The occurrence of congenital deafness or childhood deafness is very common in China, and a large percentage of these patients have large vestibular canal syndrome. The occurrence of this disease brings a huge psychological shock and financial burden to the families of the affected children, and parents want to get the right help and guidance on its causes, prevention, treatment and future reproductive guidance. Large vestibular canal syndrome is a developmental malformation of the inner ear in which the canal linking the vestibule to the cranial cavity becomes abnormally enlarged, as if a triangular defect in the solid dam that separates a miniature pond from a giant reservoir, so that small pressure changes in the cranial cavity can be translated into an overwhelming impact in the small inner ear cavity, destroying the inner ear structure and causing hearing loss. This is how mild head trauma can lead to hearing loss in patients with large vestibular aqueduct. Many parents believe that they have never seen a similar case in their own family or in their own family and believe that the disease is caused by an external factor causing inner ear dysplasia, but in fact it is an autosomal recessive disease and both parents must be carriers of the SLC26A4 (PDS) gene mutation for one quarter of their children to develop the disease. Modern genetic diagnosis techniques can detect PDS mutations in about 95% of Chinese patients with large vestibular aqueduct. Patients with large vestibular aqueducts are born with hearing that can range from completely normal to very severe hearing loss, and most patients have good residual hearing, so it is important to preserve residual hearing for these patients to maintain their language development and learning ability. Due to their special anatomical characteristics, patients with large vestibules are extremely intolerant to head impacts, so it is important to avoid all sports and behaviors that may cause head trauma or increased intracranial pressure, such as bicycling, weight lifting, and confrontational sports. Because colds also often cause hearing loss in patients with large vestibular aqueducts, it is also very important to prevent and treat colds in children with the disease. For each significant hearing loss of the affected child, it is important to pay attention to it and to give treatment according to the principles of treatment of sudden deafness, which can be given to improve microcirculation, neurotrophic, vitamin B drugs for treatment, and in case of occasional, more serious sudden hearing loss, appropriate amount of cortisol hormone is given if necessary, and rest and braking should be paid attention to, through these treatments, the hearing of many patients can be restored to its original level.