About 40% of ANSD is associated with genetic factors, such as autosomal recessive genes OTOF and PJVK (Varga et al., 2003; Delmaghani et al., 2006), autosomal dominant gene OPA1 (Amati-Bonneau et al., 2009), and X-chromosome recessive gene AIMF1 (Zong et al., 2015). This evidence concerns the gene PJVK and auditory neuropathy.