title: Evidence-based algorithm for the evaluation of a child with bilateral sensorineural hearing loss.
abstract: OBJECTIVE
  To develop an evidence-based algorithm for determining the etiology of bilateral sensorineural hearing loss (SNHL) in a child.
  
  
  METHODS
  The frequency of different etiologies was previously determined. A systematic review of the literature for articles published between 1940 and January 2003 was performed for studies providing information on the diagnosis of each etiology relevant to their clinical presentation.
  
  
  RESULTS
  Connexin mutation testing is highly sensitive and specific. CT scanning of the temporal bones is frequently valuable in detecting inner ear malformations. Routine laboratory studies are rarely helpful. ECG is particularly valuable when a history of syncope or arrhythmias or a family history of sudden death in a young child is elicited. There is no literature to support routine urinalysis for the diagnosis of Alport syndrome and thyroid studies lack specificity in the absence of physical findings (goiter).
  
  
  CONCLUSIONS
  An evidence-based algorithm was developed that included: history, physical and audiological evaluation, and ophthalmological evaluation. Further directed investigations may include genetic testing for the Cx26 mutation, CT scan of the temporal bones, ECG and urinalysis.
authors.affiliation: Division of Otolaryngology, University of British Columbia.
authors.forename: Sanjay
authors.initials: S
authors.lastname: Morzaria
authors.forename: Brian D
authors.initials: BD
authors.lastname: Westerberg
authors.forename: Frederick K
authors.initials: FK
authors.lastname: Kozak
mesh_terms: D000465:Algorithms; D002648:Child; D000072259:Connexin 26; D017630:Connexins / Q000235:genetics*; D007758:Ear, Inner / Q000000981:diagnostic imaging / Q000473:pathology*; D004562:Electrocardiography; D019317:Evidence-Based Medicine / Q000379:methods*; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D006909:Hydroxyproline / Q000652:urine; D009154:Mutation; D009394:Nephritis, Hereditary / Q000175:diagnosis; D013701:Temporal Bone / Q000000981:diagnostic imaging; D013960:Thyroid Function Tests; D014057:Tomography, X-Ray Computed
chemical_list: D017630:Connexins; C000607042:GJB2 protein, human; D000072259:Connexin 26; C026215:3-hydroxyproline; D006909:Hydroxyproline