Evidence-based algorithm for the evaluation of a child with bilateral sensorineural hearing loss.
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.
Morzaria
Sanjay
S
Division of Otolaryngology, University of British Columbia.
Westerberg
Brian D
BD
Kozak
Frederick K
FK
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
D017630:Connexins; C000607042:GJB2 protein, human; D000072259:Connexin 26; C026215:3-hydroxyproline; D006909:Hydroxyproline