OTOF and auditory neuropathy: The OTOF gene is reported to be the causative gene for non-syndromic recessive sensorineural HL (DFNB9) [3] and is also reported as a major causative gene for auditory neuropathy spectrum disorder (ANSD) [4], which is characterized by an abnormal auditory brainstem response (ABR) or auditory steady state response (ASSR) with the normal appearance of otoacoustic emissions (OAE) [5].