In this study, we screened 9 cases of patients with ANSD who had normal results in distortion product otoacoustic emissions (DPOAE)/cochlear microphonic potentials (CM) but abnormal auditory brainstem responses (ABR); of which, we discovered 3 cases of OTOF gene mutation that were responsible for causing ANSD, and 6 cases of mutations in TBC1D24, LARS2, TIMM8A, MITF, and WFS1 genes. The gene discussed is OTOF; the disease is auditory neuropathy.