[abstract] Objective:To explore and analyze the clinical characteristics of children's binaural sensory nerve deafness and the mutations of connexin 26(GJB2)and connexin 31(GJB3).Methods: In this study,150 children with binaural sensorineural hearing loss who were treated in our hospital's health checkup and ENT clinic from March 2020 to March 2021 were selected as the research objects, and 150 patients with normal hearing who participated in the physical examination during the same period were selected. Of children served as a control group. The kit method was used to extract genomic DNA from blood leukocytes, and the GJB2 and GJB3 genes are detected by polymerase chain reaction (PCR)in the coding region. Two groups of children GJB2 and GJB3 gene test results, the degree of deafness in children with binaural sensorineural hearing loss, the mutation rate of GJB2 and GJB3 genes in children with different degrees of binaural sensorineural hearing loss, and the mutation rate of GJB2 and GJB3 genes in children with binaural sensorineural hearing loss.GJB2 and GJB3 deafness gene mutations in parents' deafness were observed. Results: There were 5 pathogenic mutations in GJB2 gene(35insG,95G>A,176-191del16,235delC,257C>G).There were 50 cases of GJB2 gene mutation(33.33%),42 cases were related to 235delC mutation, accounting for 84.00%,and 3 kinds of polymorphic gene changes(79G>A,341A>G,427C>T).There were 6 cases of GJB3 gene mutation(538C→T,547G→A).A total of 3 children in the control group had GJB2 gene mutations(2.00%).There were two pathogenic mutations(95G>A,235delC),and two polymorphic gene changes(79G>A,341A>G).No GJB3 gene was found mutation. The mutation rate of 235delC in children in the study group was significantly higher than that in the control group(P<0.05);moderate to severe cases of binaural sensorineural hearing loss accounted for 59.33%(89/150);The mutation rates of GJB2 and GJB3 deafness genes in children with different degrees of binaural sensorineural hearing loss were significantly higher, which were 22.50%and 7.5%,respectively;Among 150 parents of children with binaural sensorineural hearing loss,17 cases(5.67%)of the father's GJB2 gene mutation were found, and 25 cases(8.33%)of the mother's gene mutation; There were 3 cases of GJB3 gene mutation in fathers(1.00%)and 1 case in mothers(0.33%);neither of the parents had detected 2 homozygous mutations, coincident hybrid mutations and GJB3 gene mutations. Conclusion: There is currently no clinically effective treatment for binaural sensorineural hearing loss. Therefore, reasonable and effective prevention is extremely important. For children with binaural sensorineural hearing loss, GJB2 and GJB3 gene mutation detection can be used as a treatment and prevention for children. Important guidance methods help reduce the incidence of deafness.