Otoferlin (OTOF) gene located on chromosome 2p23.3 (48 exons), is associated with nonsyndromic autosomal recessive deafness, DFNB9.7 It is mainly involved in the fusion of synaptic vesicles with plasma membrane of the inner hair cells of cochlea and release of neurotransmitter at the ribbon synapse,8 participating in conduction of sound signals to the brain. Here, OTOF is linked to hearing loss, autosomal recessive.