ABSTRACT
Large vestibular aqueduct syndrome [LVAS] is a radiologically diagnosed syndrome. Children with delayed speech and hearing loss without any middle ear disorder should arouse the suspicion of inner ear congenital anomaly. A computerized tomography [CT] scan is essential to rule out the possibility of LVAS in these children. Until recently no satisfactory treatment was available either to improve the hearing or to prevent further deterioration of hearing loss in this condition. The hearing loss in these two sisters began in childhood and progressed slowly. One of them also had a history of sudden diminution of hearing at one stage. Neither of them has had any vestibular symptoms. They were fitted with hearing aids and both the sisters are doing well in their education in normal school. Another two cases