X-Linked Stapes Gusher: CT Findings in One Patient
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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.

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Imaging of inner ear malformations: a primer for radiologists

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Brothers with X-linked congenital hearing loss

Figure 2 from CT and MRI findings in X-linked progressive deafness.

Brothers with X-linked congenital hearing loss

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Systematic Review of Outcomes After Cochlear Implantation in Children With X -Linked Deafness-2 - Joshua D. Smith, Nour El-Kashlan, Owen A. F. Darr, Marc C. Thorne, 2021

PDF] CT and MRI findings in X-linked progressive deafness.
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