Abstract

We report a case of a patient with superior semicircular canal dehiscence syndrome, a recently described condition in which vestibular imbalance and/or hearing loss results from the discontinuity of the bone overlying the superior semicircular canals. Case report. A 46-years-old women presented with autophony in the left ear and imbalance when shouting (Tullio phenomenon). Temporal bone computer tomography revealed a defect of the left superior semicircular canal caused by an enlarged superior petrosal sinus receiving drainage from a large cerebellar developmental venous anomaly. Conclusion. We review superior semicircular canal dehiscence syndrome and its management, and we discuss common aetiologies. We conclude that superior semicircular canal dehiscence syndrome may present with a solely developmental aetiology, despite presenting late in life.

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