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Osteogenesis Imperfecta (OI) is a genetic connective tissue disorder marked by reduced bone mass, leading to bone fractures, deformities, and growth issues. More than 50% of OI patients experience hearing loss and its management includes hearing aids (HAs), but for patients who do not benefit sufficiently from HAs, cochlear implants (CIs) are an option. Due to histopathological changes such as increased bone vascularity and narrowing of the cochlear spirals, surgery can be more challenging. This study aims to report the case of a patient with osteogenesis imperfecta who underwent cochlear implantation, highlighting possible difficulties and complications associated with the surgical procedure due to the anatomical variations in these patients, as well as potential approaches to the common problems encountered in such cases. Despite limitations and potential difficulties, cochlear implantation remains an important option for hearing rehabilitation, improving the quality of life for these patients.
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Macêdo et al. (2024) studied this question.
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