GJB2 and hearing loss disorder: Although it has been recommended to consider SLC26A4 mutation analysis if there is progressive hearing loss, goiter, Mondini dysplasia, or EVA (Hilgert, Smith & Van Camp, 2009), the clinical testing strategy often adheres to the following algorithm: For individuals with congenital hearing loss that is consistent with autosomal recessive inheritance and that appears non-syndromic, diagnostic testing typically starts with GJB2 sequence analysis.