title: Cochlear implantation in osteogenesis imperfecta.
abstract: A case is reported in which a Nucleus 22 channel intracochlear implant was applied to a Hungarian woman (age 50 yr) with profound deafness associated with osteogenesis imperfecta. Successful intracochlear insertion of the 22 electrodes resulted in a 70 dB hearing improvement at frequencies 250-2000 HZ. Nevertheless, a characteristic facial twitching appeared upon activation of electrodes 9-13. Inactivation of these electrodes abolished the non-acoustic nerve excitation with preservation of acoustic performance. Osteogenesis imperfecta may involve a state of risk for non-acoustic nerve activation in cochlear implant patients possibly resulting from a reduced impedance to current spread by abnormal bone tissue. This, however can be overcome by simple programming manoeuvres.
authors.affiliation: Dept. Otolaryngology, Albert Szent-Gyorgyi Medical University Szeged, Hungary.
authors.forename: J
authors.initials: J
authors.lastname: Szilvássy
authors.forename: J
authors.initials: J
authors.lastname: Jóri
authors.forename: J
authors.initials: J
authors.lastname: Czigner
authors.forename: F
authors.initials: F
authors.lastname: Tóth
authors.forename: Z
authors.initials: Z
authors.lastname: Szilvássy
authors.forename: J G
authors.initials: JG
authors.lastname: Kiss
mesh_terms: D019929:Cochlear Implantation; D003054:Cochlear Implants / Q000009:adverse effects; D003638:Deafness / Q000209:etiology* / Q000534:rehabilitation*; D005154:Facial Nerve / Q000502:physiology; D005260:Female; D006801:Humans; D008875:Middle Aged; D010013:Osteogenesis Imperfecta / Q000150:complications*