The large vestibular aqueduct syndrome (LVAS) is characterized by the presence of a enlarged vestibular aqueduct, the most common form of inner ear abnormality, and the association with a sensorineural hearing loss. It was first named by Valvassori and Clemis in 1978, when they described radiographic findings in congenitally malformed inner ears. The clinical picture is usually variable. Hearing loss ranges from profound to mild, varying from fluctuating to stepwise progressive or sudden. Vestibular disturbances may be associated. The purpose of this study is report three cases of LVAS and review the literature. Two patients were female and one male. The age varied form 3 to 32 years. Vertigo was referred in only one patient, just like the association of inner ear abnormality. Management of the sensorineural hearing loss that accompanies the LVAS has been frustrating since the syndrome was first described. Conservative management has been advocated and includes avoidance of head or barotrauma and amplification as necessary. However, despite conservative measures, a large portion of affected patients continue to experience progressive hearing loss. Cochlear implantation has proven to be effective in increasing both auditory perception and speech production in children with congenital and acquired deafness from diverse causes. All the three cases reported in our study, besides the conservative management, have been followed by the cochlea implantation group of our department for a opportune surgical procedure.