течение трех последних десятилетий отмечен значительный рост выживаемости детей с онкологическими заболеваниями. Такие результаты лечения достигнуты благодаря использованию программной химиолучевой терапии, имеющей как непосредственные, так и отдаленные токсические эффекты. Цисплатин и карбоплатин широко применяются в онкологической практике. Платиносодержащие препараты включены в состав химиотерапевтических режимов, используемых для лечения опухолей головного мозга, нейробластомы, гепатобластомы, нефробластомы, остеогенной саркомы, герминогенно-клеточных и ряда других опухолей у детей. Один из дозолимитирующих эффектов применения препаратов платины-ототоксичность, манифестирующая двусторонним необратимым снижением слуха. Механизм развития цисплатиновой ототоксичности обусловлен накоплением платины в различных структурах улитки: в сосудистой полоске, в базилярной мембране, вестибулярной лестнице. Повреждения получают опорные клетки органа Корти (клетки Дейтерса), затем слуховые рецепторы: наружные и внутренние волосковые клетки. Цисплатин и карбоплатин при проникновении внутрь клеток вызывают высвобождение проапоптотических факторов и образование большого количества свободных радикалов, что в свою очередь запускает каскад реакций,

Cisplatin and its derivatives are widely used chemotherapeutic agents for the treatment of many cancers, including hepatoblastoma, brain tumors, and germ-cell tumors. This therapy contributed to the dramatic increase in the survival rate. However, its use is restricted by the high incidence of irreversible ototoxicity associated with cisplatin application (in more than 60% of the children receiving it). Some studies have reported that genetic variants of TPMT (rs 12201199), COMT (rs4646316), and ABCC3 (rs 1051640) are conferring increased risk of developing cisplatin-induced hearing loss. However, in other studies the results were not replicated. In the present study, we replicated the previous studies based on an independent cohort of Russian patients. SNP genotypes for rs 12201199, rs4646316 and rs 1051640 were determined in DNA samples obtained from 16 patients who developed hearing loss and a group of 34 patients whose hearing was retained. The association between TPMT (rs 12201199), COMT (rs4646316), and ABCC3 (rs 1051640) variants and the hearing loss was not observed in our cohort.

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.