Large vestibular aqueduct syndrome (LVAS) is a recessive, non-syndromic hearing disorder that presents with fluctuating hearing loss after birth. The main patient group is children and adolescents during their growth and development. The pathogenesis of the disease is complex, and there is a lack of specific and effective methods for early diagnosis and treatment, and no clear explanation for its pathogenesis is available at this time. Possible factors include: ① Family history. ② Infection during pregnancy. (3) Ototoxic drugs, etc. Clinical manifestations】 LVAS mostly develops in early childhood, mainly manifests as bilateral asymmetric hearing loss, and the hearing loss is progressive and fluctuating, and its fluctuation is often related to head trauma and cold, which may or may not be accompanied by vertigo. In addition to history, clinical symptoms and audiological examination, the diagnosis of LVAS mainly relies on high-resolution CT and MRI scans of the temporal bone. (1) People with residual hearing should wear hearing aids to maintain good hearing. When there are fluctuating changes in hearing, it is important to treat them actively and adjust the output power of hearing aids in time. During treatment, it is recommended that hearing aids not be used for the time being. However, because the disease can deteriorate at any time, such patients should always be prepared for cochlear implantation (2) For patients with LVAS who are extremely deaf, cochlear implantation should be considered when hearing aids are ineffective. Prevention】 After LVAS is diagnosed, the following should be taken into consideration: ② Check your hearing regularly, develop good hearing follow-up habits, and seek medical attention promptly when hearing problems arise. ② Avoid trauma to the head, even if it is a minor bump. ③ Do not participate in strenuous sports, such as roller coaster, bungee jumping, etc. ④ Do not use ototoxic drugs. ⑤ Avoid exposure to strong noise. ⑥ Prevent colds, pay attention to the child’s emotional control, and reduce stressful actions such as blowing the nose.