title: Connexin-related (DFNB1) hearing loss: is routine computed tomography imaging necessary?
abstract: OBJECTIVE
  Determine if routine computed tomography (CT) imaging is necessary in the workup for children with connexin-related (DFNB1) sensorineural hearing loss (SNHL).
  
  
  METHODS
  Case-control retrospective chart and imaging review.
  
  
  METHODS
  Tertiary care otolaryngology practice.
  
  
  METHODS
  High-resolution temporal bone CT scans of children (n = 21) with DFNB1 SNHL were compared to age-matched controls with either conductive hearing loss (CHL, n = 33) or a nonsyndromic, non-DFNB1 SNHL (n = 33). Sixteen measurements of cochleo-vestibular structures were recorded. Statistical analysis was performed using a repeated analysis of variance model that controlled for both age and gender. Area under the curve (AUC) and multidimensional AUC (MAUC) analyses were also performed.
  
  
  RESULTS
  Overall, no statistically significant differences were found between the 3 experimental groups. In addition, comparisons between the DFNB1 and CHL groups, DFNB1 and non-DFNB1 SNHL groups, and CHL and non-DFNB1 SNHL groups failed to demonstrate any statistically significant differences. AUC and MAUC analyses also failed to detect any significant differences between the 3 groups.
  
  
  CONCLUSIONS
  Patients with DFNB1 SNHL do not have significant anatomic differences on temporal bone CT scans when compared to non-DFNB1 SNHL and CHL control groups. Based on the above analysis, it is reasonable to avoid routine CT imaging of the temporal bones in children with known DFNB1 SNHL unless otherwise clinically indicated.
authors.affiliation: Department of Otolaryngology/Head and Neck Surgery, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.
authors.forename: John P
authors.initials: JP
authors.lastname: Dahl
authors.affiliation: Department of Otolaryngology/Head and Neck Surgery, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.
authors.forename: Michael E
authors.initials: ME
authors.lastname: Stadler
authors.affiliation: Department of Radiology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.
authors.forename: Benjamin Y
authors.initials: BY
authors.lastname: Huang
authors.affiliation: Department of Biostatistics, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.
authors.forename: Di
authors.initials: D
authors.lastname: Miao
authors.affiliation: Department of Otolaryngology/Head and Neck Surgery, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.
authors.forename: Mihir R
authors.initials: MR
authors.lastname: Patel
authors.affiliation: Department of Otolaryngology/Head and Neck Surgery, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.
authors.forename: Oliver F
authors.initials: OF
authors.lastname: Adunka
authors.affiliation: Department of Otolaryngology/Head and Neck Surgery, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.
authors.forename: Craig A
authors.initials: CA
authors.lastname: Buchman
authors.affiliation: Department of Biostatistics, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.
authors.forename: Jason P
authors.initials: JP
authors.lastname: Fine
authors.affiliation: Department of Otolaryngology/Head and Neck Surgery, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA zdanski@med.unc.edu.
authors.forename: Carlton J
authors.initials: CJ
authors.lastname: Zdanski
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D000072259:Connexin 26; D017630:Connexins / Q000235:genetics*; D004252:DNA Mutational Analysis; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000151:congenital / Q000000981:diagnostic imaging* / Q000235:genetics; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D013701:Temporal Bone / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed
chemical_list: D017630:Connexins; C000607042:GJB2 protein, human; D000072259:Connexin 26
keywords: DFNB1; computed tomography; hearing loss