Cochlear implant surgery with perimodiolar array insertion in patients with osteogenesis imperfecta: case report and literature review

Osteogenesis imperfecta (OI) is a heterogeneous genetic disease of the connective tissues characterized by a deficit in the synthesis of type I collagen, resulting in bone fragility, defective dentition, ligamentous laxity, vascular fragility, and hearing impairment. In cases of severe-to-profound hearing loss, where patients do not benefit from conventional amplification, cochlear implantation may enable the recovery of functional social hearing. We report a case of cochlear implant surgery with perimodiolar array insertion in a 50-year-old woman with severe-to-profound bilateral mixed hearing loss due to OI, along with a review of the pertinent literature, with the aim to analyze the influence of implant type on postoperative outcomes. The implant was easily inserted and successfully activated 4 weeks postoperatively. No facial twitching was observed during implant mapping. Six months after the activation, the mean free-field threshold with cochlear implant was approximately 25 dB, and the maximum word recognition score was 100% at 50 dB presentation level. Consistent with previous literature, the use of perimodiolar arrays helps to prevent facial nerve stimulation and optimize audiological outcomes.