A review of the literature of osteogenesis imperfecta (OI) and hearing loss in the last century is given. This includes the first association in 1912 by Adair-Dighton of OI with hearing loss, the triad of VAN DER HOEVE and DE KLEYN and different clinical and audiometric studies and classifications of the disease. A case report of a 14 year old patient is also included. The genetics of OI and the complexity in correlating genetics with clinical classifications is elucidated. Finally, the findings reported after middle ear surgery, the results of surgery and comparisons with otosclerosis are reviewed.