Otosclerosis is a disease affecting the bony metabolism of the otic capsule and stapes footpalate, and is among the most common causes of acquired hearing loss. It occurs most commonly among Caucasians with an incidence of 1%, and women are affected twice as often as men. Otosclerosis is considered a multifactor disease, caused by both genetic and environmental factors. It is inherited in an autosomal dominant pattern with incomplete penetrance. The role od autoimmunity, viruses, hormones and many other factors in the etiopathophysiology of disease has been subject of recent studies, but the exact cause of otosclerosis is unknown. Progressive conductive hearing loss is the predominant symptom but patients also present with tinnitus and, rarely, dizziness. The clinical presentation usually occurs between the second and fifth decades of life. The diagnosis is based on medical history, clinical features, and hearing tests. The audiometric exam typically shows conductive hearing loss with a wider air-bone gap at lower frequencies. The stapedial reflex is absent when stapedial fixation is present. As medical treatment with sodium fluoride can only slow down the progression of disease, surgical management is preferred. Approaches to surgical management include total stapedectomy, partial stapedectomy, and stapedotomy.