In our study, there was high prevalence of endolymphatic hydrops in patients with genetic mutations of GJB2, SLC26A4, and LMX1A. In ears with GJB2 variants, 37.5% (3/8) had vestibular hydrops and 36.3% (4/7) had cochlear hydrops in our cohort. This evidence concerns the gene SLC26A4 and endolymphatic hydrops.