Otosclerosis is a localized progressive disease of bone remodeling within the otic capsule of the human temporal bone. Histomorphologically, active cochlea otosclerosis (otospongiosis) is diagnosed in the presence of foci of demineralization in the otic capsule. The clinical symptoms of otosclerosis are associated with mixed and sensorineural hearing loss. With recent technological advances, diagnostic imaging of the inner ear is becoming more and more important in the evaluation of diseases affecting the cochlea. Studies could demonstrate that otosclerosis has a characteristic appearance on high-resolution computed tomography (HRCT), producing a distinctive pericochlear hypodense double ring. Its appearance on MRI is not as readily appreciated, producing a ring of intermediate signals in the pericochlear and perilabyrinthine regions on T1-weighted images, demonstrating mild to moderate enhancement after gadolinium administration. The ethiology and pathophysiology of otosclerosis are described and a review of the literature is given to illustrate the clinical relevance of imaging in otosclerosis.