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.

Imaging of inner ear malformations: a primer for radiologists

PDF] Facial nerve stimulation following cochlear implantation for X-linked stapes gusher syndrome leading to identification of a novel POU3F4 mutation.

Congenital X-linked Stapes Gusher Syndrome in an Infant

PDF) CT and MRI findings in X-linked progressive deafness

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

X-linked deafness/incomplete partition type 3: Radiological evaluation of temporal bone and intracranial findings. - Document - Gale Academic OneFile

Cerebrospinal Fluid Leak During Stapes Surgery: The Importance of Temporal Bone CT Reconstructions in Oblique Anatomically Oriented Planes - Massimo Re, Mirko Giannoni, Alfonso Scarpa, Claudia Cassandro, Massimo Ralli, Pietro De Luca

Brothers with X-linked congenital hearing loss

X-linked mixed hearing loss in a young boy with developmental delay and

New Imaging Findings of Incomplete Partition Type III Inner Ear Malformation and Literature Review

X-linked deafness with stapes gusher in females - ScienceDirect

PDF) X-Linked Stapes Gusher: CT Findings in One Patient

Figure 1 from CT and MRI findings in X-linked progressive deafness.

Audiology Research, Free Full-Text