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Autosomal recessive non-syndromic sensorineural hearing loss (ARNSHL) represents a large portion of congenital hereditary HL. Our study was conducted on 13 patients from 13 unrelated families. The majority of patients presented with congenital severe to profound bilateral sensorineural HL. All patients were subjected to detailed family history and three-generation pedigree analysis to exclude any environmental cause and to ensure an autosomal recessive mode of inheritance. Molecular analysis was performed using the whole exome sequencing (WES) technique for the recruited patients. Three variants in the <jats:italic>MYO7A<\/jats:italic> and <jats:italic>OTOF<\/jats:italic> genes were reported for the first time in patients with ARNSHL (one nonsense, one frameshift, and one splice variant). Ten previously reported variants were detected in seven genes (<jats:italic>GJB2<\/jats:italic>, <jats:italic>MYO15A<\/jats:italic>, <jats:italic>BSND<\/jats:italic>, <jats:italic>OTOF<\/jats:italic>, <jats:italic>CDH23<\/jats:italic>, <jats:italic>SLC26A4<\/jats:italic>, and <jats:italic>TMIE<\/jats:italic>). They varied between missense, nonsense, frameshift, and splice variants. 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