Large Vestibular Aquduct Syndrome (LVAS), which is characterized by fluctuating sensorineural deafness and vertigo in young children, was officially named a congenital genetic disorder in 1978 and is associated with autosomal recessive inheritance and does not combine with other inner ear abnormalities except for enlarged vestibular aqueducts. Hearing loss occurs after a fall or a cold, and then slowly improves, but after a few drops, complete hearing loss occurs, which means extreme deafness. Large vestibular aqueduct syndrome, early diagnosis and active prevention and treatment are important to prevent further hearing loss. If the hearing loss is fluctuating, CT of the temporal bone can confirm the diagnosis. The main manifestations are (1) pure tone hearing loss within 70 dB and hearing brainstem evoked potential threshold within 9O dB; (2) further hearing loss triggered by cold, exertion, head trauma, mental stimulation, symptoms such as vertigo, tinnitus, etc., but can be recovered or reduced within a short period of time (3-5 d); (3) no clear family history, no intellectual disability or mental retardation. (3) no clear family history, no mental retardation or other diseases involving hearing syndrome; (4) the overall condition is slowly changing (worsening or decreasing) and is not fixed. For the treatment of large vestibular aqueduct syndrome: when the hearing impairment is aggravated by obvious causes, active treatment with vasodilators, neurotrophic agents, dehydration and adrenal glucocorticoids can have some effect, and hearing can be restored to its original level. At the same time, hyperbaric oxygen therapy can increase the oxygen saturation and improve the microcirculation in the inner ear. For the surgical treatment of large vestibular aqueduct syndrome, some scholars have performed endolymphatic bursa decompression, shunt surgery or muscle flap filling, but the results are not satisfactory and there is a risk of total deafness. Hearing aids and cochlear implants have proven to be the only means of improving hearing in children with this condition. For children with severe hearing loss that cannot be effectively compensated by hearing aids, or those with progressive hearing loss, early cochlear implantation is the only option.