Abstract: Objective: To explore the diagnosis, hearing and treatment of large vestibular aqueduct syndrome（LVAS）. Method: A retrospective analysis was conducted on the history, auditory and vestibular function，imaging examination, and treatment in 9 cases from 2000 to 2005. Results: All 9 cases presented bilateral large vestibular aqueduct syndrome, and showed progressive hearing loss. Although sensorineural hearing loss was predominant, conductive hearing loss existed in lowtone frequencies in 8 cases. Longterm results of conservative treatment were not ideal. Conclusions: Hearing loss in children with uncertain reasons should be excluded from congenital diseases. Other factors combined can increase difficulties in diagnosis of LVAS. Hearingaids should be used in patients with progressive hearing loss as early as possible and cochlear implantation should be considered.

Key words: Diagnosis, Cochlear implantation , Large vestibular aqueduct syndrome