Variability in genetically induced age-related impairment of auditory brainstem response thresholds

In genetically induced hearing impairment or deafness, the recessive mode of inheritance is by far the most common, but it has seldom been feasible to document audiological aberrations. In this study, auditory brainstem response (ABR) thresholds were analysed in groups of 10 heterozygote jerker (je/+) mice each, at ages of 3, 6 and 9 months in the frequency range 2-31.5 kHz. The animals were thereafter regularly tested up to a final age of 12 months. Forty age-related CBA/CBA mice were used as controls. The individual ABR thresholds in 3-, 6-, 9- and 12-month-old CBA/CBA mice were very similar, as also were those in 3- and 6-month-old je/+ mice (mean values within the normal range). In contrast, at 9 months--and even more so at 12 months--all je/+ animals showed large individual variations, with ABR thresholds varying randomly from near-normal to profound impairment. The wide individual spread of ABR thresholds in old je/+ animals was interpreted as reflecting the expression of a random influence of recessive jerker vis-à-vis the wild type of gene.