[The evolution of ideas about cyst-like stretching of the paranasal sinuses. Part I]. / Evolyutsiya predstavlenii o kistovidnom rastyazhenii okolonosovykh pazukh. Chast' I.

Treatment of patients with severe chronic and recurrent forms of sinusitis, complicated by pathological stretching of the paranasal sinuses, is not a trivial task. This is especially true for those clinical cases where a pathological increase in the size of the sinus leads to widespread destruction of its walls and may be accompanied by serious complications from adjacent structures. The paper presents an analytical review of publications on the topic of pathological stretching of the paranasal sinuses. Potential factors influencing the development of this pathology, mechanisms of pathogenesis and classification options are described in detail. Modern approaches are considered in the surgical treatment of this pathology, which can be carried out in one or two stages, depending on the presence of secondary aesthetic defects.

[Intraosseous cavernous hemangioma of the middle turbinate. Clinical cases]. / Vnutrikostnaya kavernoznaya gemangioma srednei nosovoi rakoviny. Klinicheskie sluchai.

Intraosseous vascular pathology of the turbinates is extremely rare in the practice of an otorhinolaryngologist and can be presented in various histopathological variants. The article presents two clinical cases in which an intraosseous cavernous hemangioma was hidden under the mask of a hypertrophied middle turbinate. The final diagnosis was established by the results of histological examination. The analysis of these clinical cases indicates that, despite the low prevalence, atypical clinical and CT picture, intraosseous formations of the nasal cavity can be of a vascular nature and certainly require a comprehensive examination, including CT, CT with contrast and/or MRI of the nose and paranasal sinuses. These clinical observations indicate that preliminary embolization of feeding vessels before surgical treatment is not required.

[Features and results of surgical rehabilitation of hearing loss in osteogenesis imperfecta]. / Osobennosti i rezul'taty khirurgicheskogo lecheniya tugoukhosti pri nesovershennom osteogeneze.

Osteogenesis imperfecta (OI) is a form of congenital osteoporosis. Depending on the type of OI, patients experience various types of hearing loss. Depending on the type and degree of hearing loss, various methods of hearing rehabilitation are used in this category of patients. OBJECTIVE: To evaluate the features and results of surgical rehabilitation of hearing loss in patients with osteogenesis imperfecta. MATERIAL AND METHODS: During the period from 2009 to 2022, 2221 primary stapedoplasty was performed in the department, of which 23 (1.04%) in 21 patients were performed in patients with OI. There were 14 women and 7 men. According to TPA, bilateral hearing loss was detected in 19 patients and unilateral in 2. Conductive hearing loss was observed in 9 cases and mixed - in 14. The average thresholds for bone conduction (BC) were 22.7±8.04 dB, and the bone-air interval (ABG) - 36.1±5.3 dB. According to CT of the temporal bones, all patients showed a bilateral and symmetrical decrease in the density of the auditory ossicles, and in 7 patients there were extensive areas of non-uniform decrease in the density of the bone labyrinth up to +500 - +1000 HU.21 patients underwent 23 operations: in 21 cases stapedoplasty with laser assistance and in 2 cases ossiculoplasty. RESULTS: BC thresholds 6 months after surgery averaged 24.6±8.2 dB, and ABG - 12.1±2.9 dB. Closing of ABG ≤10 dB at spoken frequencies was detected in 30.5%, ABG ≤20 dB - in 95%. After 12 months or more after the operation, no change in the audiological parameters was noted. CONCLUSIONS: Stapes surgery for conductive and mixed hearing loss in OI patients is functionally effective. The best results are achieved after therapy with bisphosphonates with preparations of sodium fluoride, calcium and vitamin D, performing the operation when the density of demineralization zones reaches 1000 HU and using laser assistance. Taking into account the demineralization of the bone structures of the temporal bone, it is recommended to use autocartilaginous stirrup prostheses to restore sound conduction or to cover the attachment area of other prostheses with autologous tissues to prevent necrosis of the long stalk of the incus and stabilize long-term functional results.

[Clinical and radiological classification of otosclerosis]. / Kliniko-rentgenologicheskaya klassifikatsiya otoskleroza.

The article presents various classifications of forms of otosclerosis (OS), which change with the development of diagnostic methods. At the same time, according to the literature, a unified OS classification has not yet been adopted. All existing classifications are imperfect to some extent. The classification of clinical forms of OS according to TPA data makes it possible to determine the indications for surgical treatment and to suggest its possible effect, but not the localization of OS foci. X-ray classifications of localization of OS foci indicate their diversity, distribution, and do not always correlate with the type of hearing loss. At the same time, modern diagnostics of OS should be based on audiological data, localization of foci and their density according to the results of X-ray methods of examination. Based on the examination and treatment of 1532 patients with various forms of OS, a modern clinical and radiological classification of the disease is proposed, based precisely on these provisions. This classification, in our opinion, will improve the quality of diagnosis of various forms of OS, will allow to differentiate the tactics of treating patients with this disease to stabilize hearing loss, indications for surgical treatment, suggest its effectiveness with a reduction in the risk of surgical failures and possible further rehabilitation of the patient.

[Spontaneous otoliquorrhea, complicated by recurrent secondary meningitis, in a 6-year-old child with an abnormality of the inner ear]. / Spontannaya otolikvoreya, oslozhnennaya retsidiviruyushchim vtorichnym meningitom, u rebenka 6 let s anomaliei razvitiya vnutrennego ukha.

Congenital anomalies in the inner ear structures development are one of the reasons for unilateral hearing loss in children. Unilateral hearing loss is predominantly congenital, thus children with this pathology are initially deprived of the ability to hear with both ears, which leads to the formation of a specific «individual hearing norm¼. Due to this, children do not complain of hearing loss, and unsuspecting parents can only guess why their child is poorly studying and lagging behind his peers. Unfortunately, in some situations the detection of malformations of inner ear structures occurs only after the development of complications, namely spontaneous otoliquorrea, leading to bacterial meningitis, and exactly episodes of meningitis (sometimes recurrent) become the cause for examination of patient and diagnosis of this pathology. We present one of this clinical observations.

[Partial sequestration of the labyrinth in a child with chronic suppurative otitis media with cholesteatoma]. / Chastichnaya sekvestratsiya labirinta u rebenka s khronicheskim gnoinym srednim otitom s kholesteatomoi.

A clinical case of a 16-year-old child with chronic suppurative otitis media with cholesteatoma complicated by partial labyrinth sequestration is presented. The case describes is a rare disorder. Computed tomography of temporal bones consistent with intraoperative data was decisive in establishing the diagnosis and the surgery extent.

[Atresia or severe stenosis of the external auditory canals with normal auricles in children]. / Atreziya ili vyrazhennyi stenoz naruzhnykh slukhovykh prokhodov pri normal'nykh ushnykh rakovinakh u detei.

In the article we present the experience of observing 27 children with bilateral stenosis or atresia of the external auditory canals with normal or slightly hypoplastic auricles. Three children had bilateral stenosis of the external auditory canals due to abnormal or premature fusion of the skull bones. The remaining 24 children had external auditory canals atresia in the bone part or throughout. 9 of them had a syndrome of deletion of the long arm of chromosome 18, 1 child had a mosaic form of Edwards syndrome. The average age at the time of diagnosis was 4 years 3 months. Atresia of external auditory canals in normal auricle is often caused by abnormalities of 18 pairs of chromosomes, and severe stenosis of external auditory canals can be observed in children with craniostenosis and other anomalies of the skull bones.

[Unilateral subjective tinnitus «vanishing bone¼ disease (Goram-Stout syndrome).] / Odnostoronnii sub"ektivnyi shum pri bolezni «ischezayushchei kosti¼ (sindrom Gorkhema ­Stouta).

The article presents the case history of an 11-year-old girl with Goram-Stout disease, who debuted with unilateral subjective tinnitus. A review of the literature, cases with damage of the temporal bones and modern methods of treatment of this disease is made.

[Diagnosis and surgical outcomes in the management of petrous bone cholesteatoma]. / Diagnostika i effektivnost' khirurgicheskogo lecheniya kholesteatomy piramidy visochnoi kosti.

OBJECTIVE: To evaluate the effectiveness of surgical treatment of patients with petrous bone cholesteatoma (PBC) depending on the localization of the pathological process. MATERIAL AND METHODS: The analysis of surgical treatment using various surgical approaches and its results in 32 patients with PBC, depending on the type, localization in petrous bone and intraoperative findings, is presented. Patients with supralabirint PBC underwent extended atticoantromastoidotomy with tympanoplasty and mastoidoplasty with automaterials (n=19), labyrinthectomy (n=4), subtotal petrozectomy with labyrinthectomy and suturing of the external auditory meatus (EAM) (n=2). In infralabirint and infralabirint-apical PBC, a transotic approach was used with Rambo suturing of EAM (n=9). The pre-sigmoid approach was performed in 1 patient. With an extradural subtemporal approach, PBC of apical localization was removed in 1 case. RESULTS: After surgical treatment, hearing remained at the same level in 15 (47%) patients, 14 of them had deafness. In the early postoperative period, a temporary increase in bone conduction hearing thresholds by 10-20 dB was detected in 14 (44%) patients with their gradual recovery over 3 months. Deafness in the postoperative period developed in 3 (9%) patients after removal of supralabirint cholesteatoma. In the early postoperative period, 3 (9%) patients developed systemic dizziness, which was stopped after 3 months. In 25 (78%) patients, the function of the facial nerve in the early postoperative period remained at the same level, of which 14 (44%) were normal, and 11 (34%) had the same degree according to the House-Brackmann (HB) classification. Improvement of function by one degree of HB classification was observed in 4 (12.5%) patients on average 5 months after surgery. CONCLUSION: An adequate personalized choice of surgical treatment methods allowed mainly to preserve the function of hearing and facial nerve. In cases of deterioration of facial nerve function in the postoperative period, gradual improvement was observed for 3-10.5 months with further positive dynamics.

[Tendoplasty and its effectiveness in the rehabilitation of hearing loss in patients with otosclerosis]. / Tendoplastika i ee effektivnost' pri khirurgicheskoi reabilitatsii patsientov s tugoukhost'yu, obuslovlennoi otosklerozom.

The article briefly presents the physiology of the stapes muscle tendon (SMT), and features in its defeat. Its length was studied using high-resolution multispiral computed tomography of the temporal bones. We also studied the possibility of its restoration using the author's method of tendoplasty using metallized stapes prostheses. Tendoplasty with stapedoplasty was performed in 74 patients with otosclerosis (OS), and 48 patients had stapedoplasty without tendoplasty. As a result of research, the average length of the SMT was 2.38±0.02 mm, which explains the need to use a 3 mm long venous autograft for tendoplasty. The author's method of tendoplasty for stapedoplasty allows restoring the acoustic reflex in 54.1% of OS patients using artificial stapes prostheses. The preservation of the vascular bed in the thickness of the restored tendon can improve the trophy of the long incus process and reduce the risk of its dystrophic changes in the postoperative period. In addition, this fact confirms the importance of the stapes muscle in the acoustic reflex.

[Clinical and diagnostic criteria and differential diagnosis in the first and second stages of the development of Ménière's disease - A modern view of the problem]. / Kliniko-diagnosticheskie kriterii i differentsial'naya diagnostika pri pervoi i vtoroi stadiyakh razvitiya bolezni Men'era: sovremennyi vzglyad na problemu.

The results of a comprehensive examination of 82 patients with clinical signs of definite Ménière's disease, unilateral lesion and confirmed by extratympanic electrocochleography endolymphatic hydrops are presented. The results of the study showed that only 38% of patients had cochleovestibular syndrome due to Ménière's disease. In 45% of patients, Ménière's disease was combined with other diseases: benign paroxysmal positional vertigo, vestibular migraine, persistent postural-perceptual dizziness, superior semicircular canal dehiscence. In 17% of patients cochleovestibular syndrome was due to other reasons: vestibular migraine, tumor of the posterior cranial fossa, superior canal dehiscence syndrome, Cogan's syndrome, enlarged vestibular aqueduct, otosclerosis. Complaints, medical history of the disease and life, brain MRI, temporal bone CT and pure tone audiometry in dynamics are important in suspected Ménière's disease. For 1 and 2 Ménière's disease stages the most important characteristic are: progressive unilateral sensorineural hearing loss and reduced slow faze velocity values of caloric nystagmus in dynamics, mainly in warm response if attacks of vertigo is maintain; normal head impulse test between vertigo attacks and pathological result of this test in attack of vertigo with the normalization of gain during the first day.

[A case of obliteration of a round window in otosclerosis]. / Sluchai obliteratsii kruglogo okna pri otoskleroze.

The article presents a brief review of the literature on the anatomy and physiology of the round window (RW) niche, the causes of occlusion, the diagnosis of this pathology, the features of auditory disorders and tactics when it is detected in patients with otosclerosis (OS). A clinical case of diagnosis and effective surgical treatment for obliteration of RW in a patient with advanced OS, which occurred in 2019 in 0.7% of cases, is described. Removal of RW niche obliteration was carried out with a curette and microcresis by smoothing the canopy over RW until partial visualization of the secondary membrane. It was this stage of the operation that made it possible to restore the normal hydrodynamics of the inner ear fluids and contributed to a functional result. Thus, the diagnosis of RW obliteration in patients with OS is difficult, but possible when using computed tomography of the temporal bones and assessing the mobility of the RW membrane or analyzing changes in hearing at the end of the operation, if it is performed under local anesthesia. The simultaneous performance of stapedoplasty and the removal of bone obliteration of RW makes it possible to safely achieve functional rehabilitation in patients with OS.

[Acoustic neurinomes of childhood]. / Nevrinoma preddverno-ulitkovogo nerva u detei.

The article presents a review of the literature on the vestibular schwannoma of childhood, the features of its clinical manifestations, diagnostic methods, methods of treating education and indications for their use.

[Artificial temporal bone]. / Iskusstvennaya visochnaya kost'.

This article presents assembly technology and the main stages of dissection on artificial temporal bone. This sample of artificial temporal bone is a domestic product. The use of this material makes it possible to develop basic dissection skills, such as anthromastoidotomy, posterior tympanotomy, and facial nerve decompression. Artificial temporal bone can be used as a teaching tool for students, residents and postgraduate students who train otosurgical skills in the form of basic stages of dissection work on complex structures of the temporal bone.

[Exudative otitis media as a manifestation of spontaneous otoliquorrhea]. / Ekssudativnyi srednii otit kak proyavlenie spontannoi otolikvorei.

Spontaneous otoliquorrhea (SOL) often mimics the picture of exudative otitis media (EOM), which leads to delayed diagnosis of the underlying disease. PURPOSE OF STUDY: Is to evaluate causes, clinical features and diagnostic algorithm for patients with SOL. PATIENTS AND RESEARCH METHODS: From 2007 to 2018, 33 patients with SOL underwent examination and treatment in the Department of Ear Microsurgery of the Institute. At the preoperative stage, all patients underwent a complete examination, including examination of ENT organs, anthropometric, biochemical, audiological, ultrasound and radiological tests, which made it possible to make the correct diagnosis. RESULTS: SOL was more likely to occur in women over 50 who are obese (69.7%, stage II-III). The disease onset in 51.6% of patients was preceded by conditions accompanied by a short-term or prolonged increase in intracranial pressure. SOL was manifested by the clinical picture of EOM in 72.7% of cases, only in 45.5% of cases was accompanied by release from the ear and nose, was often intermittent and complicated by meningitis in 12.1% of patients. Difficulties in diagnosing the disease are caused by clinical manifestations of exudative or acute otitis media in the absence of a causative factor, which led to the verification of SOL from 1 to 4 years in 81.9% of patients who received long-term treatment for other ENT diseases. CONCLUSION: The master factors in the diagnosis of SOL are otomicroscopy and endoscopy of the nasopharynx, Halo test and screening determination of glucose level in the obtained discharge, as well as high-resolution CT of the temporal bone, which allows to localize the cerebrospinal fluid fistula and to determine the size of the defect.

[Variants of treatment of the head direction at the festulars of the labi-rint of inflammatory genesis]. / Varianty lecheniia golovokruzheniia pri fistulakh labirinta vospalitel'nogo geneza.

PURPOSE: To analyze the effectiveness of various methods of treating dizziness with fistulas of the labyrinth (FL) of inflammatory genesis. An analysis of the surgical treatment of 216 patients with chronic suppurative otitis media (CSOM) with cholesteatoma complicated by FL using a variety of FL treatment methods is presented. All patients underwent a debridement operation on the middle ear or revision of the postoperative cavity: in 48 patients with a matrix of cholesteatoma in situ on the fistula, in 109 patients with matrix removal followed by plastic repair or FL filling with various autotissues, in 59 patients with matrix conservation, plastic removal or filling FL and SLD labyrinth. In the last two groups, filling of a semicircular canal (SC) defect was performed in 33 patients, and mastoidoplasty - in 47. CONCLUSION: All used FL treatment techniques are effective in the treatment of peripheral vestibular disorders. Careful treatment of SC fistula after removal of all pathological conditions in the absence of active inflammation and the appointment of hormone therapy in the postoperative period are the main factors in preserving the functions of the labyrinth in any surgical technique. Filling of the lumen of the SC and mastoidoplasty in patients with CSOM with FL can eliminate peripheral dizziness.

[Diaphanoscopy palatal tonsils with use of the diode laser]. / Diafanoskopiia nebnykh mindalin s primeneniem diodnogo lazera.

For the purpose of decrease in intraoperative blood loss during a tonsillectomy the technique of a laser diaphanoscopy (DS) of the palatal tonsil (PT) and paratonsil space is developed. In the conditions of a transillumination of tissues of throat vessels paratonsil space which are preventively coagulated by the holmium laser are visualized. In carrying out DS of structures of a throat the 'pilot' red laser of the surgical laser LUMENIS Versa Pulse Power Suite 20W system was used. In a research there were 60 patients with chronic tonsillitis. Laser DS was carried out before tonsillectomy, at the same time 120 PT were subjected to raying. As result of the conducted research established that 60% of PT (n=72) anatomic were deeply shipped in a sidewall of a throat, 44,2% of PT (n=53) - had the expressed top pole, 0,8% of PT (n=1) - an additional segment. Also it was established that laser transillumination of paratonsil space is possible in the conditions of infiltration of fabrics isotonic physiological NaCl solution of 0,9%. Carrying out preventive coagulation of vessels of paratonsil space of Ho:YAG laser in the conditions of DS allows to reduce the volume of intraoperative blood loss to 4,17±0,37 ml that is 10,1 time less in comparison with the tonsillectomy which is carried out on traditional techniques.

[Middle ear osteoma: case report and literature review]. / Osteoma barabannoi polosti. Klinicheskii sluchai i obzor literatury.

The short literary review of frequency of occurrence, complaints, results of diagnostics and surgical treatment of patients about osteomine is presented to a drum cavity in article. Two own cases of clinical inspection and surgical treatment of patients about osteomine of a drum cavity are described.

[The application of stapedoplasty for the treatment of hearing loss in the patients suffering from obliterative otosclerosis]. / Stapedoplastika v lechenii tugoukhosti u bol'nykh obliteriruiushchim otosklerozom.

This article presents the results of analysis of the data obtained during the examination and the surgical treatment of the patients presenting with the obliterative form of otosclerosis and suffering from hearing impairment with special reference to the intraoperative findings, technical aspects of stapedoplasty, and its effectiveness. A total of 14 patients (17 ears) were recruited for the participation in the present study including 10 women at the mean age of 38.8±6.2 years and 4 men (mean age 44.8±3.9 years). The duration of the hearing loss in the period preceding the surgical treatment in 7 (50%) patients was more than 8 years. According to the results of tonal threshold audiometry (TTA), the mean bone conduction (BC) threshold for conductive hearing loss in the frequency range from 0.5 to 4.0 kHz was 24.9±8.1 dB with the mean bone air gap (BAG) equaling 38±5.1 dB. Computed tomography (CT) of the temporal bones revealed grade 1 obliterative otosclerosis in 4 patients, grade II of the same condition in 6 patients, and grade III in 7 ones. These findings were confirmed intraoperatively. In 15 cases, stapedostomy was carried out with the use of the non-contact CO2 laser-based system, in the remaining cases a microdrill was employed. Five patients underwent laser-assisted piston stapedoplasty while in 11 others the stapes prosthesis was placed on the autovein. One patient was treated by the same method with the use of the autocartilaginous prosthesis. The functionally acceptable results within 1 year after the surgical intervention were obtained in all the treated patients with the mean bone air gap equaling 13.2±3.4 dB. The best outcome (the reduction of the BC threshold and BAG by 7 dB and 25.9 db on the average respectively within 1 year after surgery was achieved in the patients with grade III obliterative otosclerosis.

[Results of molecular genetic testing in Russian patients with Pendred syndrome and allelic disorders].

Pendred syndrome is an autosomal recessive inherited disorder characterized by a combination of sensorineural hearing impairment and euthyroid goiter; its clinical manifestation in children is hardly distinguishable from nonsyndromic hearing loss. Pendred syndrome is one of the most frequent types of syndromic hearing loss. Hearing impairment is accompanied by abnormal development of the bony labyrinth­enlarged vestibular aqueduct (EVA) and occasionally combined with Mondini dysplasia. Mutations in the SLC26A4 gene, which encodes the pendrin protein, are responsible for both Pendred syndrome and for allelic disorder (nonsyndromic enlarged vestibular aqueduct). The present study for the first time conducted molecular genetic analysis in 20 Russian patients with Pendred syndrome, EVA and/or Mondini dysplasia. As a result, six pathogenic mutations in the SLC26A4 gene were revealed in four patients. The mutation c.222G>T (p.Trp74Cys) was detected for the first time. Mutations were found in patients with Pendred syndrome and nonsyndromic EVA with or without Mondini dysplasia. Mutations were not detected in patients with isolated Mondini dysplasia. One proband with clinical diagnosis Pendred syndrome was homozygous for the c.35delG mutation in the GJB2 gene. The absence of frequent mutations, including well-known ones or "hot" exons in the SLC26A4 gene, was reported. Therefore, the optimal method to search for mutations in the SLC26A4 gene in Russian patients is Sanger sequencing of all exons and exon-intron boundaries in the SLC26A4 gene.