title: Different cortical metabolic activation by visual stimuli possibly due to different time courses of hearing loss in patients with GJB2 and SLC26A4 mutations.
abstract: CONCLUSIONS
  We have demonstrated differences in cortical activation with language-related visual stimuli in patients who were profoundly deafened due to genetic mutations in GJB2 and SLC26A4. The differences in cortical processing patterns between these two cases may have been influenced by the differing clinical courses and pathogenesis of hearing loss due to genetic mutations. Our results suggest the importance of hearing during early childhood for the development of a normal cortical language network.
  
  
  OBJECTIVE
  To investigate the cortical activation with language-related visual stimuli in patients who were profoundly deafened due to genetic mutations in GJB2 and SLC26A4.
  
  
  METHODS
  The cortical activity of two adult patients with known genetic mutations (GJB2, SLC26A4) was evaluated with fluorodeoxyglucose-positron emission tomography (FDG-PET) with a visual language task and compared with that of normal-hearing controls.
  
  
  RESULTS
  A patient with a GJB2 mutation showed activation in the right auditory association area [BA21, BA22], and the left auditory association area [BA42] even with visual language task; in contrast, a patient with an SLC26A4 mutation showed no significant activation in the corresponding area.
authors: Department of Otorhinolaryngology, Shinshu University School of Medicine, Matsumoto, Japan.
authors: Hideaki
authors: H
authors: Moteki
authors: Yasushi
authors: Y
authors: Naito
authors: Keizo
authors: K
authors: Fujiwara
authors: Ryosuke
authors: R
authors: Kitoh
authors: Shin-ya
authors: SY
authors: Nishio
authors: Kazuhiro
authors: K
authors: Oguchi
authors: Yutaka
authors: Y
authors: Takumi
authors: Shin-ichi
authors: S
authors: Usami
mesh_terms: D000328:Adult; D002540:Cerebral Cortex / Q000000981:diagnostic imaging* / Q000378:metabolism; D000072259:Connexin 26; D017630:Connexins / Q000235:genetics*; D003638:Deafness / Q000000981:diagnostic imaging* / Q000235:genetics / Q000378:metabolism; D005260:Female; D019788:Fluorodeoxyglucose F18; D059907:Functional Neuroimaging; D005947:Glucose / Q000378:metabolism; D006801:Humans; D008297:Male; D026901:Membrane Transport Proteins / Q000235:genetics*; D049268:Positron-Emission Tomography; D000076988:Sulfate Transporters; D014796:Visual Perception; D055815:Young Adult
chemical_list: D017630:Connexins; C000607042:GJB2 protein, human; D026901:Membrane Transport Proteins; C085609:SLC26A4 protein, human; D000076988:Sulfate Transporters; D019788:Fluorodeoxyglucose F18; D000072259:Connexin 26; D005947:Glucose