X-linked stapes gusher: CT findings in one patient

Abstract

A 31-year-old male patient presented with progressive mixed hearing loss since birth. A stapedectomy was attempted and was unsuccessful because of perilymph gushing. CT of the temporal bones showed bulbous dilatation of the fundi of the internal auditory canals and absence of the bone plates separating them from the base of the cochleas. This unusual abnormality was found after the attempted stapedectomy and explains the clinical findings. The findings in male patients are fairly typical X-linked congenital deafness.

Fig 1.

X-linked congenital deafness. A, Axial CT section (1 mm thick) of the right temporal bone shows a wide IAC. There is absence of bone (arrowhead) at the base of the cochlea allowing it to communicate with the IAC. The cochlea is dysplastic and the modiolus not well seen. B, On the same side, the labyrinthic portion (arrowhead) of the facial nerve is wide. C, Axial CT section of the left temporal bone also shows a wide IAC communicating (arrowhead) with the basal turn of the cochlea. The cochlea is dysplastic, and the modiolus not well seen.