We report a Han Chinese male, born to consanguineousparents, presenting with nonsyndromic sensorineural hearing loss, whose clinicalphenotype was also consistent with auditory neuropathy spectrum disorder (ANSD).After exome sequencing, a gap junction protein beta 2 gene(GJB2) c.235delC variant in the homozygous state wasdetected in the patient. Here, GJB2 is linked to auditory neuropathy.