Monge Naldi Arianne, Belfrage Celina, Jain Neha, Wei Eric T, Canto Martorell Belén, Gassmann Max, Vogel Johannes
Department of Inner Ear Research, Clinic for Otolaryngology, Head and Neck Surgery, University Hospital Zürich, University of Zürich, Zürich, Switzerland.
Department of Inner Ear Research, Clinic for Otolaryngology, Head and Neck Surgery, University Hospital Zürich, University of Zürich, Zürich, Switzerland; Institute of Veterinary Physiology, Vetsuisse Faculty, University of Zürich, Zürich, Switzerland.
Neurobiol Aging. 2015 Dec;36(12):3278-3287. doi: 10.1016/j.neurobiolaging.2015.08.015. Epub 2015 Aug 24.
So far, typical causes of presbycusis such as degeneration of hair cells and/or primary auditory (spiral ganglion) neurons cannot be treated. Because erythropoietin's (Epo) neuroprotective potential has been shown previously, we determined hearing thresholds of juvenile and aged mice overexpressing Epo in neuronal tissues. Behavioral audiometry revealed in contrast to 5 months of age, that 11-month-old Epo-transgenic mice had up to 35 dB lower hearing thresholds between 1.4 and 32 kHz, and at the highest frequencies (50-80 kHz), thresholds could be obtained in aged Epo-transgenic only but not anymore in old C57BL6 control mice. Click-evoked auditory brainstem response showed similar results. Numbers of spiral ganglion neurons in aged C57BL6 but not Epo-transgenic mice were dramatically reduced mainly in the basal turn, the location of high frequencies. In addition, there was a tendency to better preservation of inner and outer hair cells in Epo-transgenic mice. Hence, Epo's known neuroprotective action effectively suppresses the loss of spiral ganglion cells and probably also hair cells and, thus, development of presbycusis in mice.
到目前为止,诸如毛细胞和/或初级听觉(螺旋神经节)神经元退化等典型的老年性聋病因无法得到治疗。由于先前已显示促红细胞生成素(Epo)具有神经保护潜力,我们测定了在神经元组织中过表达Epo的幼年和老年小鼠的听力阈值。行为听力测定显示,与5月龄小鼠相比,11月龄的Epo转基因小鼠在1.4至32kHz之间的听力阈值低达35dB,并且在最高频率(50 - 80kHz)时,仅在老年Epo转基因小鼠中可测得阈值,而在老年C57BL6对照小鼠中则无法测得。短声诱发的听觉脑干反应显示了类似的结果。在老年C57BL6小鼠而非Epo转基因小鼠中,螺旋神经节神经元数量显著减少,主要发生在高频所在的蜗底。此外,Epo转基因小鼠的内外毛细胞有更好的保留趋势。因此,Epo已知的神经保护作用有效地抑制了螺旋神经节细胞的丢失,可能还抑制了毛细胞的丢失,从而抑制了小鼠老年性聋的发展。