Green Kari L, Swiderski Donald L, Prieskorn Diane M, DeRemer Susan J, Beyer Lisa A, Miller Josef M, Green Glenn E, Raphael Yehoash
Kresge Hearing Research Institute, Department of Otolaryngology - Head and Neck Surgery, The University of Michigan, Ann Arbor, MI, USA.
Sci Rep. 2016 Mar 11;6:22690. doi: 10.1038/srep22690.
Dietary supplements consisting of beta-carotene (precursor to vitamin A), vitamins C and E and the mineral magnesium (ACEMg) can be beneficial for reducing hearing loss due to aminoglycosides and overstimulation. This regimen also slowed progression of deafness for a boy with GJB2 (CONNEXIN 26) mutations. To assess the potential for treating GJB2 and other forms of hereditary hearing loss with ACEMg, we tested the influence of ACEMg on the cochlea and hearing of mouse models for two human mutations: GJB2, the leading cause of childhood deafness, and DIAPH3, a cause of auditory neuropathy. One group of mice modeling GJB2 (Gjb2-CKO) received ACEMg diet starting shortly after they were weaned (4 weeks) until 16 weeks of age. Another group of Gjb2-CKO mice received ACEMg in utero and after weaning. The ACEMg diet was given to mice modeling DIAPH3 (Diap3-Tg) after weaning (4 weeks) until 12 weeks of age. Control groups received food pellets without the ACEMg supplement. Hearing thresholds measured by auditory brainstem response were significantly better for Gjb2-CKO mice fed ACEMg than for the control diet group. In contrast, Diap3-Tg mice displayed worse thresholds than controls. These results indicate that ACEMg supplementation can influence the progression of genetic hearing loss.
由β-胡萝卜素（维生素A的前体）、维生素C和E以及矿物质镁（ACEMg）组成的膳食补充剂，可能有助于减少因氨基糖苷类药物和过度刺激导致的听力损失。对于一名患有GJB2（连接蛋白26）突变的男孩，这种疗法还减缓了耳聋的进展。为了评估用ACEMg治疗GJB2和其他形式遗传性听力损失的可能性，我们测试了ACEMg对两种人类突变小鼠模型的耳蜗和听力的影响：GJB2是儿童期耳聋的主要原因，DIAPH3是听觉神经病的一个病因。一组模拟GJB2的小鼠（Gjb2-CKO）在断奶后不久（4周）开始接受ACEMg饮食，直至16周龄。另一组Gjb2-CKO小鼠在子宫内和断奶后接受ACEMg。模拟DIAPH3的小鼠（Diap3-Tg）在断奶后（4周）接受ACEMg饮食，直至12周龄。对照组接受不含ACEMg补充剂的食丸。通过听觉脑干反应测量的听力阈值，喂食ACEMg的Gjb2-CKO小鼠明显优于对照饮食组。相比之下，Diap3-Tg小鼠的阈值比对照组更差。这些结果表明，补充ACEMg可以影响遗传性听力损失的进展。
