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There are several pathologies that can change the anatomy of the otic capsule and that can distort the bone density of the bony structures of the inner ear, but otosclerosis is one of the most frequent. Similar behavior has been shown in patients affected by osteogenesis imperfecta (OI), a genetic disorder due to a mutation in the genes coding for type I (pro) collagen. In particular, we note that otosclerosis and OI can lead to bone resorption creating pericochlear cavitations in contact with the internal auditory canal (IAC). In this regard, we have collected five cases presenting this characteristic; their audiological data and clinical history were analyzed. This feature can be defined as a potential cause of a third-window effect, because it causes an energy loss during the transmission of sound waves from the oval window (OW) away from the basilar membrane. Full article

Background and Objectives: We aimed to identify prognostic computed tomography (CT) findings in retrofenestral otosclerosis, with particular attention paid to the role of otosclerotic lesion area in predicting post-stapedotomy outcome. Materials and Methods: We included 17 subjects (23 ears) with retrofenestral otosclerosis who underwent stapedotomy. On preoperative CT, the presence of cavitating lesion and involvement of various subsites (cochlea, round window [RW], vestibule, and semicircular canal) were assessed. Pre- and post-stapedotomy audiometric results were compared according to the CT findings. The surgical outcomes were analyzed using logistic regression with Firth correction. Results: Cavitating lesions were present in 15 of 23 ears (65.2%). Involvement of the RW was the strongest predictor of unsuccessful surgical outcome, followed by involvement of the internal auditory canal (IAC) and the cochlea. Conclusions: RW and IAC involvement in retrofenestral otosclerosis were shown to predict unsuccessful outcomes. While a “third window” effect caused by extension of a cavitating lesion into the IAC may dissipate sound energy and thus serve as a barrier to desirable postoperative audiological outcome, a “single window” effect due to an extension of retrofenestral otosclerosis into the RW may preclude a good surgical outcome, even after successful stapedotomy, due to less compressible cochlear fluid and thus decreased linear movement of the piston. Full article