CT and MRI findings in X-linked progressive deafness


ALTAY H., SAVAŞ R. , Oeguet F., KİRAZLI T. , ALPER H.

DIAGNOSTIC AND INTERVENTIONAL RADIOLOGY, vol.14, no.3, pp.117-119, 2008 (Journal Indexed in SCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 14 Issue: 3
  • Publication Date: 2008
  • Title of Journal : DIAGNOSTIC AND INTERVENTIONAL RADIOLOGY
  • Page Numbers: pp.117-119

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.