Large vestibular aqueduct syndrome (LVAS) is characterized by the enlargement of the vestibular aqueduct associated with sensorineural hearing loss (SNHL). The aim of this study was to report three cases of LVAS encountered at the Okmeydani Education and Research Hospital. Two of these three cases were siblings. Subjects included two males and one female, with an age range from 14 to 23 years. Siblings also presented a family history of deafness in their grandfather. The other case was a 23-year-old female who had been diagnosed with SNHL. Diagnosis, manifestations and treatment of LVAS are presented with literature data.