Importantly, the DFNA27 mutation (OMIM#612431) that changes the splicing acceptor site of exon 4 in the human REST gene prevents SRRM4-dependent alternative splicing of this exon and causes progressive hearing loss [29], further illustrating the requirement of SRRM4-mediated post-transcriptional regulation in REST inactivation for hair cell development and function. Here, SRRM4 is linked to hearing loss disorder.