X-linked stapes gusher: CT findings in one patient.
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.
Kumar
Gaurav
G
Department of Radiology, University of North Carolina School of Medicine, Chapel Hill, NC 27599-7510, USA..
Castillo
Mauricio
M
Buchman
Craig A
CA
D000328:Adult; D041321:Chromosomes, Human, X; D003051:Cochlea / Q000002:abnormalities* / Q000000981:diagnostic imaging; D003638:Deafness / Q000000981:diagnostic imaging / Q000235:genetics*; D007758:Ear, Inner / Q000002:abnormalities* / Q000000981:diagnostic imaging; D006311:Hearing Disorders / Q000000981:diagnostic imaging* / Q000235:genetics*; D034381:Hearing Loss; D006320:Hearing Tests; D006801:Humans; D007431:Intraoperative Complications / Q000000981:diagnostic imaging*; D008297:Male; D010498:Perilymph; D012729:Sex Chromosome Aberrations; D013201:Stapes Surgery; D013577:Syndrome; D014057:Tomography, X-Ray Computed