SOX10 and Werner syndrome: According to a previous report, in the appropriate clinical context, bilateral agenesis or hypoplasia of the semicircular canals or both, associated with an enlarged vestibule and cochlear deformity, strongly suggests a diagnosis of WS linked to a SOX10 mutation, so we speculate that the spontaneous SOX10 c.246delC mutation may be the cause of the type II WS of the proband [9].