title: Improved diagnostic effectiveness with a sequential diagnostic paradigm in idiopathic pediatric sensorineural hearing loss.
abstract: OBJECTIVE
  To determine whether a stepwise diagnostic paradigm is more diagnostically efficient and cost-effective than a simultaneous testing approach in the evaluation of idiopathic pediatric sensorineural hearing loss (SNHL).
  
  
  METHODS
  Prospective prevalence study.
  
  
  METHODS
  Tertiary referral children's hospital.
  
  
  METHODS
  Consecutive children (n = 150) presenting with idiopathic SNHL in the last 2 years.
  
  
  METHODS
  All children were evaluated with full diagnostic evaluations including GJB2 screens, temporal bone computed tomography scans, and laboratory investigations.
  
  
  METHODS
  1) Diagnostic yields of GJB2 screens, imaging, and laboratory results per SNHL category; 2) Cost analysis comparing a sequential versus a simultaneous testing approach.
  
  
  RESULTS
  Overall, 12.0% of patients had biallelic mutations in the GJB2 gene, whereas 30% of patients had an abnormality on temporal bone scan. Laboratory testing did not reveal the SNHL etiology in any patient. While maintaining diagnostic accuracy, significant cost savings were inferred by using a sequential diagnostic algorithm. Our data show children with severe to profound SNHL should first be tested with a GJB2 screen, as opposed to those with milder SNHL, who should undergo imaging as the initial testing step. In patients with initially positive GJB2 or imaging screens, logistic regression analysis significantly predicted negative results on further testing.
  
  
  CONCLUSIONS
  A stepwise diagnostic paradigm tailored to the level of the hearing loss in children with bilateral SNHL is more diagnostically efficient and cost effective than the more commonly used full, simultaneous testing approach. Laboratory investigation should not be routine but based on clinical history.
authors: Center for Hearing and Deafness Research (CHDR) and the Division of Pediatric Otolaryngology,Cincinnati, Ohio 45229-3039, USA.
authors: Diego A
authors: DA
authors: Preciado
authors: Louise
authors: L
authors: Lawson
authors: Colm
authors: C
authors: Madden
authors: David
authors: D
authors: Myer
authors: Chris
authors: C
authors: Ngo
authors: John K
authors: JK
authors: Bradshaw
authors: Daniel I
authors: DI
authors: Choo
authors: John H
authors: JH
authors: Greinwald
mesh_terms: D000293:Adolescent; D000483:Alleles; D002648:Child; D002675:Child, Preschool; D019411:Clinical Laboratory Techniques; D000072259:Connexin 26; D017630:Connexins / Q000235:genetics; D003363:Cost Control; D003430:Cross-Sectional Studies; D005820:Genetic Testing; D017048:Health Care Costs; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis* / Q000235:genetics; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D009154:Mutation; D011446:Prospective Studies; D013701:Temporal Bone / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed
chemical_list: D017630:Connexins; C000607042:GJB2 protein, human; D000072259:Connexin 26