Auditory neuropathy(AN)is a disorder in which hearing loss is caused by dysfunction of the inner hair cells,the synapses of the auditory nerve,or the auditory nerve itself,accounting for 7％～10％of permanent hearing impairment in children.Existing studies suggest that genetic factors are the main causative factors in auditory neuropa-thy.There are many genes clearly associated with AN,among which the OTOF gene is the first gene identified and its pathogenic mechanism is relatively well studied.Gene therapy associated with the OTOF gene has been studied in ani-mal models and is expected to be applied to the treatment of auditory neuropathy in the near future.In this paper,we will review the literature and provide an outlook on OTOF mutation-related auditory neuropathies in terms of pathogenesis,audiological and clinical phenotype,and therapeutic advances.
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