Introduction. Hearing loss affects 1 to 2 out of every 1000 newborns. Detecting anomalies in the inner ear is a challenging task even for experienced specialists. Aim. To develop a fully automated sequence of commands with a pipeline data transfer for the classification of inner ear defects and processing of CT images of inner ear anomalies in patients using this program data. Materials and мethods. This study presents the first automated method for classifying congenital inner ear anomalies. In the experimental part, a 3D cochlear structure network was developed for 346 standard and 121 atypical structures using a common segmentation scheme trained exclusively on normal anatomy. From 2018 to 2024, 98 patients were examined at the Federal State Budgetary Institution of Science, Otolaryngology, Federal Medical and Biological Agency of Russia, including 54 (55.5%) boys and 44 (44.5%) girls aged from 8 months to 6 years (average age 2.5 years) with inner ear developmental anomalies and severe hearing impairments, who subsequently underwent cochlear implantation. Results. We achieved a generalized average accuracy of 77% across 7 different pathological subgroups of disorders compared to the professional diagnosis of an otolaryngologist specializing in congenital inner ear defects. Discussion. Although automatic detection of various types of inner ear anomalies is essentially a classification task, the lack of representative and heterogeneous datasets that accurately represent the diversity of these congenital developmental defects necessitates the use of a parametric approach. This method is employed with standard data to extract implicit information that could potentially detect anomalies in a non-controlled manner. Conclusions. We proposed the first method for the automatic detection of congenital anomalies of the inner ear and demonstrated that the use of 3D information about the shape of the cochlea, extracted using a model trained exclusively on standard structures, is sufficient for classifying developmental defects. © Попадюк ВИ, Диаб ХМ, Пащинина ОА, Михалевич АЕ, Харири М, Шукурян МА, Кириченко ИМ, 2025