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Kabuki syndrome is a rare hereditary disease characterized by distinctive facial features, skeletal abnormalities, mental retardation, developmental delay, and anomalies in multiple organ systems development.Congenital hyperinsulinism is a rare manifestation of his Kabuki syndrome. However, early diagnosis is crucial to prevent neurological complications of hypoglycemia.There are 2 types of Kabuki Syndrome depending on severity of symptoms. Kabuki syndrome Type 1 is associated with heterozygous mutations in gene KMT2D. Kabuki syndrome Type 2 is inherited in an X-linked manner. It's associated with heterozygous mutations in gene KDM6A and characterized by more severe course of the disease.This paper presents 2 cases of children with congenital hyperinsulinism as the feature of Kabuki syndrome Type 1 and Type 2.

Older age is characterized by a high prevalence of dizziness/vertigo, including various variants - central and peripheral vestibular disorders, feelings of instability, balance disorders. High frequency of dizziness and chronic cerebral ischemia in the elderly age category supports traditional ideas about relationship of these pathologies. However, there is a known overestimation of Chronic cerebral ischemia as a cause dizziness of and unclear mechanisms of this process. With a high prevalence of vestibular disorders in the elderly and their verification, in some cases, the causes of dizziness cannot be identified. This has prompted the designation of this type of dizziness as «unexplained». The article cites recent studies demonstrating a relationship between «unexplained» dizziness and chronic cerebrovascular pathology. The theories explaining the mechanisms of this association are proposed. The role of choline alphoscerate in the treatment of dizziness in Chronic cerebral ischemia has been demonstrated. The mechanisms of positive effect of choline alphocerate and prospects of its using.

Objective: To study the clinical features and the possibility of objectification of vestibular dysfunction in patients with vestibular migraine., Material and Methods: The study included 46 patients (10 men and 36 women) aged 18 to 50 years, with vestibular migraine and control groups. A targeted collection of complaints was carried out with an assessment of vestibular symptoms; a detailed history; a study of neurological status, with an emphasis on the study of oculomotor reactions, vestibular tests. Functional computer stabilometric tests were used to objectify vestibular dysfunction., Results: Clinical features, as well as features of stabilometric parameters in patients with vestibular migraine have been identified, which can be used to obtain a quantitative assessment of vestibular dysfunction and objectification of vertigo in this category of patients., Conclusion: A purposeful and complete collection of complaints and anamnesis detailing the features of the symptoms in patients with vestibular migraine helps to identify the clinical polymorphism of the pathology in question and contributes to the correct diagnosis. And the use of functional stabilometric tests contributes to the objectification of vestibular dysfunction in this category of patients, quantifying the distinctive features of the balance system in vestibular migraine.

The article provides a brief overview of the epidemiology, diagnosis, clinical manifestations and surgical methods of treatment of labyrinthine fistulas (LF) in patients with chronic suppurative otitis media (CSOM) with cholesteatoma. The efficacy of various LF treatment techniques, their complications, and the principles of prevention of cochleovestibular disorders during surgical treatment are described. The prognostic criteria for the deterioration of bone conduction thresholds during LF surgery, which include a large LF size, the opening of the membranous labyrinth, and the extent of damage to the structures of the inner ear, are presented. A clinical case of surgical treatment of widespread LF in CSOM with cholesteatoma is presented, which proves the possibility of preserving the auditory and vestibular functions while observing the stages of sanitation, manipulations on the LF and obliteration of semicircular canals defects with auto tissues. Combined surgery of such a plan must certainly be accompanied by local instillation of solutions of hormonal preparations in case of a deficiency of perilymphatic fluid in the labyrinth and postoperative antibacterial and hormonal therapy.

Chronic dizziness is defined as a complex of symptoms lasting months or years, including oscillopsia, nystagmus, and postural instability. Diagnostic search includes peripheral vestibulopathy - chronic unilateral vestibulopathy resulting from vestibular neuronitis, schwannoma of the 8th pair of cranial nerves, medical or surgical ablation of the labyrinth, and bilateral vestibulopathy; central vestibulopathy - syndromes accompanied by central vertical nystagmus, small vessel disease in patients with chronic cerebral ischemia; general somatic diseases, peripheral polyneuropathy, side-effects of medications as well as persistent postural-perceptual dizziness. Often, when collecting an anamnesis, it is not possible to identify specific features of dizziness and instability, therefore, clinical and instrumental examination of the patient is of particular importance. In the otoneurological examination, the greatest attention is paid to static/coordination tests, oculomotor tests aimed at identifying signs of damage to the vestibulo-ocular reflex. The principles of treatment depend on the diagnosed cause of dizziness and instability and can, to varying degrees, combine pharmacotherapy, vestibular rehabilitation and psychotherapy, as well as correction of therapy for the underlying disease that caused vestibulopathy.

Vertigo, instability, oscillopsia and concomitant autonomic disorders are classical and well-known symptoms of vestibular disorders. At the same time, recent studies suggest that there are more complicated vestibular dysfunctions caused by the cortical projections of the vestibular system. The central vestibular system includes parietal temporal cortex and insular, anterior intraparietal sulcus, posterior parietal and medial parts of the superior temporal gyrus, singular gyrus retrosplenial cortex, hippocampus and parahippocampal area. The central part of the vestibular system closely interacts with other afferent systems forming a multisensory structure of higher brain functions. Dysfunctions of higher vestibular function play an important role in the development of clinical syndromes including pusher syndrome, room tilt illusion, unilateral spatial neglect syndrome, impairment of spatial memory and navigation. These syndromes can develop due to the direct damage of the cortical vestibular system or as a result of disconnection between the vestibular cortex and other parts of the sensory cortex.

The objective of the present work was to study the influence of the dry extract from the leaves of Ginkgo biloba EGb 761 (used as monotherapy at a dose of 120 mg twice daily during 4 months) on the vestibular function of the patients presenting with cochleovestibular pathology of peripheral and mixed genesis. We present the results obtained by the objective and subjective methods for the evaluation of the vestibular function as well as the neurological and psychoemotional state of the 40 patients that was carried out during the four months of memoplant monotherapy. It is concluded that monotherapy with the use of the dry extract from Ginkgo biloba leaves can be applied for the purpose of improvement of static and dynamic balancing state. Moreover, this memoplant preparation can be used as a means of prophylaxis of recurrent dizziness that in addition reduces the severity of anxiety and depression without producing adverse side effects.

The objective of the present study was to estimate the diagnostic significance of the physical vestibular tests designed to investigate the oculomotor reactions in order to determine the severity of disturbances in the vestibular analyzer during the primary examination of the patients presenting with dizziness symptoms. The results of the study suggest the desirability of using the Frenzel goggles for the detection of the presence of latent spontaneous nystagmus and the performance of additional testing. The study of the vestibular-ocular reflex should begin from the head turn test to be supplemented, in the case of the dubious result, by the dynamic visual acuity test. The smooth pursuit test, the visual saccade test, and the optic nystagmus test need to be performed on a full scale. The subjective visual vertical test should be carried out at the later stages of diagnostics when the detailed investigation of the vestibular function is conducted. The positional manoeuvers are the indispensable components of the examination of the patients presenting with pathological changes in the vestibular analyzer by virtue of their high diagnostic significance for the detection of benign paroxysmal positional dizziness.

The objective of the present publication was to demonstrate a clinical case of peripheral vestibular paroxismia verified in a woman with the help of the MRI technique. Vestibular paroxismia is a relatively rare disease manifested in such characteristic signs and symptoms as sudden and short-lived episodes of dizziness, unstable gait, and the concomitant vegetative disorders accompanied as a rule by tympanophonia, impairment of hearing, and falls. In typical cases, the duration of such episodes varies from several minutes to a few days. A case of vestibular paroxismia associated with the lesion in the peripheral section of the vestibular system is described; it was caused by compression of the nerve by a blood vessel as shown by means of magnetic resonance imaging of cranial nerves.

The objective of the present study was to estimate the efficacy of the combined treatment of spondylogenic cochlear-vestibular disorders with the use of both medicamental and non-medicamental modalities. Computed static stabilometry was applied for diagnostics of postural disbalance and evaluation of the treatment outcomes. It was shown that the application of manual therapy for the management of 56 patients presenting with spondylogenic cochlear-vestibular disorders resulted in the decrease of tinnitus and the improvement of vestibular and cochlear functions.

There were studied peculiarities of provocative nystagmus among 60 military servicemen with nervous system in anamnesis. Latent vestibular disfunction--a frequently detected syndrome among persons with diseases of nervous system in anamnesis. To examine a provocative nystagmus, was used a range of vestibulometric loading tests, which influences on different parts of vestibular system. Was marked a high role of several loading testes in diagnostics of latent vestibular disfunction. It has been found that in conditions of syndrome of vegetative imbalance of different genesis, the most effective are tests of De Klein with hyperventilation and hyperventilation test. In conditions of minimal neurological deficit the most effective is tests of Valsalve. In conditions of peripheral vestibular syndrome in anamnesis and vertebral-genic vestibular disfunction a very effective is sinocarotid test.

The necessity of studying the dynamic stabilization of the human body vertical position is substantiated. New capacities of a stabilometric vector analysis in diagnosing equilibrium dysfunction, evaluating the efficiency of treatment, and determining the statistical significance of differences in the results in a patient rather than in a group of subjects are shown.

Psychosomatic status was studied in 106 patients aged 18-65 years with acute and chronic cochleovestibular disorders. Hearing problems, noise in the ears and vestibular disorders have a negative psychogenic effect in patients with cochleovestibulopathy. The majority of the patients need psychological and psychopharmacological care to relieve anxiety and form adequate attitude to the disease. Correction of psychosomatic disorders raises efficacy of the treatment of cochleovestibular diseases.

The study aimed at (1) identification of the main vestibulometric characteristics of peripheral cochleovestibular syndrome caused by arterial hypertension, atherosclerosis and vegetative vascular dysfunction, (2) elucidation of the pathogenic peculiarities of its formation and (3) evaluation of therapeutic efficacy of betaserc in peripheral vertigo. Eighty-five patients with peripheral cochleovestibular syndrome (PCVS) were studied. On the base of the data of ultrasonic examination of major cranial arteries, arterial blood pressure, central hemodynamics and structural cerebral changes, it is shown that abnormalities of vertebrobasilar system play an important role in PCVS development. Stenosis of spinal and internal carotid arteries was found in a limited number of cases. A conclusion is made on involvment of hemodynamic mechanism of blood circulation insufficiency in the inner ear at microcirculating level in ischemic PCVS pathogenesis. A study of the structural cerebral changes revealed that PCVS were not accompanied by the ischemic foci in the brain. Pharmacotherapy of peripheral vertigo of vascular etiology is based on the right choice of the drugs optimizing blood circulation in combination with vestibulolytics. Therapeutic efficacy of 2-month betaserc course in peripheral vertigo has been proved.

Equilibrium function was investigated with computer-assisted stabilography (CS) in patients with acute neurosensory hypoacusis. This new diagnostic tool was employed in combination with extended vestibulometric and audiologic examinations. Correlations were found between stabilographic and vestibulometric findings. CS is recommended as a method of screening diagnosis in examination of patients with imbalanced equilibrium.

Hearing of 502 subjects occupationally exposed to noise was evaluated using threshold, suprathreshold and speech audiometry. 240 of them worked in conditions of impulse noise, 262 were exposed to permanent noise. Impulse noise proved more harmful for the acoustic analyzer. More unfavourable effect of impulse noise vs permanent one should be accounted for when conducting prophylactic examinations of subjects exposed to impulse noise.

Clinical, electrophysiological investigations, evaluation of autonomic nervous system reactivity with estimation of Kerdo vegetative and stability indices were carried out in 58 patients with cochleovestibulopathy (CVP) and 10 normal controls. CVP patients with compensated disease had sympathicotonia, those with decompensated CVP exhibited parasympathicotonia. Functional loading tests and the stability index values revealed compensatory nature of sympathicotonia. Quantitative and qualitative characteristics of the compensation were related to age (under 40, over 41) and pathogenesis of the disease (central or peripheral). In CVP decompensation poor tolerance of mental work load was reported.