Large Vestibular Aqueduct Syndrome (LVAS) is characterized by fluctuating sensorineural deafness and vertigo in young children, and was officially named in 1978 as a congenital genetic disorder associated with autosomal recessive inheritance, which is not associated with any other malformations of the inner ear except for enlargement of the vestibular aqueduct. Colds and trauma are often the cause of the disease. Hearing loss occurs after a fall or a cold, and then slowly improves, but after a few falls, there is a complete loss of hearing, which means that extreme deafness occurs. Early diagnosis and active prevention and treatment of large vestibular aqueduct syndrome is important to prevent further hearing loss. If hearing loss is found to be fluctuating, CT of the temporal bone can confirm the diagnosis. When the diameter of the vestibular aqueduct is measured deep in the axial CT of the temporal bone and exceeds 1.5mm, the disease should be considered, and the diagnosis can be made by combining with the clinical manifestations. The main manifestations are: (1) pure tone hearing loss within 70 dB, listening to the brainstem evoked potential threshold within 90 dB; (2) due to colds, fatigue, head trauma, mental stimulation triggered by a further decline in hearing, vertigo, tinnitus and other symptoms, but in a short period of time (3-5 d) can be recovered or alleviated; (3) there is no clear family history of mental retardation and other diseases involved in hearing syndrome; (4) the overall condition of the disease can be diagnosed by the combination of clinical manifestations. (4) The overall condition is slowly changing (exacerbating or alleviating) and is not fixed. For the treatment of vestibular aqueduct syndrome: when the hearing disorder is aggravated by obvious triggers, active treatment with vasodilators, neurotrophic agents, dehydration and adrenal glucocorticoids can have a certain effect, and the hearing can be restored to the original level. At the same time, the use of hyperbaric oxygen therapy can improve the blood oxygen saturation, improve the inner ear microcirculation effect is better. For the surgical treatment of large vestibular aqueduct syndrome, some scholars have performed endolymphatic sac decompression, shunt surgery or myoflap tamponade, but the efficacy is not satisfactory, and there is a risk of total deafness. Hearing aids and cochlear implants have been proven to be the only means of improving hearing in children. For those who have severe hearing loss and cannot be compensated by hearing aids, or those with progressive hearing loss, cochlear implantation is the only way to go.