Webster D B
Kresge Hearing Research Laboratory, Department of Otorhinolaryngology, Louisiana State University Medical Center, New Orleans 70112.
Exp Neurol. 1992 Jan;115(1):27-31. doi: 10.1016/0014-4886(92)90216-d.
The deafness mouse is a Mendelian recessive mutant which never hears and has no stimulus-related receptor or neural auditory responses. From birth through 12 days after birth (DAB), the organ of Corti develops normally as seen with light microscopy, except that the space of Nuel does not fully develop. The inner and outer hair cells are degenerating between 12 and 24 DAB and are gone by 45 DAB. As the hair cells degenerate, other cells of the organ of Corti become less recognizable, appear to collapse, and lose their identities as differentiated cells. By 45 DAB, from base to apex, the organ of Corti is composed of a low, roughly cuboidal epithelium with no distinguishing cell types; a hint of a tunnel of Corti remains at the apex. In the basal turn, the organ of Corti remains in this degenerated state through at least 460 DAB (senility for these mice). In the apical organ of Corti, considerable regeneration occurs between 45 and 90 DAB. By 90 DAB the apical turn of the organ of Corti has readily identifiable inner and outer pillar cells, inner and outer supporting cells, Hensen's cells, and Claudius' cells. A tunnel of Corti and space of Nuel are also present in the apex but there are no hair cells. Mechanisms are not known for either the degeneration or the regeneration.
耳聋小鼠是一种孟德尔隐性突变体，从不具备听力，没有与刺激相关的感受器或神经听觉反应。从出生到出生后12天（DAB），用光学显微镜观察发现，柯蒂氏器正常发育，只是纽尔间隙没有完全发育。内、外毛细胞在出生后12至24天之间开始退化，到45天DAB时消失。随着毛细胞退化，柯蒂氏器的其他细胞变得难以辨认，似乎发生塌陷，并失去其作为分化细胞的特性。到45天DAB时，从底部到顶部，柯蒂氏器由一层低矮、大致呈立方形的上皮组成，没有明显的细胞类型；在顶部仍有一丝柯蒂氏隧道的痕迹。在基部转弯处，柯蒂氏器至少在460天DAB（这些小鼠的衰老期）时仍处于这种退化状态。在顶部的柯蒂氏器中，在45至90天DAB之间发生了大量再生。到90天DAB时，柯蒂氏器的顶部转弯处有易于辨认的内、外柱细胞、内、外支持细胞、亨森细胞和克劳迪乌斯细胞。顶部也有柯蒂氏隧道和纽尔间隙，但没有毛细胞。对于退化和再生的机制均尚不清楚。
