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.

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Audiology Research, Free Full-Text

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

Brothers with X-linked congenital hearing loss

Brothers with X-linked congenital hearing loss

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Brothers with X-linked congenital hearing loss

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Audiology Research, Free Full-Text

Imaging and Anatomy for Cochlear Implants

Cochlear dysplasia (modiolus deficiency) on the left from X-linked