In conclusion, we identified a novel variant c.2020G>T (p.Gly674Trp) in WFS1 responsible for autosomal dominant low-frequency sensorineural HL in a Chinese family, extending the variant spectrum of WFS1. Based on the variant location in the ER domain, we speculate that the variant might cause hearing impairment due to ER stress. The gene discussed is WFS1; the disease is Hearing impairment.