An intronic sequence alteration causing gain-of-function of REST in hair cells leads to autosomal dominant deafness 27 (DFNA27) (Nakano et al. 2018) while inactivating mutations lead to other forms of non-syndromic progressive autosomal dominant hearing loss (Manyisa et al. 2021) and Jones syndrome, a dominant syndrome characterized by gingival fibromatosis and progressive sensorineural hearing loss (Rahikkala et al. 2023). This evidence concerns the gene REST and Progressive sensorineural hearing impairment.