Inner ear pathology in osteogenesis imperfecta congenita

Abstract

This temporal bone report describes the inner ear deformities which were found in addition to the bony pathology in a case of osteogenesis imperfecta congenita. The labyrinthine pathology includes anomalously positioned and enlarged vestibular spaces, the existence of a scala communis (on one side) and the existence of hematoxylin dark-stained material in the basal zone of the stria vascularis.The appearance of temporal bone reports of osteogenesis imperfecta congenita is sporadic. Friedmann (1974)described changes in the bony capsule in one case of osteogenesis imperfecta congenita Zajtchul and Lindsay(1975)reported three cases of the congenital form of osteogenesis imperfecta with their temporal bone findings. Within recent years, Altmann reported three cases in 1962, Bretlau and Jorgensen reported one case in 1969, Bergstrom and others reported one case in 1972, and Bergstrom described the temporal bone findings in four infants in 1977. The pathologic description of the temporal bone in osteogenesis imperfecta congenita has been focused more or less on the structures of the bony labyrinth and the middle ear ossicles, with a brief description of the inner ear. In this report, we describe anomalous inner ear structures in osteogenesis imperfecta congenita.

Pathology of the otic capsule

“…Sensorineural deafness may also occur in osteogenesis imperfecta. As well as the poorly formed otic capsule, abnormalities of the inner ear have been noted in osteogenesis imperfecta (Igarishi et al, 1980), including abnormalities of the vestibule, the existence of a scala communis and basophilic concretions in the stria vascularis. These concretions can however be seen in a number of conditions and may be of no pathological importance.…”