title: A diagnostic paradigm for childhood idiopathic sensorineural hearing loss.
abstract: OBJECTIVE
  Our objective was to determine the diagnostic yield of laboratory testing, radiological imaging, and GJB2 mutation screening in a large cohort of patients with differing severities of idiopathic sensorineural hearing loss (SNHL).
  
  
  METHODS
  We undertook a retrospective study of patients presenting with SNHL at our institution from 1993 to 2002.
  
  
  RESULTS
  Laboratory testing had an extremely low yield. Patients with unilateral SNHL had a significantly higher imaging yield than those with bilateral. The diagnostic yield of GJB2 screening was significantly higher in patients with severe to profound SNHL than in those with less severe SNHL. However, a relatively large number of patients with mild to moderate SNHL had positive GJB2 screens.
  
  
  CONCLUSIONS
  Based on diagnostic yields, we propose a cost-effective stepwise diagnostic paradigm to replace the more commonly used and costly simultaneous testing approach.
  
  
  METHODS
  C.
authors.affiliation: Center for Hearing and Deafness Research, Division of Pediatric Otolaryngology--Head and Neck Surgery, Cincinnati Children's Hospital Medical Center, Ohio 45229-3039, USA.
authors.forename: Diego A
authors.initials: DA
authors.lastname: Preciado
authors.forename: Lynne H Y
authors.initials: LH
authors.lastname: Lim
authors.forename: Aliza P
authors.initials: AP
authors.lastname: Cohen
authors.forename: Colm
authors.initials: C
authors.lastname: Madden
authors.forename: David
authors.initials: D
authors.lastname: Myer
authors.forename: Chris
authors.initials: C
authors.lastname: Ngo
authors.forename: John K
authors.initials: JK
authors.lastname: Bradshaw
authors.forename: Louise
authors.initials: L
authors.lastname: Lawson
authors.forename: Daniel I
authors.initials: DI
authors.lastname: Choo
authors.forename: John H
authors.initials: JH
authors.lastname: Greinwald
mesh_terms: D000293:Adolescent; D000465:Algorithms; D002648:Child; D002675:Child, Preschool; D019411:Clinical Laboratory Techniques / Q000191:economics; D015331:Cohort Studies; D000072259:Connexin 26; D017630:Connexins / Q000235:genetics; D003362:Cost-Benefit Analysis; D004562:Electrocardiography; D005820:Genetic Testing / Q000191:economics; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis* / Q000191:economics / Q000235:genetics; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging / Q000191:economics; D012189:Retrospective Studies; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed / Q000191:economics
chemical_list: D017630:Connexins; C000607042:GJB2 protein, human; D000072259:Connexin 26