Osteogenesis imperfecta (OI), or the Van der Hoeve-de Kleyn syndrome, is a heterogeneous group of connective tissue disorders. The key features in this disease are bone fragility with a tendency to spontaneous fractures and deformations. The classical traid of symptoms involves a conductive and/or sensorineural hearing impairment together with a tendency to spontaneous bone fractures and blue sclerae. Between January 1988 and December 1994, ear surgery was performed on eight ears of six OI patients who presented with mixed hearing loss preoperatively. Pathological changes observed in the middle ear were atrophy and/or fractures of the stapedial crura in combination with thickening and fixation of the stapes footplate. Partial stapedectomy was performed in seven cases and a neo-window was created in the promontory of one patient when an overhanging facial canal obscured visualization of the oval window niche. Pre- and postoperative bone conduction thresholds did not differ in any of the patients. Postoperatively, mean values of the air-bone gap in the main speech frequency range were below 10 dB. Functional results following stapes surgery in patients with otosclerosis during the same time interval (n = 857) did not differ significantly. These data indicate that stapes surgery in OI patients can be performed with the same functional predictability as in otosclerosis patients, even though the underlying etiology is considerably different.