Large vestibular aqueduct syndrome, also known as congenital enlargement of the vestibular aqueduct, is an autosomal recessive disorder characterized by progressive or fluctuating hearing loss and sudden deafness. The disease can begin at any time from birth through adolescence. It is characterized by progressive or fluctuating hearing loss, sudden deafness in some patients, and episodic vertigo with fluctuating hearing loss in others. Auxiliary tests are mainly CT, inner ear MRI and other tests, there is no effective treatment, once diagnosed with the disease, we must do everything possible to prevent sudden hearing loss of the affected ear, such as avoiding head trauma, avoiding participation in competitive sports, avoiding excessive emotional excitement, and so on. When sudden deafness or episodes of vertigo occur, you should go to the ENT department of the hospital as soon as possible for professional consultation and treatment. If the above symptoms occur, it is recommended to go to a specialized hospital as soon as possible for a professional examination, a clear diagnosis and then under the guidance of the doctor for the next step in the treatment.