Cochlear implantation in a child with osteogenesis imperfecta.
Osteogenesis imperfecta (OI) is a hereditary disease of connective tissue and affects bone, dentine, sclera, joint, tendon, blood vessels, heart valves, and skin. Approximately 50% of the adult patients with OI have associated hearing impairment. To date, only three cases of cochlear implantation in adults with OI have been reported, but none in children. We present a case of cochlear implantation in a congenitally deaf 6-year-old boy with OI. The Nucleus 24 Contour device was successfully implanted using the suprameatal approach (SMA). At 6 months post-initial stimulation there was no evidence of non-acoustic nerve excitation (i.e. facial twitching) or discomfort, and significant progress in auditory abilities was manifested by open set word identification.
Department of Otolaryngology and Head and Neck Surgery, Sheba Medical Center, Tel Hashomer 52621, Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel. henkin@post.tau.ac.il
Lela
L
Migirov
Yael
Y
Henkin
Minka
M
Hildesheimer
Jona
J
Kronenberg
D001299:Audiometry; D002648:Child; D019929:Cochlear Implantation; D003638:Deafness / Q000209:etiology* / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D008297:Male; D010013:Osteogenesis Imperfecta / Q000150:complications* / Q000503:physiopathology / Q000601:surgery; D020127:Recovery of Function / Q000502:physiology