Osteogenesis imperfecta (OI) is an inherited connective-tissue disorder of variable penetrance. With OI, the triad of blue sclera, osseous fragility, and a conductive hearing loss is known as the van der Hoeve-de Kleyn syndrome. Blue sclera with a conductive loss may be a clinical subgroup of OI. Clinical findings and long-term surgical results in 62 operations in 43 patients with blue sclera are given. Osteogenesis imperfecta differs from otosclerosis in the following ways: (1) earlier onset (in the second and third decades of life), (2) more severe middle ear involvement, and (3) a higher incidence of sensorineural hearing loss. One year after stapedectomy, 38 (75%) of 51 operations had complete closure of the air-bone gap. Of the 24 operations followed up for an average of seven years (range, two to 18 years), 15 patients (62%) had no deterioration in their immediate postoperative hearing gain. Our study supports the concept that OI is clinically distinct from otosclerosis and establishes surgical intervention for its conductive hearing loss as a reasonable alternative to amplification.