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Otosclerosis is a complex and progressive disease of pathological bone remodeling that affects the otic capsule of the temporal bone, resulting in hearing loss. Although traditional diagnostic methods are still used, improvements in technology and research have paved the way for additional diagnostic techniques and advancements. The traditional treatment of otosclerosis, stapes surgery, is now being augmented or replaced by innovations in hearing aid technology and cochlear implants. Earlier diagnosis of otosclerosis can occur through understanding of the cause, risk factors, and current diagnostic testing.