Otosclerosis or otospongiosis is a bone degeneration that occurs in the otic capsule, the bone structure that surrounds the cochlea and labyrinth. Is an aberrant process of bone resorption of the labyrinthine capsule followed by reparative deposition of new, immature sclerotic bone (Abdurehim, 2016) . This disease most often starts at the base of the stapes, which is the smallest bone in the human body, receiving the name of fenestral otosclerosis. Over time, it can progress to the cochlea and even reach the internal auditory meatus. Therefore, it is far from being a simple “calcification” of a small ear bone, requiring correct diagnosis, long-term follow-up, and personalized treatment.