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.

X-linked deafness, Radiology Case

X-Linked Stapes Gusher: CT Findings in One Patient

Spectrum of Third Window Abnormalities: Semicircular Canal Dehiscence and Beyond. - Abstract - Europe PMC

Audiology Research, Free Full-Text

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A Case Series of X-Linked Deafness-2 with Sensorineural Hearing Loss, Stapes Fixation, and Perilymphatic Gusher: MR Imaging and Clinical Features of Hypothalamic Malformations

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X-linked deafness with stapes gusher in females - ScienceDirect

Hypothalamic hamartomas and inner ear diverticula with X-linked stapes gusher syndrome - new associations?

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Audiology Research, Free Full-Text