RESULTS FROM 4 COCHLEAR IMPLANT PATIENTS WITH USHERS-SYNDROME

Individual results are presented of 4 patients with Usher's syndrome t ype 1 who received a cochlear implant. Both single-channel and multich annel implants were used. Because of implant failure, one of the singl e-channel systems was replaced by a Nucleus multichannel system. Resul ts are compared to the results of 5 other prelingually deaf cochlear i mplant users. The performance of the patients with Usher's syndrome on suprasegmental and segmental speech perception tests and on a connect ed discourse tracking task did not differ significantly from the perfo rmance of the other prelingually deaf patients. A significant improvem ent over time was found at the suprasegmental level for the combined g roup of Usher's and other patients. No obvious differences were found between the scores from the patients with a single-channel and the pat ients with a multichannel device. The rehabilitation of the Usher's pa tients required very little extra effort in comparison with that of th e other prelingually deaf patients; all patients reported considerable advantages in hearing abilities and social life.