A Case of Cochlear Implantation in a Postligual Deaf Patient with Osteogenesis Imperfecta

Osteogenesis imperfecta is a systemic heritable disorder of connective tissues with the predominant manifestation of spontaneous bone fractures. It features an abnormal biosynthesis of collagen with the formation of pathologic, immature collagen, resulting in a triad of brittle bones, blue sclera and hearing impairment. A 39-year-old woman with osteogenesis imperfecta began noticing hearing loss as a teenager. At the time of her presentation, she was aided by a hearing aid, which she had been using for 25 years but had not been benefited from. Her temporal CT shows severe otospongiosis, with radiolucent bone surrounding the cochlea. The patient underwent cochlear implantation to her left ear with no postoperative complication. She has obtained significant hearing improvement after the cochlear implantation. Cochlear implantation in cases of osteogenesis imperfecta with otospongiosis appears to be extremely rare and we present this case with a review of the related literature. (Korean J Otorhinolaryngol-Head Neck Surg 2009;52:289-92)