Evidence-based algorithm for the evaluation of a child with bilateral sensorineural hearing loss. 
To develop an evidence-based algorithm for determining the etiology of bilateral sensorineural hearing loss (SNHL) in a child.  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.  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).  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.