Cochlear implant outcomes in genetic sensorineural hearing loss

This data collection contains the pre- peri- en post-implantation data of genotyped cochlear implant (CI) recipients. Cochlear implants (CI) are an effective rehabilitation option for individuals with severe-to-profound sensorineural hearing loss (SNHL). While genetic factors play a significant role in SNHL, the variability in CI outcomes remains unclear. This data set was used to evaluate CI outcomes in a large, genotyped cohort, both at short- and long-term, and to correlate these outcomes with causative genes and their cochlear site-of-lesion. The dataset includes retrospective trans-sectional data of genotyped CI recipients, presenting the following variables: 1. Demographic data: gender, self-reported age of onset of SNHL, use of HA before implantation, vestibular function, and age at the time of implantation. 2. Genetic data: identified pathogenic variant(s) with the associated protein change(s and type of variant (truncating or missense). The identified genes are classified in different cochlear site-of lesions, including hair cells, stria vascularis, tectorial membrane, spiral ganglion neurons or cochlear nerve. 3. Implantation related data: type of implant and electrode used, pre-implantation imaging, and type of insertion. 4. Audiological measurements pre-implantation and outcome measurements post-implantation, including the pure tone average (PTA) aided and unaided, and phoneme scores measured at 65 dB SPL aided and unaided.