PJVK and auditory neuropathy: Interestingly, of 118 deafness genes, several genes have been reported to be associated with auditory neuropathy, a hearing dysfunction characterized by impaired transmission of signal to the auditory nerve by the presynaptic inner hair cells (Starr et al. 1996), such as PJVK (Delmaghani et al. 2006), NARS2 (Simon et al. 2015), SLC17A8 (Ruel et al. 2008), DIAPH3 (Schoen et al. 2010) and DIAPH1 (Wu et al. 2020).