Speech Recognition and Communication Outcomes With Cochlear Implantation in Usher Syndrome Type 3

Background: Usher syndrome Type 3 (USH3) is an autosomal recessive disorder characterized by variable type and degree of progressive sensorineural hearing loss and retinitis pigmentosa. Cochlear implants are widely used among these patients. Objectives: To evaluate the results and benefits of cochlear implantation in patients with USH3. Study Design: A nationwide multicenter retrospective review. Materials and Methods: During the years 1995-2005, in 5 Finnish university hospitals, 19 patients with USH3 received a cochlear implant. Saliva samples were collected to verify the USH3 genotype. Patients answered to 3 questionnaires: Glasgow Benefit Inventory, Glasgow Health Status Inventory, and a self-made questionnaire. Audiological data were collected from patient records. Results: All the patients with USH3 in the study were homozygous for the Finnish major mutation (p.Y176X). Either they had severe sensorineural hearing loss or they were profoundly deaf. The mean preoperative hearing level (pure-tone average, 0.5-4 kHz) was 110 +/- 8 dB hearing loss (HL) and the mean aided hearing level was 58 +/- 11 dB HL. The postoperative hearing level (34 +/- 9 dB HL) and word recognition scores were significantly better than before surgery. According to the Glasgow Benefit Inventory scores and Glasgow Health Status Inventory data related to hearing, the cochlear implantation was beneficial to patients with USH3. Conclusion: Cochlear implantation is beneficial to patients with USH3, and patients learn to use the implant without assistance.