GSDME and deafness: To this day, NCSS mutations have been reported and functionally verified in only a handful of deafness-causative genes, including a c.1282-12T > A in EYA4 lead to non-syndromic deafness DFNA10 (Hildebrand et al., 2007), a c.6050-15G > A mutation in CDH23 lead to atypical USH1 syndrome (Valero et al., 2019), and a number of NCSS mutations involving splicing of exon 8 in DFNA5 (Booth et al., 2018).