Evaluating Cochlear Implant Outcomes in DFNA9 Subjects

This data collection contains the pre- peri- and post-implantation data of cochlear implant (CI) recipients with DFNA9. DFNA9 is a progressive type of non-syndromic sensorineural hearing loss (SNHL) inherited in an autosomal dominant manner, typically emerging in mid-life due to mono-allelic pathogenic variants in COCH (1). It often includes vestibular deterioration, emerging between the third and seventh decade, depending on the genotype. With this data set is was assessed whether the Fazekas score could account for the variability in cochlear implantation (CI) outcomes among individuals with DFNA9 and evaluated signal loss in the semicircular canals (SCCs) on magnetic resonance imaging (MRI) among individuals with DFNA9. It includes retrospective cross-sectional data of CI recipients with DFNA9, presenting the following variables: 1. Demographic data: gender, self-reported age of onset of hearing loss, use of hearing aids before implantation, age at time of implantation 2. Genetic data: identified pathogenic variant with the associated protein change(s). 3. Cardiovascular risk factors: hypertension, diabetes mellitus (DM), elevated cholesterol levels, current or former smoking habits, the use of anticoagulant drugs, and a history of myocardial infarction and/or stroke. 4. The American Society of Anaesthesiologists (ASA) score 5. 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. 6. MRI findings: Fazekas score, additional brain or cochleovestibular abnormalities, and abnormalities at the level of the semicircular canals of the vestibular organ. 7. Vestibular function.