Hereditary deafness is divided into syndromic hearing loss and non-syndromic hearing loss according to whether it is accompanied by other system dysfunction. The early identification and diagnosis of syndromic hearing loss is very important, including clinical and molecular diagnosis. Early diagnosis can predict the progress of hearing loss, other systemic disorders and guide treatment. Thus otolaryngologists are likely to become the first doctors to treat children with syndromic hearing loss, it is more necessary to master the clinical and molecular diagnosis methods of common syndromic hearing loss, and cooperate with doctors of other relevant departments for early intervention and treatment. Therefore, this article reviewed the common features, molecular diagnostic methods and treatment strategies for syndromic hearing loss.