Introduction: Superior Semicircular Canal Dehiscence Syndrome is a pathological entity that causes incapacitating auditory and vestibular symptoms. It is common that these patients are erroneously diagnosed with simulators or psychiatric diseases, or with any other vestibular pathologies.Etiology: It seems to have a genetic predisposition supported in a higher frequency of Semicircular Canal Dehiscence and bone defects in the tegmen tympani in patients with DFNA 9 (gen COCH). In other cases there is no specific cause justifying communication between the SSC and cranial cavity. Over the years, and the progressive thinning of the bone, the communication between membranous labyrinthine and cranial cavity, will occur.Pathophysiology: Superior Semicircular Canal Dehiscence Syndrome is characterized by thinning or loss of bone that covers the SSC and which separates it from of the cranial cavity. A pathologic third window on the vestibular side of the cochlea allows for a portion of acoustic energy to be shunted away from the cochlear partition, producing a decrease in the sound pressure within the vestibule, thus resulting in a loss of hearing sensitivity to air-conducted sound (conductive hearing loss) and increases the pressure difference between the two sides of the cochlear partition by lowering the impedance on the vestibule side, thereby improving the cochlear response to bone conduction (supraliminal levels). Diagnosis:Is based on the clinical history (auto phony, fluctuating hearing loss, acute or short-term vertigo). Typically patients complain of auto phony and feeling of being "in a box". The audio vestibular tests will confirm the clinical diagnosis: PTTA with GAP in low frequencies, VEMPs with threshold elevation in affected ear and CT with SSC dehiscence. Treatment:The initial treatment of this pathology should be conservative. If the symptoms are disabling it may raise a surgical solution that is the closure of the communication between the SSC and the floor of the middle fossa. The different types of surgical treatment are: access to the middle cranial fossa by temporal craniotomy; access to the SSC by upper Tran's mastoid access, and finally, strengthening of the oval and round windows membranes by endaural access.Case Report: Present four patients diagnosed and treated surgically in our hospital. Comment their post operatory evolution and the functional results. Conclusion:The SCD is a pathologic entity that must be present in the differential diagnosis of all patients with audio logical and vestibular symptoms. Their initial treatment should be conservative, but if the symptoms are disabling, surgery should be recommended since results are very good, compensating risks.