Korean J Otorhinolaryngol-Head Neck Surg.  2009 Mar;52(3):289-292. 10.3342/kjorl-hns.2009.52.3.289.

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

• Heo JY
• Lee KY
• Park JH
• Lee SH

• KMID: 2277027
• DOI: http://doi.org/10.3342/kjorl-hns.2009.52.3.289

Abstract

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.

Keyword

Osteogenesis imperfecta; Hearing loss; Cochlear implants

MeSH Terms

Adolescent
Adult
Cochlea
Cochlear Implantation
Cochlear Implants
Collagen
Connective Tissue
Ear
Female
Fractures, Bone
Hearing
Hearing Aids
Hearing Loss
Humans
Osteogenesis
Osteogenesis Imperfecta
Otosclerosis
Postoperative Complications
Sclera
Collagen