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 DFNA 9 patients who received a cochlear implant with cochlear implant patients with adult-onset progressive sensorineural hearing loss.

Methods: Prospective comparative design; results were collected cross-sectionally. Methods: Tertiary referral center. Methods: Eleven DFNA 9 patients were included in the study as well as a comparative group of 39 post-lingually deafened cochlear implant subjects with adult-onset progressive sensorineural hearing loss. Methods: All patients received a cochlear implant. Subjects were implanted with either the Nucleus 24 M/RCS or Med-el Combi 40+ cochlear implant systems implementing the SPEAK, ACE, or CIS+ coding strategies. Methods: 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 DFNA 9 patients do not differ significantly from the control group (p=0.179; p=0.56).

Conclusions: In spite of the fact that DFNA 9 is a disease that is known to involve cochlear dendrites, cochlear implantation is a good option for treatment of deafness in DFNA 9.