Audiological findings after multichannel cochlear implantation in patients with Mondini dysplasia

Mondini dysplasia is a congenital malformation of the inner ear. To date, five individuals with this malformation have received cochlear implants at the South of England Cochlear Implant Centre. The aim of this study was to review the audiological findings of these individuals after implantation. The soundfield thresholds after implantation are in the region of 30-40 dB (A). The results of suprathreshold speech recognition tasks show substantial variability in performance but this is no greater than that obtained from implant users with no malformation. All individuals were able to detect and recognize a variety of environmental sounds that would previously have been inaudible. These findings, along with the reported improvement in quality of life, mean that Mondini dysplasia is not a contra-indication for multichannel cochlear implantation. This information will be useful to other centres when considering implantation in similar patients.