title: CT and MRI findings in X-linked progressive deafness.
abstract: Congenital X-linked mixed deafness is a rare anomaly that has typical features and can be diagnosed on the basis of progressive mixed hearing loss and the typical imaging findings. Recognition of these findings may alter the course of treatment and perilymph gushing can be avoided. A 10-year-old male patient presented with a history of progressive hearing loss. Computed tomography of the temporal bones showed bulbous dilatation of the fundi of the internal auditory canals (IAC) and the absence of the bony plates separating the basal turn of the cochleas and IAC. Magnetic resonance imaging demonstrated obliteration of the labyrinthine spaces at the right side.
authors.affiliation: Department of Radiology, Ege University School of Medicine, Izmir, Turkey. hakanaltay@lycos.com
authors.forename: Hakan
authors.initials: H
authors.lastname: Altay
authors.forename: Recep
authors.initials: R
authors.lastname: Savaş
authors.forename: Fatih
authors.initials: F
authors.lastname: Oğüt
authors.forename: Tayfun
authors.initials: T
authors.lastname: Kirazli
authors.forename: Hüdaver
authors.initials: H
authors.lastname: Alper
mesh_terms: D002648:Child; D041321:Chromosomes, Human, X; D003051:Cochlea / Q000002:abnormalities / Q000000981:diagnostic imaging; D003638:Deafness / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000235:genetics*; D007758:Ear, Inner / Q000002:abnormalities / Q000000981:diagnostic imaging; D050172:Genes, X-Linked; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D013701:Temporal Bone / Q000002:abnormalities* / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed / Q000379:methods*