Heterogeneous Group of Genetically Determined Auditory Neuropathy Spectrum Disorders.

Auditory neuropathy spectrum disorder (ANSD) is often missed by standard hearing tests, accounting for up to 10% of hearing impairments (HI) and commonly linked to variants in 23 genes. We assessed 122 children with HI, including 102 with sensorineural hearing loss (SNHL) and 20 with ANSD. SNHL patients were genotyped for common variants using qPCR, while ANSD patients underwent whole exome sequencing, with variants analyzed across 249 genes.

[Compression elastography as a new method of ultrasound imaging in the differential diagnosis of chronic tonsillitis].

Unlabelled: Chronic tonsillitis remains as an urgent problem in modern otorhinolaryngological practice, which requires improving the diagnostic methods of this pathology.

Objective: To increase the diagnostic information content of objective methods for differential diagnosis of different forms of chronic tonsillitis.

Material And Methods: The study involved 97 patients who were divided into two groups: the 1st group (39 patients) - with a simple form; the 2nd group (58 patients) - with a toxic-allergic form of chronic tonsillitis.

[Assessment of the risk of hearing loss in children with cystic fibrosis].

Background: Cystic fibrosis (CF) is a severe hereditary disease with a multisystem lesion. Manifestations of CF include severe infectious purulent lesions of all parts of the respiratory tract, including purulent rhinosinusitis with nasal polyps. The involvement of the sinonasal region and the need for systemic use of ototoxic drugs (primarily aminoglycosides to treat resistant bacterial infection) potentially create a risk of both conductive and sensorineural hearing loss (SNHL).

[Choanoplasty in children of the first year of life: new opportunities and rehabilitation without stents].

Unlabelled: The article presents literature and our own data on surgical treatment and options for solving the problem of restenosis for congenital choanal atresia in children under one year of age. A new stentless choanoplasty technique using fibrin glue for fixation of posterior septal flaps is presented. This method has patent No.

[Our experience in the treatment of congenital nasal median heterotopias in children and an overview of various treatment tactics].

Unlabelled: Dermoid nasal cysts (congenital nasal median heterotopias) are a rare congenital pathology in children.

Objective: Yo consider the clinical picture, methods of radiation diagnosis and to study the surgical results of a dermoid cyst of the nose according to the literature.

Materials And Methods: A retrospective review of medical histories with the diagnosis of "Dermoid cyst of the back of the nose and nasal cavity, epidermal cyst of the back of the nose, glioma, encephalocele" was conducted from 2017 to 2022 in the Pediatric Otorhinolaryngological Department of the National Medical Research Center for Otorhinolaryngology of the Federal Medical-Biological Agency of Russia.

[Optimization of laser exposure parameters during surgical correction of soft palate tissues in patients with ronchopathy and obstructive sleep apnea syndrome].

Objective: Substantiation of optimization of laser radiation parameters when performing surgery on the soft palate to improve the results of treatment of patients with ronchopathy and obstructive sleep apnea syndrome.

Material And Methods: Based on the experience of performing laser sculptural uvulopalatoplasty in 309 patients with ronchopathy and obstructive sleep apnea syndrome, a rational choice of parameters of laser radiation used during the operation is justified.

Results: Optimization of laser radiation parameters during laser sculptural uvulopalatoplasty allowed to improve the positive results of treatment of patients with ronchopathy and obstructive sleep apnea syndrome in 98.

[Jugular foramen anatomy in lateral skull base paraganglioma surgery].

Unlabelled: In this article we present the surgical approaches to the temporal bone paraganglioma based on the anatomical studies.

Objective: To detalize the anatomy of the jugular foramen according to the comparison of cadaver dissections findings and the CT scans data that were performed before the dissections, for improvement of quality of treatment of patients with temporal bone paraganglioma (Fisch type C).

Material And Methods: The data of CT scans and the steps of the approaches to the jugular foramen (retrofacial and infratemporal approaches with opening of jugular bulb and identification of the anatomical structures of jugular foramen) were analyzed on 10 cadaver heads, 20 sides.

Adult Cochlear Implantation Under Local Anesthesia and Conscious Sedation with Dexmedetomidine: Efficacy and a Method to Interact with the Conscious and Cooperative Patient.

Background: This study describes the efficacy of cochlear implantation under local anesthesia with conscious sedation with dexmedetomi- dine in adult patients and proposes a method to communicate with the conscious and cooperative patient intraoperatively. This less invasive anesthetic procedure is suitable for patients with comorbidities preventing general anesthesia.

Methods: Unilateral cochlear implantation with Oticon Medical systems was performed in 10 adult patients with comorbidities preventing general anesthesia.

[New criteria for professional selection and professional suitability by hearing to work in conditions of exposure to industrial hazards].

New regulations of professional selection and professional suitability for the state of auditory function for persons entering for the first time and already working under the influence of harmful and (or) hazardous production factors are discussed.

[A clinical case of radio-induced neurogenic sarcoma of the soft tissues of the neck].

The article presents a clinical observation of the surgical treatment of a patient with radio-induced neurogenic sarcoma of the soft tissues of the neck that occurred 6 years after radiation therapy with SOD 66 Gy for stage II laryngeal cancer T2N0M0.

[The effectiveness of auditory training using virtual reality technologies in persons with chronic sensorineural hearing loss].

Objective: To study the effectiveness of auditory training using virtual reality technologies in people with chronic sensorineural hearing loss in dynamics.

Material And Methods: The study included 68 patients aged from 24 to 97 years (mean age 57.63±15.

The Interdisciplinary Approach and New Technologies in the Scientific and Clinical Development of Otorhinolaryngology.

The experience of the FMBA NMRCO of Russia in implementing the concept of an interdisciplinary approach and the use of new technologies in the diagnosis, treatment, and rehabilitation of diseases of the ENT organs is discussed. It is noted that otorhinolaryngology is closely associated with a number of basic medical sciences and related clinical disciplines, which is reflected in the structure of the center: in addition to otorhinolaryngological departments, in which high-tech surgical interventions on ENT organs are performed, there are scientific and clinical departments: maxillofacial and plastic surgery, ophthalmology and neurosurgery, as well as the country's only center for professional pathology of ENT organs. Thus, in the general structure of the NMRCO's medical services, the share of medical care in specialties related to otorhinolaryngology is about 40%.

[Modern methods of early diagnosis of tumors of the larynx and pharynx].

Unlabelled: Worldwide, there is a constant increase in mortality from malignant neoplasms, which is largely due to late diagnosis. One of the reasons for late detection is the lack of conditions for conducting a detailed examination at the outpatient level, since the routine method of examining ENT organs today remains examination using mirrors, but in recent decades, endoscopic methods of examination have become widespread - indirect endoscopy, fibrolaryngoscopy, contact endoscopy, NBI-endoscopy, endoscopy using light filters of the SPIES system, as well as diagnostics using fluorescent technologies. Also, a promising and new direction is the diagnosis of malignant neoplasms at the preclinical stage and precancerous conditions, for which molecular diagnostic methods are used - SCCA and microRNA.

[Anatomical and functional results of ossiculoplasty with adjustable length titanium prostheses with and without hydroxyapatite].

Objective: To compare the anatomical and functional results of the ossicular chain reconstruction with classic titanium adjustable prostheses and titanium adjustable prostheses with hydroxyapatite cap.

Material And Methods: The 300 patients (360 cases) with chronic suppurative otitis media were examined and operated. The patients are divided into 2 groups.

[Audiological profile of patients with SARS-Co-V-2 PCR-positive cases].

COVID-19 is a new pandemic caused by a novel coronavirus, SARS-CoV-2. COVID-19 has spread throughout China and received worldwide attention. On 11 February 2020, World Health Organization (WHO) officially declared COVID-19.

[The histiocytoma of the oropharynx (clinical cases)].

The paper presents a case of rare localization histiocytoma of the oropharynx. Some authors consider the existence of this category of tumors controversial, others prefer to consider them tumors of unclear Genesis. Benign fibrous histiocytoma is quite common in the form of a small node with fairly clear boundaries, most often localized on the skin of various parts of the body.

[Tumor-like amyloidosis of the upper respiratory tract].

The paper describes 11 cases of local tumor-like amyloidosis (LTA) of the upper respiratory tract, among which laryngeal amyloidosis was most common. The clinical diagnosis of suspected local amyloidosis was made in only two cases. The diagnosis of local amyloidosis was established at a morphological examination of a distant neoplasm, by using special Congo red staining followed by polarizing microscopy.

[Human papillomavirus and Epstein-Barr virus in the pathogenesis of inverted papilloma and associated sinonasal carcinoma].

Objective: To determine whether Epstein-Barr virus and human papillomavirus (HPV) types 6, 11, 16, 18, 35, and 43 DNA can be present in inverted papilloma (IP) and associated sinonasal carcinoma.

Material And Methods: The investigation was carried out using tissue samples obtained during surgery from 76 cases of IP and 4 cases of sinonasal carcinoma that had developed in the presence of IP. PCR was performed in 41 cases of IP and in 4 cases of sinonasal carcinoma; immunohistochemistry was done in 15 cases of IP to identify HPV L1 capsid protein.

[The interdisciplinary approach to the rehabilitation of the patients presenting with congenital atresia of the external auditory canal and the concomitant microtia].

The objective of the present study was to develop and introduce into the clinical practice the method for the combined aesthetic and functional rehabilitation of the patients presenting with congenital atresia of the external auditory canal (CAEAC) and the concomitant microtia. A total of 8 patients at the age from 6 to 21 years with unilateral CAEAC and microtia were given the surgical treatment. During the intervention, atresia was resolved using the trans-mastoid approach, tympanoplasty of autofasciae and ossiculoplasty making use of the partial titanium prosthesis and the placement of cranial osteointegratable titanium implants.

[The results of bilateral cochlear implantation in the children who survived meningitis].

The objective of the present study was the prospective analysis of the results of bilateral cochlear implantation (CI) in the children presenting with bilateral ossification of the cochlea after they had survived meningitis. A total of 15 patients underwent the surgical intervention. In those exhibiting bilateral ossification of the basal cochlear helix over the 5 mm segment (up to first bend of the cochlear turn) and partial ossification of the second helix (in 6 children), the affected portions were removed with the placement of two choleostomies, the lower one (from the ossified membrane of the cochlear window) and the upper one (toward the second helix).

[Nasal glial heterotopia: Clinical and morphological characteristics].

The paper describes a case of nasal glial heterotopia in a 10-month-old girl with a mixed (intranasal and subcutaneous) localization, which is accompanied by the divergence of the nasal bones. Histological examination supplemented by immunohistochemical reactions with antibodies to vimentin, S100 protein, neuron-specific enolase, as well as Ki-67 and smooth muscle actin confirmed the neural nature of the tumor. Fields of mature astrocytic glia including individual cells with neuronal differentiation were found among the fibrous and fibrovascular tissues.

[The non-damaging method for the insertion of a standard electrode for cochlear ossification].

The objective of the present study was to develop the non-damaging method for the insertion of a standard electrode for cochlear ossification with a view to improving the results of hearing and speech rehabilitation of the patients presenting with grade IV sensorineural impairment of hearing. Twenty preparations of the cadaveric temporal bone were used to investigate topographic and anatomical relationships in the main structures of the middle and internal ears, viz. the second cochlear coil, vestibulum and its windows, processus cochleaformis, spiral lamina, and modiolus.

[PECULIARITIES OF ANESTHESIA DURING COCHLEAR IMPLANTATION IN PEDIATRICS.].

The Aim: analysis of the use of laryngeal mask in anesthesia for cochlear implantation.

Materials And Methods: 10 patients aged from 1 year to 5 years were operated on according to the classical method KI with the use of laryngeal masks. As anesthesia was performed a balanced multimodal anesthesia by Sevoflurane and Fentanyl.

[Optimization of the approach to the spiral (Rosenthal's) canal of the cochlea in the patients presenting with cochlear-vestibular abnormalities].

The objective of the present study was to improve the effectiveness of cochlear implantation (CI) in the patients presenting with cochlear-vestibular abnormalities based on the development and practical application of the algorithm for the insertion of an electrode arrayinto the spiral (Rosenthal's) canal of the cochlea taking into consideration the specific anatomical features of the middle and inner ears. The study included 25 patients with congenital malformations of the inner ear and bilateral grade IV sensorineural loss of hearing or deafness selected for CI. Indications for drilling a cochleostomy were the high localizationof the jugular bulb and the absence of its bone wall (5 patients, 20%).