Osteogenesis imperfecta (OI) is a heterogenous connective tissue disorder. The classical triad of symptoms involves a conductive and/or sensorineural hearing impairment together with a tendency to spontaneous bone fractures and blue sclerae. Between 1993-2004, primary stapes surgery was performed on 14 ears of 11 OI patients who presented with conductive and/or mixed hearing loss. Pathological findings included atrophy and/or fractures of the stapedial crura in combination with thickening and fixation of the stapes footplate and hypervascularity of the promontory mucosa. All the patients with stapes surgery had significant hearing gain and bone conduction thresholds did not differ significantly in any of the cases; the mean postoperative air-bone gap at the main speech frequency range was within 10 dB in 13/14 (93%) and within 20 dB in 14/14 (100%). Hearing results following stapes surgery in patients with otosclerosis during the same time interval (n = 132) did not differ significantly. These data indicate that stapes surgery in OI can be performed safely with comparable functional predictability as in otosclerosis.