, transkranialny and ekstrakranialny UZDG, duplex scanning) is necessary for diagnosis of the main disease with which emergence of attacks is connected. At suspicion on the central nature of relative deafness conduct a research of the acoustical caused potentials.
Diagnostics of the increased endolymphatic pressure which is the cornerstone of Menyer's disease is carried out by means of the glitserol-test. For this purpose the patient accepts inside mix of glycerin, water and fruit juice from calculation 1,5g a glitserola on 1 kg of weight. The test result is considered positive if in 2-3 hours when carrying out a threshold audiometriya decrease in acoustical thresholds by 10 dB not less, than at three sound frequencies, or on 5 dB on all frequencies comes to light. If increase in acoustical thresholds is noted, then the test result is regarded as negative and confirming irreversibility of the pathological process happening in a labyrinth.
The differential diagnosis of a disease of Menyer is carried out with a sharp labirintit, evstakhiity, otosklerozy, otitis, tumors of an acoustical nerve, a labyrinth fistula, a vestibular neyronit, psychogenic violations.
Treatment of a disease of Menyer
Medicamentous therapy of a disease of Menyer has 2 directions: long-term treatment and knocking over of the arisen attack. Complex treatment of a disease of Menyer includes the medicines improving microcirculation of structures of an inner ear and reducing permeability of capillaries, diuretic medicines, venotonik, atropine medicines, neuroprotectors. Well proved in treatment of a disease of Menyer , having gistaminopodobny effect.
Knocking over of an attack is carried out by various combination of the following medicines: neuroleptics (trifluoperazina hydrochloride, ), medicines of a skopolamin and atropine, vasodilating means (acid nicotinic, ), antihistaminic (, Dimedrol, ), diuretics. As a rule, treatment of an attack of disease of Menyer it can be carried out in out-patient conditions and does not demand hospitalization of the patient. However at repeated vomiting intramuscular or intravenous administration of medicines is necessary.
Treatment of a disease of Menyer has to be carried out against the background of adequate food, the correct mode and psychological support of the patient. At Menyer's disease it is recommended not to limit physical activity during the periods between attacks, to regularly carry out exercises for a training of coordination and a vestibular mechanism. Drug treatment of a disease of Menyer in most cases promotes reduction of noise in an ear, to reduction of time and frequency of attacks, decrease in their weight, but it is not capable to stop relative deafness progressing.
Lack of effect of the carried-out medicamentous therapy is the indication to surgical treatment of a disease of Menyer. Surgeries at Menyer's disease are subdivided on draining, destructive and autonomic nervous system operations. Various decompressive operations directed to increase in outflow of an endolymph from a cavity of an inner ear belong to the draining interventions. Among them are the most widespread: drainage of a labyrinth through a middle ear, perforation of the basis of a stirrup, a fenestration of the polukruzhny channel, drainage of an endolymphatic bag. Destructive Menyer's disease operations are: intrakranialny crossing of a vestibular branch of the VIII nerve, removal of a labyrinth, lazerodestruktion of a labyrinth and destruction of its cages ultrasound. Intervention on the autonomic nervous system at Menyer's disease can consist in a cervical simpatektomiya, a resection or crossing of a drum string or a drum texture.
The chemical ablyation consisting in introduction to a labyrinth of alcohol, gentamycin or streptomycin belongs to alternative techniques of treatment of a disease of Menyer. At the bilateral nature of defeat of hearing patients with Menyer's disease need hearing aid.
Forecast of a disease of Menyer
Menyer's disease does not pose a threat for the patient's life. But the increasing relative deafness and violations in operation of the vestibular analyzer impose certain restrictions for professional activity of the patient and over time lead to his invalidization. Performing expeditious treatment at early stages of a disease of Menyer is capable to improve the forecast at most of patients, however does not allow to achieve hearing restoration.