Osteogenesis imperfecta of the stapes: an histological study.

Two cases of Van der Hoeve's Syndrome are presented with histological findings consistent with the diagnosis of osteogenesis imperfecta. These findings support the view that otosclerosis can be clearly differentiated histologically from osteogenesis imperfecta. Pre-operative impedance studies are recommended on each patient with osteogenesis imperfecta as a useful diagnostic adjunct to determine whether the conductive component in the hearing loss is secondary to stapedial crural deformity and/or footplate fixation.