title: Neurotologic and functional MRI findings in a patient with bilateral profound deafness having Brown-Vialetto-Van Leare syndrome.
abstract: BACKGROUND
  We report the first findings of functional magnetic resonance imaging of the auditory cortex in a young woman with a bilateral cochleovestibular deficit as first manifestation of Brown-Vialetto-Van Leare syndrome. The patient had no open speech discrimination, even with hearing aids, and is depending on lip reading for communication.
  
  
  METHODS
  To evaluate the possible efficiency of a cochlear implantation, we investigated hemodynamic responses within the central auditory pathways using an auditory functional magnetic resonance imaging paradigm.
  
  
  RESULTS
  Blood oxygen level-dependent correlates were detected bilaterally along the auditory pathways after exposure to intermittent clicking tone stimulation at 2 kHz.
  
  
  CONCLUSIONS
  These results suggest integrity of the central auditory pathways and represent a positive argument to propose a cochlear implantation with the aim to restore hearing.
authors.affiliation: *Department of Otorhinolaryngology, Head and Neck Surgery; †Department of Diagnostic and Interventional Neuroradiology; and ‡Department of Psychiatric Neurophysiology, University Hospital of Psychiatry, Inselspital Bern, Switzerland.
authors.forename: Cinzia
authors.initials: C
authors.lastname: Salmina
authors.forename: Franca
authors.initials: F
authors.lastname: Wagner
authors.forename: Roland
authors.initials: R
authors.lastname: Wiest
authors.forename: Andrea
authors.initials: A
authors.lastname: Federspiel
authors.forename: Pascal
authors.initials: P
authors.lastname: Senn
authors.forename: Marco
authors.initials: M
authors.lastname: Caversaccio
authors.forename: Dominique
authors.initials: D
authors.lastname: Vibert
mesh_terms: D000328:Adult; D001303:Auditory Cortex / Q000503:physiopathology*; D001306:Auditory Pathways / Q000503:physiopathology; D010244:Bulbar Palsy, Progressive / Q000175:diagnosis* / Q000503:physiopathology*; D019929:Cochlear Implantation / Q000379:methods; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis* / Q000503:physiopathology*; D006320:Hearing Tests; D006801:Humans; D008279:Magnetic Resonance Imaging; D013067:Speech Perception