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.
Migirov
Lela
L
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
Henkin
Yael
Y
Hildesheimer
Minka
M
Kronenberg
Jona
J
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