A small number of mouse mutants with continuous tremor and hearing impairment have been reported (MGI, OMIM), including several other ion channel mutants [Atp2b2 (plasma membrane calcium-transporting ATPase 2): deafwaddler2J (Noben-Trauth et al. 1997) and deafwaddler3J (McCullough & Tempel 2004); Spnb4 (spectrin beta 4): quivering (Parkinson et al. 2001); Kcnj10 (ATP-sensitive inward rectifier potassium channel 10) (Marcus et al. 2002; Neusch et al. 2001)]. Here, SPTBN4 is linked to Birt-Hogg-Dubé syndrome.