"cochlear implantation in patients with inner ear malformations"

performing cochlear implantation in patients with inner ear malformation has always been a matter of dispute. this study was designed to analyze the operative findings,complications, and postoperative performance of patients with inner ear anomalies who underwent cochlear implantation. six patients with inner ear malformations underwent implantation in our academic tertiary referral center from 1997 to 2002. the average follow-up period was 27 months. malformations included one incomplete partition, one common cavity, one narrow internal acoustic canal (iac) in a patient with riley-day syndrome and 3 cases of large vestibular aqueduct. all received multi-channel implants either nucleus 22 or clarion device. facial nerve was anomalous in 2 cases. csf gusher occurred in 4 patients, which was controlled with packing the cochleostomy site. in all cases, the full length of electrode array was inserted, except one with mondini's dysplasia where insertion failed in the first operation and was referred to another center for a successful surgery on the opposite ear. no other surgical complications were encountered. in 4 cases, all the 22 electrodes could be activated. all patients showed improved hearing performance after implantation. four showed open-set speech recognition. the one with narrow iac showed improved awareness to environmental sounds. in the other case (common cavity), the perception tests could not be performed because of very young age. cochlear implantation in patients with inner ear malformations is a successful way of rehabilitation, although complications should be expected and auditory responses may be highly variable and relatively moderate.