Osteogenesis imperfecta.Osteogenesis imperfecta (fragilitas ossium) is an inherited connective tissue disorder characterized by osteoporosis, blue sclera sclera: see eye. , poor dentition dentition, kind, number, and arrangement of the teeth of humans and other animals. During the course of evolution, teeth were derived from bony body scales similar to the placoid scales on the skin of modern sharks. , and mixed deafness. There is severe demineralization demineralization /de·min·er·al·iza·tion/ (de-min?er-al-i-za´shun) excessive elimination of mineral or organic salts from tissues of the body. de·min·er·al·i·za·tion n. of the bone involving the otic capsule, which is remarkably similar to that which occurs in cochlear otosclerosis otosclerosis: see deafness. (otospongiosis). [1] The hearing loss is believed to be the result of encroachment of the reparative vascular and fibrous tissue in and about the cochlea or the result of hemorrhages and microfractures. Despite their differences, osteogenesis imperfecta and cochlear otosclerosis are similar on computed tomography with respect to both fenestral fe·nes·tra n. pl. fe·nes·trae 1. Anatomy A small opening, especially either of two windowlike apertures in the medial wall of the middle ear. 2. The opening in a bone made by surgical fenestration. and cochlear manifestations (figure). Reference (1.) Swartz JD, Harnsberger HR. The otic capsule and otodystrophies. In Swartz JD, Harnsberger HR, eds. Imaging of the Temporal Bone. 2nd ed. New York: Thieme Medical Publishers, 1992:192-246. From the Department of Radiology, MacNeal Hospital, Berwyn, Ill. |
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