Summary

Osteopetrosis is a rare bone disorder characterized by a failure of osteoclastic activity. There are two forms of genetic presentation: dominant, which is benign, and the recessive, also called malign. We present the case of a patient suffering from bilateral conductive hearing loss, that was interpreted like clinical otosclerosis, after audiometry. Stapedotomy on the right ear was the indicated management. During the surgery, we found a malformation of the incus, and fixation with the malleus. Incus-stapedian desarticulation, and ossicular reconstruction with tragus cartilage were the procedures made.