Based on our previous experience with GLRA1 and SLC6A5, we consider it likely that a number of recessive SNVs remain to be discovered in GLRB. Based on the discovery of four new GLRB variants associated with hyperekplexia in this year alone (Al-Owain et al., 2012; Lee et al., 2012, this study), we recommend that GLRB should have equal status alongside GLRA1 and SLC6A5 in the molecular genetic diagnosis of startle disease. This evidence concerns the gene GLRA1 and hereditary hyperekplexia.