Heterozygous mutations in LGI1, which impair LGI1 secretion or binding to its receptors,9 cause autosomal dominant lateral temporal lobe epilepsy (ADLTE; OMIM #600512).10 Although ADAM22 and ADAM23 mutations have not been reported in human diseases, knockout mice for Lgi1,2,11,12Adam22,13 and Adam2314 manifest with lethal seizures during the first postnatal weeks. This evidence concerns the gene LGI1 and autosomal dominant epilepsy with auditory features.