The GJB3 gene is the first indigenous deafness-related gene localized and cloned by our academician Jiahui Xia in two large lines of autosomal dominant non-syndromic deafness in China, and its missense mutation 538C>T is thought to be associated with postlingual high-frequency hearing loss, following an autosomal dominant inheritance pattern. Since the GJB3 mutation is carried at a low rate (less than 3%) in the Chinese population, and its genotype is not closely related to the phenotype, and even several pure mutation carriers have been found to have normal hearing in clinical practice, it is considered that this mutation may not be the sole cause of deafness. The detection of GJB3 gene 538C>T heterozygous or pure mutation in neonatal deafness screening suggests that the child carries the GJB3 mutation, but since the GJB3 genotype is not closely related to the phenotype, the child may not necessarily develop deafness, and if the child passes the hearing screening, the child’s hearing and speech development can be closely observed because some carriers of the GJB3 mutation are more closely related to late-onset deafness. It is recommended that the newborn be reviewed regularly. It is recommended that the newborns should have their hearing checked regularly and that they should avoid environmental factors such as noise, ototoxic drugs, head trauma, colds and fever. If there is a hearing loss, it should be treated promptly.