Han Fengchan, Yu Heping, Zhang Jiangping, Tian Cong, Schmidt Cecilia, Nava Casey, Davisson Muriel T, Zheng Qing Y
Department of Otolaryngology-HNS, Case Western Reserve University, Cleveland, OH 44106, USA.
Int J Exp Pathol. 2009 Oct;90(5):480-8. doi: 10.1111/j.1365-2613.2009.00677.x.
The Ts65Dn mouse shares many phenotypic characteristics of human Down syndrome. Here, we report that otitis media, characterized by effusion in the middle ear and hearing loss, was prevalent in Ts65Dn mice. Of the 53 Ts65Dn mice tested, 81.1% had high auditory-evoked brainstem response (ABR) thresholds for at least one of the stimulus frequencies (click, 8 kHz, 16 kHz and 32 kHz), in at least one ear. The ABR thresholds were variable and showed no tendency toward increase with age, from 2 to 7 months of age. Observation of pathology in mice, aged 3-4 months, revealed middle ear effusion in 11 of 15 Ts65Dn mice examined, but only in two of 11 wild-type mice. The effusion in each mouse varied substantially in volume and inflammatory cell content. The middle ear mucosae were generally thickened and goblet cells were distributed with higher density in the epithelium of the middle ear cavity of Ts65Dn mice as compared with those of wild-type controls. Bacteria of pathogenic importance to humans also were identified in the Ts65Dn mice. This is the first report of otitis media in the Ts65Dn mouse as a model characteristic of human Down syndrome.
Ts65Dn小鼠具有许多人类唐氏综合征的表型特征。在此，我们报告以中耳积液和听力损失为特征的中耳炎在Ts65Dn小鼠中很普遍。在测试的53只Ts65Dn小鼠中，81.1%的小鼠至少一只耳朵在至少一种刺激频率（咔嗒声、8 kHz、16 kHz和32 kHz）下具有高听觉脑干反应（ABR）阈值。从2到7个月龄，ABR阈值是可变的，且没有随年龄增加的趋势。对3 - 4个月龄小鼠的病理学观察显示，在检查的15只Ts65Dn小鼠中有11只出现中耳积液，而在11只野生型小鼠中只有2只出现。每只小鼠的积液在体积和炎性细胞含量上有很大差异。与野生型对照相比，Ts65Dn小鼠中耳黏膜普遍增厚，中耳腔上皮中杯状细胞分布密度更高。在Ts65Dn小鼠中还鉴定出了对人类具有致病重要性的细菌。这是关于Ts65Dn小鼠中耳炎作为人类唐氏综合征模型特征的首次报道。
