Otosclerosis

otosclerosis, ear disorder characterized by abnormal bone growth in the middle ear, typically affecting the stapes (stirrup), a bone in the region of the oval window. It is at the oval window that the footplate of the stapes comes into contact with the fluids of the inner ear and acts as a piston to conduct sound energy from the eardrum into the fluids of the inner ear. In otosclerosis, a gradual buildup of new spongy bony tissue around the stapes welds it against the wall of the surrounding bone and immobilizes it, preventing the vibrations that permit soundwaves to travel through the ear. The result is conductive hearing loss. Sensorineural hearing loss, which affects the inner ear, sometimes also occurs, frequently in combination with conductive hearing loss. Sensorineural hearing loss usually emerges late in the disease’s course, when otosclerosis has progressed to affect structures in the cochlea.

Otosclerosis appears to be a hereditary disorder. It is the most common type of progressive hearing impairment in young adults; onset typically is between ages 10 and 30. It usually affects one ear before the other (but both eventually) and occurs more frequently in females than in males. Surgery is generally the most effective treatment and usually today consists of a stapedectomy, in which the encrusted stapes is removed and replaced by a plastic or wire substitute. Patients with mild otosclerosis and patients whose hearing loss persists after surgery may benefit from the use of a hearing aid.