Congenital deafness is a common genetic disorder in otolaryngology, with an incidence of 1 case/1000-2000 births, mainly autosomal recessive, accounting for more than 75%, and non-genetic congenital deafness accounting for about 20%. The genetic deafness is divided into two major categories, nonsyndromic and syndromic, accounting for about 80% and 20% of the cases, respectively. More than 100 loci have been reported for nonsyndromic hereditary deafness, of which more than 40 genes have been identified, including DFNA for autosomal dominant, DFNB for autosomal recessive, and DFN for X-linked. Syndromic hereditary deafness is a concomitant symptom of some syndromes such as Alport syndrome, X-linked Charcot-Marie-Tooth, Goldenhar syndrome, Stickler syndrome, Waardenberg syndrome, and Usher syndrome. In addition, there is a group of deafness caused by mitochondrial disease. Non-genetic deafness can be caused by congenital infections, metabolic factors in pregnant women, etc. The treatment of congenital deafness depends on the type of congenital deafness, including surgical correction of middle ear malformation; hearing aid and speech rehabilitation for those with residual hearing; congenital sensorineural deafness is usually irreversible and there is no effective drug or surgical treatment; congenital deafness is caused by various factors during the fetal period, resulting in impaired development or damage to the auditory organs, and hearing impairment exists at birth. Causes: The causes of congenital deafness can be divided into three categories: genetic factors, pregnancy factors and maternal factors. Hereditary factors Hereditary deafness is deafness caused by abnormalities in genes and chromosomes. It accounts for about 35% of all childhood deafness. The main causes of congenital deafness in fetus during pregnancy are poisoning and infection. (1) Poisoning Pregnant women Any application of ototoxic drugs, such as aminoglycoside antibiotics (streptomycin, neomycin, gentamicin, kanamycin), quinine and aspirin, during any period, especially in the early and middle stages of conception, can cause inner ear toxicity and degenerative necrosis of the spiral organ, resulting in congenital deafness. (2) Maternal infection during pregnancy is a common cause of fetal deafness. It occurs mostly in the first trimester of pregnancy and is often irreversible sensorineural deafness. After the third month of pregnancy, deafness occurs less frequently because of the fully developed spiral apparatus. The main routes of fetal infection are vaginal upstream and placental bloodstream infections due to viraemia and the toxic effects of other substances that prevent the normal development of the inner ear. Common causes of infection during pregnancy are rubella, simple envelope rash, cellular mastitis virus infection, toxoplasmosis, and congenital syphilis. In addition, various toxic diseases during pregnancy, diabetes mellitus, nephritis, abdominal X-ray exposure, and pre-term abortion can affect the development of the fetal inner ear and lead to deafness. 3. Perinatal factors include lesions that occur during the perinatal period and the postpartum period. The perinatal period generally refers to the period from the 28th week of pregnancy to 7 days after birth. The common causes of deafness during this period include late pregnancy toxemia, birth injury during delivery, hypoxia caused by premature birth or obstructed labor, and neonatal hemolytic jaundice caused by Rh factor incompatibility. There are many causes of deafness in the postpartum period, such as various infections of the middle and inner ear, as well as various ototoxic drugs and trauma to the ear. Treatment Most congenitally deaf children have sensorineural deafness, which is very difficult to treat, therefore, prevention should be the main focus. Therefore, prevention should be the main focus. We should strongly advocate eugenics and prohibit marriage between close relatives; strengthen genetic counseling and popularize knowledge of ototoxic drugs. Early hearing screening of infants and children, if there is residual hearing, can be corrected by trial fitting of hearing aids, and early speech training of the affected children. For certain children with genetic deafness, symptomatic treatment can be provided according to the situation. For example, in Crouzen syndrome, early surgery can be performed to separate the healed skull sutures with polyethylene and silicone film. In Pendrea syndrome, thyroxine or iodine-containing foods can be given to help prevent thyroid enlargement and hearing deterioration. in Alport syndrome, kidney transplantation is possible. in certain patients with congenital conductive deafness, such as Treacher-Collins syndrome and Apert syndrome, tympanoplasty can be considered.