Objective: Investigate the prevalence and spectrum of GJB2 and SLC26A4 mutations in the patients with autosomal recessive nonsyndromic hearing loss in Zhejiang Methodology: A total of 176 unrelated pediatric patients with autosomal recessive nonsyndromic hearing loss were enrolled in this study.Genomic DNA was extracted from the peripheral blood sample.We employed polymerase chain reaction and sequenced the products to screen the mutations of GJB2.Meanwhile, we utilized SNaPshot sequencing method to detect four hotspot mutations of SLC26A4 (IVS7-2A＞G, c.2168A＞G) and GJB3 (c.538C＞T, c.547G＞A).All patients were performed temporal bone computer tomography to identify the enlarged vestibular aqueduct.