Routine screening is typically initiated with GJB2 analysis because 30–40% of NSHL probands with European ancestry have mutations in this gene.1 Unless additional clinical symptoms hint at specific genes (i.e., goiter suggesting SLC26A4 or auditory neuropathy suggesting OTOF), the vast majority of GJB2 mutation–negative probands remain without genetic diagnoses. The gene discussed is SLC26A4; the disease is goiter.