Abstract
Background: Superior semicircular canal dehiscence (SSCD) is a rare bone defect in the petrous part of the temporal bone. In this syndrome, a third window is created between the middle cranial fossa and the bony labyrinth of the inner ear which can lead to stimulation of the vestibular system by sound. Patients usually have symptoms such as vertigo, imbalance, autophony, pulsatile tinnitus, and aural fullness. The clinical symptoms of this disease vary from person to person.
 The Case: This study reports a case of a rare bilateral SSCD in a 39-year-old woman with imbalance and autophony problems. The audiological findings showed a bilateral mild conductive hearing loss at low frequencies with symptoms of vertigo and torsional nystagmus. The SSCD was diagnosed by computed tomography. Then, the patient underwent bone repair surgery. Auditory and balance tests were performed again after the surgery to evaluate the extent of recovery.
 Conclusion: The SSCD can be diagnosed by using the mentioned test battery. By choosing the appropriate treatment methods and following up of the outcomes, most of the problems of patients with SSCD can be solved.
 Keywords: Superior semicircular canal dehiscence; bilateral; conductive hearing loss
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