Superior semicircular canal dehiscence and an abnormally wide internal auditory meatus are clinical entities characterized by vestibular and cochlear symptoms. These symptoms are induced by hypersensitivity of labyrinthine receptors secondary to a bone defect of the otic capsule. We report the case of a 41-year-old man with congenital right-sided hearing loss who presented with bilateral superior semicircular canal dehiscence that was associated with wide, bulbous internal auditory meatus and a loss of the bony wall separating the lateral end of the meatus from the cochlea. The patient was experiencing vestibular and cochlear symptoms in the right ear and disabling tinnitus in the left ear. However, he refused all treatment and was lost to follow-up.