As we all know, hearing and vision together play a vital role in people’s daily life as the two most important sensory channels in the human body. The number of people who are deaf or hard of hearing is huge in China, with the latest national census showing that there are 20 million of them, which is the highest percentage of all congenital disabilities. Studies show that more than half of all deafness is hereditary and can affect more than one in a thousand newborns. In addition, acquired deafness disorders such as drug-related deafness and age-related deafness are also closely linked to genetic factors. There are four main types of inherited deafness: recessive, dominant, companion chromosomal and mitochondrial maternal inheritance. Autosomal recessive inheritance is the most common form of inheritance, accounting for about 80% of inherited deafness. This mode of inheritance is characterized by the fact that both parents can have normal hearing, but each carries a single recessive gene mutation (carrier). In their offspring children who acquire the two genetic mutations carried by their parents through inheritance are at high risk of developing genetic deafness as a result. In China, more than 60% of hereditary deafness is caused by mutations in the GJB2 or SLC26A4 genes, and the vast majority of mutations in these two common deafness genes are inherited in a recessive manner. In general, patients with recessive deafness tend to be born with hearing impairment and have severe hearing loss, most often in severe or profound deafness. Autosomal dominant deafness accounts for about 15% of inherited deafness. In contrast to recessive inherited deafness, dominant inherited deafness is characterized by patients who carry a single dominant gene mutation that can lead to deafness. One of the patient’s parents and more recent grandparents also tend to have deafness and have a significant family history of the disease. The majority of dominantly inherited deafness is mild, often with fair hearing at birth, but gradually deteriorating with age later in life. Companion chromosomal inheritance and mitochondrial maternal inheritance are relatively low, accounting for only 2-3% of inherited deafness. However, the mitochondrial mutation A1555G is relatively more prevalent in the Chinese population compared to other countries and ethnic groups. This mutation can cause patients to be sensitive to aminoglycoside antibiotics and can easily lead to drug-related deafness. Therefore, people with mitochondrial mutation A1555G should avoid exposure to aminoglycoside antibiotics such as gentamicin and streptomycin as much as possible, especially for newborns who are more susceptible to diseases that often require antibiotic drugs. At present, the genetic deafness diagnosis service has been officially launched in China, including Xinhua Hospital of Shanghai Jiaotong University Medical College and PLA General Hospital, and the above mentioned GJB2, SLC26A4 and mitochondrial mutations are included in the test. Genetic deafness diagnosis helps to understand the type and composition of genetic mutations carried by patients and their family members, and is of particular interest to the following three groups of people. We recommend that these individuals be considered for screening for common deafness mutations prior to formal childbearing or marriage to reduce the likelihood of hereditary deafness in their offspring. If the test results prove that both partners carry the mutation that can cause hereditary deafness, prenatal amniocentesis may be considered to reduce the chances of birth defects. In this sense, genetic diagnosis of deafness can effectively advance the prevention and treatment of deafness to the prenatal or preconception period, thus promising to reduce the pain and burden caused by hereditary deafness for society, families and individuals. The second category is newborns with a family history background of hereditary deafness. However, in a significant percentage of patients with hereditary deafness, hearing loss may be delayed, gradual or sudden. If overlooked, these disorders may result in delayed or impaired speech and language development, which may affect the child’s future learning and expression. Genetic diagnosis can be an effective adjunct to hearing screening to help confirm or predict these potential early hearing disorders, thus ensuring that this group of newborns is deaf but not mute. The third group is patients with drug-related deafness who carry the mitochondrial gene A1555G mutation and are sensitive to aminoglycoside antibiotics. If the mother or maternal family has a history of drug-related deafness, we recommend that this group be tested for mitochondrial gene mutations, and if deafness-causing mutations are identified, a range of aminoglycoside antibiotics that may be associated with drug-related deafness should be avoided. In conclusion, genetic counseling for deafness and preconception and prenatal intervention can effectively reduce the incidence of congenital deafness and is a very meaningful and promising emerging medical service that deserves to be promoted in more areas and units across the country where it is available.