Corrigendum to: NCOA3 identified as a new candidate to explain autosomal dominant progressive hearing loss.

Hearing loss is a frequent sensory impairment in humans and genetic factors account for an elevated fraction of the cases. We have investigated a large family of five generations, with 15 reported individuals presenting non-syndromic, sensorineural, bilateral and progressive hearing loss, segregating as an autosomal dominant condition. Linkage analysis, using SNP-array and selected microsatellites, identified a region of 13cM in chromosome 20 as the best candidate to harbour the causative mutation. After exome sequencing and filtering of variants, only one predicted deleterious variant in the NCOA3 gene (NM_181659, c.2810C>G; p.Ser937Cys) fit in with our linkage data. RT-PCR, immunostaining and in situ hybridization showed expression of ncoa3 in the inner ear of mice and zebrafish. We generated a stable homozygous zebrafish mutant line using the CRISPR/Cas9 system. ncoa3−/− did not display any major morphological abnormalities in the ear, however, anterior macular hair cells showed altered orientation. Surprisingly, chondrocytes forming the ear cartilage showed abnormal behaviour in ncoa3−/− , detaching from their location, invading the ear canal and blocking the cristae. Adult mutants displayed accumulation of denser material wrapping the otoliths of ncoa3−/− and increased bone mineral density. Altered zebrafish swimming behaviour corroborates a potential role of ncoa3 in hearing loss. In conclusion, we identified a potential candidate gene to explain hereditary hearing loss, and our functional analyses suggest subtle and abnormal skeletal behaviour as mechanisms involved in the pathogenesis of progressive sensory function impairment. ### Competing Interest Statement The authors have declared no competing interest. * AWERB : Animal Welfare and Ethical Review Body ADNSHL : Autosomal dominant non-syndromic hearing loss BSA : Bovine serum albumin CIOMS : Council for International Organizations of Medical Sciences CRISPR : Clustered regularly interspaced short palindromic repeats Cas9 : CRISPR associated protein 9 dpf : Days post fertilization ENT : Ear, nose and throat EMBL-EBI : European Bioinformatics Institute gnomAD : Genome Aggregation Database LOD : Multipoint logarithm of odds NHLBI-ESP : National Heart, Lung, and Blood Institute Exome Sequencing Project ABraOM : Online Archive of Brazilian Mutations PFA : Paraformaldehyde P : Postnatal Day Wpf : weeks post fertilization