Zheng Q Y, Johnson K R, Erway L C
The Jackson Laboratory, Bar Harbor, ME 04609-1500, USA.
Hear Res. 1999 Apr;130(1-2):94-107. doi: 10.1016/s0378-5955(99)00003-9.
The common occurrence of hearing loss in both humans and mice, and the anatomical and functional similarities of their inner ears, attest to the potential of mice being used as models to study inherited hearing loss. A large-scale, auditory screening project is being undertaken at The Jackson Laboratory (TJL) to identify mice with inherited hearing disorders. To assess hearing sensitivity, at least five mice from each inbred strain had auditory brainstem response (ABR) thresholds determined. Thus far, we have screened 80 inbred strains of mice; 60 of them exhibited homogeneous ABR threshold values not significantly different from those of the control strain CBA/CaJ. This large database establishes a reliable reference for normal hearing mouse strains. The following 16 inbred strains exhibited significantly elevated ABR thresholds before the age of 3 months: 129/J, 129/ReJ, 129/SvJ, A/J, ALR/LtJ, ALS/LtJ, BUB/BnJ, C57BLKS/J, C57BR/cdJ, C57L/J, DBA/2J, I/LnJ, MA/MyJ, NOD/LtJ, NOR/LtJ, and SKH2/J. These hearing impaired strains may serve as models for some forms of human non-syndromic hearing loss and aid in the identification of the underlying genes.
人类和小鼠中听力损失都很常见,而且它们内耳在解剖结构和功能上存在相似性,这证明了小鼠作为研究遗传性听力损失模型的潜力。杰克逊实验室(TJL)正在开展一项大规模听觉筛查项目,以鉴定患有遗传性听力障碍的小鼠。为了评估听力敏感性,对每个近交系的至少五只小鼠测定了听觉脑干反应(ABR)阈值。到目前为止,我们已经筛查了80个小鼠近交系;其中60个表现出与对照品系CBA/CaJ的ABR阈值无显著差异的均一值。这个大型数据库为正常听力小鼠品系建立了可靠的参考。以下16个近交系在3个月龄之前表现出显著升高的ABR阈值:129/J、129/ReJ、129/SvJ、A/J、ALR/LtJ、ALS/LtJ、BUB/BnJ、C57BLKS/J、C57BR/cdJ、C57L/J、DBA/2J、I/LnJ、MA/MyJ、NOD/LtJ、NOR/LtJ和SKH2/J。这些听力受损品系可作为某些形式人类非综合征性听力损失的模型,并有助于鉴定潜在基因。