Introduction: Superior Semicircular Canal Dehiscence Syndrome is a rare disease, mainly characterized by auditory and vestibular symptoms induced by intense sound stimuli or by changes in intracranial or middle ear pressure, due to a dehiscence of the bony layer that covers the superior semicircular canal.

Objective: To perform a literature review about this syndrome, highlighting its main clinical characteristics, diagnosis and therapeutic options.

Data Synthesis: The prevalence of this condition is 0.7% in the general population and its etiopathogeny is still unknown. However, it is believed that the defect could occur during the development of the bony layer that covers the semicircular canal, followed by a head injury or a sudden increase in intracranial pressure, leading to the rupture of this abnormally thin layer. The clinical exam is extremely important, since tests considered standard for the vestibular apparatus investigation (electronystagmography and rotatory chair) may fail to show significant alterations. The diagnosis is confirmed through high-resolution temporal bone computed tomography scan. For patients who suffer with incapacitating vestibular symptom, surgical repair of the dehiscence can be very beneficial. The two most used techniques are total plugging of the superior canal and the reconstruction of the bony layer over the canal using fascia and cortical bone.

Conclusion: Despite being a rare condition this syndrome should be included among causes of vertigo and also be part of the differential diagnosis list of conductive hearing loss, especially in cases of preserved acoustic reflexes.