[Deafness in patients with osteogenesis imperfecta].

This study deals with 8 cases of progressive deafness in patients with abnormalities in growth and skeletal disease, suffering from Osteogenesis imperfecta (5 of them are a familial case). The work is both and audiological and radiological research of the temporal bone, done in order to uncover the reasons of the patient's deafness. This curses just like otosclerosis with a conductive component (fixation of the stapes) and a sensitive one (cochlear demineralization), starting up in adolescence as bilateral and symmetric process. Because of its progressive nature the AA, recommend as treatment the ear prostheses and refuse the surgery of the stapes.