Lefebvre P P, Staecker H, Van de Water T, Moonen G, Malgrange B
Dept. of Otorhinolaryngology, University of Liège, Belgium.
Acta Otorhinolaryngol Belg. 2002;56(1):45-9.
Most of the deafness are of sensorineural origin and are characterized by a loss of hair cells and of spiral ganglion neurons. At the present time, hearing aids are the only treatment. However, in some diseases of the inner ear, pharmacological treatment have been proposed and used successfully. In this paper, we will review some basic science aspects of the biology of the neurosensory structures of the inner ear, in particular of the auditory neurons, that lead to the rationale of some treatments for the inner ear diseases. Developmental studies, neuronal cell culture experiments, and analyses of gene knockout animals reveal a number of growth factors which are important for the rescue and repair of injured auditory neurons in the inner ear. These factors rescue the injured auditory neurons in vivo. Furthermore, perfusion of antioxydant to the cochlea prevented the hearing loss induced by cisplatin. These in vitro and in vivo experiments demonstrate that it is possible to manipulate the neurosensory structures of the inner ear and provide an effective treatment to prevent the degeneration of the neurons. The molecules or drugs can be administered locally to the inner ear through a direct perilymphatic perfusion or through the round window membrane. As an example, we will discuss the treatment of patients suffering from idiopathic sensorineural hearing loss which can be treated successfully by a perfusion through the round window membrane, improving their hearing threshold and their speech discrimination.
大多数耳聋源于感音神经性，其特征是毛细胞和螺旋神经节神经元缺失。目前，助听器是唯一的治疗方法。然而，在一些内耳疾病中，已提出并成功应用了药物治疗。在本文中，我们将回顾内耳神经感觉结构生物学的一些基础科学方面，特别是听觉神经元的，这些方面为内耳疾病的一些治疗提供了理论依据。发育研究、神经元细胞培养实验以及基因敲除动物分析揭示了一些对拯救和修复内耳受损听觉神经元很重要的生长因子。这些因子在体内拯救了受损的听觉神经元。此外，向耳蜗灌注抗氧化剂可预防顺铂诱导的听力损失。这些体外和体内实验表明，有可能对内耳的神经感觉结构进行调控，并提供有效的治疗方法来防止神经元退化。分子或药物可通过直接的外淋巴灌注或通过圆窗膜局部给药至内耳。例如，我们将讨论特发性感音神经性听力损失患者的治疗，通过圆窗膜灌注可成功治疗该疾病，提高其听力阈值和言语辨别能力。
