NIPBL and endolymphatic hydrops: Besides, 16 genes (ADGRV1, ARID1B, BICRA, CDK5RAP2, COL11A2, DMD, DMXL2, FLNB, LRP5, MEGF8, MYO7A, NIPBL, PCDH15, PRKDC, VCL, ZNF469) have been previously associated with SNHL in HPO, and 10 are located at cell–cell junctions according to CC GO, supporting the formation of endolymphatic hydrops observed in some MD patients [18, 19].