Cochlear implantation in children with anomalous cochleovestibular anatomy.

Our aim was to determine the influence of inner-ear anomalies on surgical difficulty and postoperative audiologic outcomes among pediatric cochlear implant (CI) recipients at our institution. We reviewed medical and audiologic records from 78 consecutive pediatric CI cases between 1985 and June 2009. Thirty patients had high-resolution temporal bone computed tomography imaging available for retrospective interpretation. Seven of these 30 patients (23%) had cochleovestibular dysplasia. Fifty percent of patients with severe dysplasia had a cerebrospinal fluid gusher intraoperatively, compared with 13% of patients with no dysplasia. Of patients with available audiologic outcome data, 17 of 26 patients with normal/mild/moderate dysplasia were able to complete CNC testing, whereas neither of the 2 patients with severe dysplasia could complete the open set test. Our experience suggests that surgical difficulty and audiologic outcomes in pediatric CI recipients may be affected by the presence and severity of a cochleovestibular anomaly.