Head and Neck Imaging

Osteogenesis imperfecta

inherited connective tissue disorder characterized by thin and long bones, with a history of multiple fractures, blue sclerae, defective dentition and severe hearing loss. The association of ostegenesis imperfecta and hearing loss is also called van der Hoeve's syndrome. The hearing loss is due to involvement of the otic capsules. The CT findings are essentially the same as those seen in severe fenestral and retrofenestral otosclerosis.

RH