There has only been one murine study evaluating auditory dysfunction in ENPP1 deficiency, in which the ENPP1asj/asj mouse, a model of ENPP1 deficiency, developed progressive conductive HL (CHL), otitis media with effusion, and features of ectopic mineralization of the middle ear structures, including fusion of the malleus and incus and a thickened and overly calcified stapedial artery [9]. Here, ENPP1 is linked to otitis media with effusion.