Large vestibular aqueduct 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, without any combination of inner ear abnormalities other than enlargement of the vestibular aqueduct. 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, a CT of the temporal bone can confirm the diagnosis, and the main manifestations are: (1) pure tone hearing loss of 70 dB or less, and hearing brainstem evoked potential threshold of 9O dB or less; (2) further hearing loss due to cold, exertion, head trauma, mental stimulation, dizziness, tinnitus and other symptoms, but in a short period of time (3-5 days) can be recovered or alleviated; (3) no clear family history, no intellectual disability, and no family history. (3) no clear family history, no mental retardation and no other diseases related to hearing syndrome; (4) the overall condition is slowly changing (worsening or decreasing) and is not fixed. 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 the 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.