X-Linked Stapes Gusher: CT Findings in One Patient

Summary: 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.

PDF] Cochlear Endoscopy in Cochlear Implantation of a X-Linked Stapes Gusher Syndrome

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Figure 4 from CT and MRI findings in X-linked progressive deafness.

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