[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.

[Neuroendocrine adenoma middle ear]. / Neiroendokrinnye adenomy srednego ukha.

Neuroendocrine adenoma (NEA) is an extremely rare pathology and accounts for less than 2% of all ear tumors. The article provides an overview of the diagnosis, classification, treatment methods and algorithm for monitoring patients with NEA of the middle ear. 6 cases of NEA of the tympanic cavity are described in detail, which were diagnosed and treated in GBUZ NIKIO named after N.N. L.I. Sverzhevsky DZM for the last 5 years. Diagnosis of NEA of the middle ear is possible when performing high-resolution multislice computed tomography of the temporal bones and magnetic resonance imaging with contrast enhancement, however, the neoplasm can be verified only after a histological examination with the determination of the immunophenotype. Differential diagnosis of NEA of the tympanic cavity with other processes of the middle ear must be carried out at each stage of the diagnostic path. Surgical treatment, depending on the volume of education, allows you to remove it completely and improve the auditory function.

[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.

[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.

[Atypical inflammation of the middle ear]. / Atipichnoe techenie vospaleniia v srednem ukhe.

The objective of the present study was to characterize the patients presenting with atypical inflammation of the middle ear and consider the currently available methods for their examination. A total of 20 patients at the age from 16 to 66 years were admitted to the Department of Ear Microsurgery during the period from 2008 and 2016 for the treatment of atypical inflammation of the middle ear. Eleven of them (18 ears) were found to have tuberculous lesions (TL) of the middle ear while the remaining 9 patients (11 ears) suffered giant cell vasculitis (GCV). All the patients underwent the general clinical and otorhinolaryngological examination, computed tomography of the temporal bones and the thoracic cavity organs, cytological, bacteriological, pathomorphological, and molecular-genetic studies including PCR diagnostics, rheumatological tests, as well as counseling by a phthisiotherapist and rheumatologist. The primary localization of TL in the middle ear was documented in 6 patients including its association with lung lesions in 5 cases. The clinical picture of the disease in 5 patients was that of smoldering exudative pathology and in 6 ones was accompanied by suppurative perforative otitis media. According to the laboratory analyses, bacteriological diagnostics proved efficient in 9% of the patients, pathomorphological and cytological diagnostics in 18% and 27.3% of the cases respectively while the effectiveness of PCR diagnostics was estimated at 55%. The diagnosis in individual patients was established within the period from 1 month to 1.5 years after they first sought medical advice in connection with complaints of the ear disease. Tuberculosis of the middle ear began to develop as exudative middle otitis that acquired the form of bilateral pathology in 4 patients. Three patients had a concomitant pulmonary disease. In 4 patuents, the diagnois of middle ear tuberculosis was established based on the presence of the specific antibodies and in 5 ones based on the results of the pathomorphological study. All the studies were carried out in duplicate. The period between the beginning of the disease and the establishment of the definitive diagnosis varied from 1 month to 1 year.

[Desmoplastic fibroma of the temporal bone]. / Desmoplasticheskaya fibroma visochnoi kosti.

This paper reports a case of the extremely rare disease, the desmoplastic fibroma of the temporal bone (DF). The clinical course of this pathology, the methods for its diagnostics, and surgical treatment are described.