The article provides literature data and description of the clinical case of CHARGE syndrome with an autosomal – dominant type of inheritance in a child of 3 years and 7 months. The name of the syndrome is formed by the first letters of the characteristic congenital malformations: “C” (coloboma) – optic disc coloboma, “H” (heart defects) – cardiovascular abnormalities, “A” (atresia of choanae) – atresia or stenosis of choan, “R” (retardation of growth and development) – growth deficit, developmental delay, “G” (genital anomalies) – genital anomalies, “E” (ear abnormalities and sensorineural hearing loss) – hearing organ abnormalities. A feature of the clinical case is the early diagnosis of CHARGE syndrome, manifested by bilateral optic disc coloboma; unilateral paresis of the facial nerve; congenital heart disease (open aortic duct); retardation of growth, psychomotor and psycho-speech development; cryptorchidism; bilateral anomaly of the outer ear (short, wide, absence of the lobe and external curl), sensorineural hearing loss; bilateral renal hypopalasia, chronic kidney disease.

Rhinogenic intracranial complications, such as brain abscess and thrombosis of the veins and sinuses of the brain, are still a formidable, life-threatening pathology, often resulting in death, despite the widespread development of antibiotic therapy and adherence of patients to the treatment. Often the cause of the development of intracranial complications is the exacerbation of a chronic inflammatory process in the paranasal sinuses, while acute ENT pathology of the sinuses leads to the development of rhinogenic complications only in a small percentage of cases. Unfortunately, the high development of radiation and laboratory diagnostics does not always allow diagnosing these conditions in time and providing adequate surgical care. In childhood and adolescence, it is important to perform a timely complete and at the same time minimally invasive, non-disabling, surgical intervention. An analysis of foreign literature in recent years shows that neurosurgical operations in various volumes (from trepanopuncture of the brain to craniotomy) occur in the vast majority of cases of treatment of rhinogenic intracranial complications. The article presents two clinical cases of patients with intracranial complications successfully treated in the Department of Otorhinolaryngology of St. Petersburg State Pediatric Medical University without a highly traumatic neurosurgical operation. The combination of endonasal endoscopic surgery and massive adequate conservative therapy (antibiotic therapy in combination with the use of anticoagulants for sinus thrombosis) allows the patient to recover without resorting to neurosurgical intervention.

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Dynamic assessment of hearing in pediatric patients having chronic suppurative otitis media with cholesteotoma was conducted. The patients included into the study were assessed after surgeries performed by canal wall up or canal wall down technique without any timpanoplasty. Functional outcomes and frequency of recurrence were estimated. We carried out a retrospective and prospective analysis of hearing ability in 35 children who had undergone surgery in ENT clinic of Saint-Petersburg State Pediatric Medical University (37 ears were operated on as two patients had bilateral lesions). Hearing ability was investigated by tone threshold audiometry. All the patients studied were followed up for not less then 1 year. Hearing ability was evaluated before the surgery,6 months and 12 months after the surgery. Hearing findings before the surgery in children operated on by canal wall up technique showed a more significant hearing loss than the ones done by the other technique. All ears demonstrated no significant hearing loss progress in both groups. However, the children after canal wall up surgery showed more frequent cholesteatoma recurrences which resulted in the need of other surgeries. Carrying out canal wall up surgery on the middle ear in children makes it possible to maintain hearing at a socially adequate level.

The high prevalence of hearing loss in children determines the need for reliable methods for the timely detection and diagnosis of hearing impairment at any age, starting from a birth. Otoacoustic emissions are widely used in hearing screening and audiological assessment as an objective tool for cochlear status evaluation. Over the past 30 years, their use in routine audiological assessments has increased significantly. Understanding the subtle processes that occur in the cochlea during the transmission of acoustic stimuli which generate otoacoustic emissions as well as knowledge about the registration parameters, otoacoustic emissions characteristics, otoacoustic emissions advantages and constraints are important for results analysis. Contemporary understanding of the occurrence of auditory sensations as well as the description of various types of otoacoustic emissions used in routine clinical practice (transient otoacoustic emissions and distorting product otoacoustic emissions) are presented in the review. The features of otoacoustic emissions using in pediatric practice are described: in newborns hearing screening, including the peculiarities of applying of this test for infants having had got their treatment in the intensive care units. Otoacoustic emissions significance for hearing diagnosis and as well as for ototoxicity monitoring is shown. Reliability, non-invasiveness, objectivity, simplicity of the otoacoustic emissions testing has done it one of the main methods both in hearing screening and diagnostics for children on any age.