Animal models with genetic abnormalities have been increasingly used in auditory research. Both TrJ mice and Po-DT-A mice are animals with peripheral myelin deficiency. In TrJ mice, the defect is due to a mutated PMP-22 gene. In Po-DT-A mice, the defect is produced by a transgene using the rat Po promotor to direct the expression of gene encoding for the bacterial diphtherial toxin A chain (DT-A). This study evaluates the auditory system both physiologically and histologically in these two strains of mice. Histological examination revealed that there was myelin deficiency of the auditory nerve fibers, accompanied by a loss of dendrites and a loss of spiral ganglion cell bodies in both strains of mice. In general, histological deficits in TrJ mice were greater than those in Po-DT-A mice. There was a strong correlation between the degree of myelin deficiency and the survival of spiral ganglion neurons. ABR measurements exhibited differences in threshold, latency and slope of the ABR growth function between myelin-deficient mice and their respective controls. These results suggest that the integrity of the myelin in the auditory nerve is important both for neural survival and for normal electrophysiological function of spiral ganglion neurons.