Large Vestibular Aquduct Syndrome (LVAS) was discovered in the late 1970s with the introduction of CT and is characterized by fluctuating sensorineural deafness and vertigo in young children. It is not combined with other inner ear malformations except for enlarged vestibular canals. The onset of the disease usually begins around 2 years of age and is characterized by fluctuating hearing loss, with some patients showing sudden deafness and others showing episodes of vertigo with fluctuating hearing loss, similar to Meniere’s disease. The hearing loss is often triggered by colds, exertion, head trauma, and mental stimulation, which eventually leads to a gradual decline in hearing and can result in severe or profound deafness. First, the loss of the normal flow restriction of the vestibular aqueduct makes it easy for the pressure changes of the cerebrospinal fluid to cause pressure fluctuations in the vestibular fluid, especially stimulating the vestibular receptors and causing vertigo. The presence of the vestibular aqueduct not only limits the flow between the ectolymphatic fluid and the cerebrospinal fluid, but also allows the pressure of the ectolymphatic fluid in the vagus to be less affected by fluctuations in cerebrospinal fluid pressure. The size of the vestibular aqueduct is very important, and if it is too wide, it may lose this flow limiting effect. The change of cerebrospinal fluid pressure directly causes the change of pressure in the vagus, forming the flow of endolymphatic fluid and the stimulation of vestibular receptors, which eventually causes the vertigo symptoms. Secondly, the endolymphatic fluid of the membranous endolymphatic canal has unidirectional mobility so that it can ensure the internal environment for the metabolism of the auditory hair cells in the inner ear. If the vestibular aqueduct is too wide causing the endolymphatic canal to be correspondingly wide, it makes the unidirectional flow of endolymph ineffective and leads to the reflux of endolymph, thus the mixing of internal and external lymph in the membranous vagus and the poisoning of auditory receptors lead to the patient’s result of sensorineural deafness. Treatment: It is proven that hearing aids and cochlear implants are the only means to improve the hearing of the affected child. And cochlear implantation is considered as an option only for those who have extremely severe hearing loss and whose hearing aids cannot achieve effective compensation, or those with progressive hearing loss to very severe deafness.