Van der Hoove syndrome is an inherited genetically determined generalized connective tissue disorder characterized by the formation of „pathologically immature type I collagen“. The literature describes the results of cochlear implantation in patients with van der Howe syndrome, after which there are often complications both intraoperative and postoperative. This article describes two clinical cases of van der Howe syndrome with severe hearing loss and deafness. In cases described: preoperative diagnosis and features of the surgical stage of cochlear implantation and postoperative results. Diagnostic features – thinning of bone tissue according to CT of temporal bones, expansion of cochlear duct, deafness according to tonal threshold audiometry. Cochlear implantation performed with the use of the endotracheal anesthesia under the control of the microscope, straight electrode grids were used, testing was normal. A distinctive feature of this syndrome is the softness of the cochlea’s bone tissue, the danger is that when the electrode array is inserted, it may fall outside the cochlea. Also, during the first connection or later, there may be pathological stimulation of the facial nerve.
Introduction. Petrous temporal bone (PTB) cholesteatoma is an epidermal cyst, which is the result of uncontrolled growth of keratinizing squamous epithelium in the petrous part of the temporal bone. Cholesteatoma is classified into congenital, acquired, and iatrogenic.Objective. To discuss the classification of infralabyrinthine petrous bone cholesteatoma (PBC), add modified classificationand to propose adequate differential surgical management.Methods. The setting was a National Medical Scientific Center of Otorhinolaryngology FMBA (Russia). The data of 14 patients who underwent surgery for different variations of infralabyrinthine PBC from 2017 till 2020 were analyzed and included into the study (with respect to localization type of the approach used, complications, recurrences and outcome). The follow-up period ranged from 6 to 34 months with a median of 18 months.Results. Based on preoperative CT scans and intraoperative findings a Scale of Cholesteatoma extension CLIF(APO) and Modified classification of infralabyrinthine cholesteatoma (in relation to mastoid segment of the facial nerve) are proposed. The scale includes the main anatomical structures of the temporal bone and the adjacent parts of the occipital and sphenoid bones, which may be involved in the cholesteatoma process: cochlea, vestibule and semicircular canals, internal auditory canal, jugular foramen, bony chanal of the internal carotid artery, petrous apex, occipital condyle. Based on the modified classification and scale we present an algorithm for decision making and surgical approach choosing.Conclusion. The implementation of the Scale of Cholesteatoma Extension in Otology and Radiology practice will allow to preoperatively diagnose the extension of PBC, unify the data of the localization of cholesteatoma; allows standardization in reporting and continuity at all stages of treatment. The modified classification proposed by us in this article facilitate the algorithm for selecting the type of surgical approach and determine whether to perform less aggressive combined microscopic approaches with endoscopic control.
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