If a drastic change in hearing has occurred in a child following a minor head trauma, change in barometric pressure, or physical exertion, large vestibular aqueduct syndrome (LVAS) should be considered. Most audiologists are unaware of LVAS or do not suspect it, in part due to the presence of a conductive component. LVAS can be seen in conjunction with Mondini's dysplasia or may appear by itself and is easily identified by a computed tomography scan. We present five cases of LVAS and discuss the natural history, audiologic and imaging findings, and relevant literature.