Results from four cochlear implant patients with Usher's syndrome

Individual results are presented of 4 patients with Usher's syndrome type 1 who received a cochlear implant. Both single-channel and multichannel implants were used. Because of implant failure, one of the single-channel systems was replaced by a Nucleus multichannel system. Results are compared to the results of 5 other prelingually deaf cochlear implant users. The performance of the patients with Usher's syndrome on suprasegmental and segmental speech perception tests and on a connected discourse tracking task did not differ significantly from the performance of the other prelingually deaf patients. A significant improvement over time was found at the suprasegmental level for the combined group of Usher's and other patients. No obvious differences were found between the scores from the patients with a single-channel and the patients with a multichannel device. The rehabilitation of the Usher's patients required very little extra effort in comparison with that of the other prelingually deaf patients; all patients reported considerable advantages in hearing abilities and social life.