Large vestibular aqueduct syndrome (LVAS) is a common clinical hereditary hearing impairment. In recent years, reports of vestibular disorders of the disease have gradually increased, and the vestibular symptoms of the disease are known to be diverse. The development of new vestibular function examination techniques, such as vestibular evoked myogenic potential and video head pulse test, has made it possible to deeply understand the vestibular pathophysiological mechanism of LVAS in clinical practice. For example, vestibular-evoked myogenic potentials in patients with LVAS have the characteristics of low threshold and high amplitude, which is in line with the characteristics of the "third window of the inner ear"; stagnant water" disease. With the development of LVAS cochlear implant surgery, related vestibular symptoms and changes in vestibular function have also been reported. Therefore, reviewing LVAS-related vestibular assessments (symptoms and vestibular function tests) can help to explore its pathophysiological mechanism, and help to take into account the patient's vestibular function in addition to hearing when making clinical decisions, thereby improving the quality of life of patients.