Superior semicircular canal dehiscence associated with external, middle, and inner ear abnormalities

We report a case of bilateral superior semicircular canal dehiscence (SSCD) in a 38-year-old man who presented with congenital hearing loss without vestibular symptoms. This case is unusual due to the association of SSCD with multiple congenital anomalies of the external, middle, and inner ears, but without any other craniofacial or systemic developmental anomalies. Findings of multiple malformations of temporal bone structures in this case lend support to the theory that SSCD may have an underlying developmental or congenital etiology.