Good speech recognition and quality-of-life scores after cochlear implantation in patients with DFNA9

Objective: To compare audiometric and quality-of-life results in DFNA9 patients who received a cochlear implant with cochlear implant patients with adult-onset progressive sensorineural hearing loss. Study Design: Prospective comparative design; results were collected cross-sectionally. Setting: Tertiary referral center. Patients: Eleven DFNA9 patients were included in the study as well as a comparative group of 39 postlingually deafened cochlear implant subjects with adult-onset progressive sensorineural hearing loss. Interventions: All patients received a cochlear implant. Subjects were implanted with either the Nucleus 24M/RCS or Med-el Combi 40+ cochlear implant systems implementing the SPEAK, ACE, or CIS+ coding strategies. Mean Outcome Measures: Speech recognition was determined by means of phonetically balanced monosyllabic word lists. The Hearing Handicap Inventory for Adults, the Glasgow Benefit Inventory, and the Scale for the Prediction of Hearing Disability in Sensorineural Hearing Loss were used to quantify the quality of life. Results: The results show that the speech perception and the quality of life of the DFNA9 patients do not differ significantly from the control group (p = 0.179; p = 0.56). Conclusion: In spite of the fact that DFNA9 is a disease that is known to involve cochlear dendrites, cochlear implantation is a good option for treatment of deafness in DFNA9.