The heterozygote mouse, Tmem43+/KI, which mimics the counterpart human ANSD subjects, showed a significant reduction of 63% in the passive conductance current in GLSs (Fig. 7 J and K), confirming the significant dominant-negative pathogenic effect of the TMEM43 variant on the function of GLSs. The gene discussed is TMEM43; the disease is auditory neuropathy.