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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.

Cochlear implantation in Van der Hoeve de Kleyn syndrome with severe hearing loss and deafness