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.

Genes, Free Full-Text

X-linked deafness with stapes gusher in females - ScienceDirect

Audiology Research, Free Full-Text

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

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

Congenital X-linked Stapes Gusher Syndrome in an Infant

X-linked deafness with stapes gusher in females - ScienceDirect

Imaging of inner ear malformations: a primer for radiologists

Enlarged Cochlear Aperture as a Risk Factor for Cerebrospinal Fluid Gusher During Cochlear Implant Surgery - Kevin Wong, Zachary G. Schwam, Vivian Z. Kaul, George B. Wanna, 2022