Coppens A G, Résibois A, Poncelet L
Laboratoire d'Anatomie Fonctionnelle, CP 619, Université Libre de Bruxelles, 808 Route de Lennik, Bruxelles, B-1070, Belgium.
J Comp Pathol. 2000 Feb-Apr;122(2-3):223-8. doi: 10.1053/jcpa.1999.0360.
Two puppies, a 4-month-old female Maltese terrier and a 6-week-old male Great Pyrenean, were presented for confirmation of bilateral deafness by electrophysiological testing. In both puppies, brainstem auditory potentials were not evoked by 90 dB NHL click stimulation of each ear. Examination of the inner ear revealed a bilateral cochleo-saccular degeneration in both animals. The lesions were characterized by generalized atrophy of the stria vascularis, collapse of the cochlear duct, degeneration of the organ of Corti, an abnormal tectorial membrane, and saccular collapse, with a normal spiral ganglion. The cochlear duct was entirely obliterated throughout the cochleae in the Maltese terrier puppy, but was locally and asymmetrically affected in the Great Pyrenean. The abnormalities observed in the Maltese terrier puppy were identical with those previously described in deaf Dalmatian puppies; the lesions observed in the Great Pyrenean, however, were less typical. This is the first histopathological description of cochleo-saccular degeneration in the Maltese terrier and Great Pyrenean breeds. In both puppies the defect was probably congenital.
两只幼犬，一只4个月大的雌性马尔济斯犬和一只6周大的雄性大白熊犬，因通过电生理测试确认双耳失聪前来就诊。在这两只幼犬中，每只耳朵经90分贝非标准化听力级（NHL）短声刺激均未引出脑干听觉电位。内耳检查显示两只动物均存在双侧蜗球囊变性。病变特征为血管纹普遍萎缩、蜗管塌陷、柯蒂氏器变性、盖膜异常以及球囊塌陷，螺旋神经节正常。在马尔济斯幼犬中，整个耳蜗的蜗管完全闭塞，但在大白熊犬中蜗管仅局部且不对称地受到影响。在马尔济斯幼犬中观察到的异常与先前在失聪斑点犬幼犬中描述的异常相同；然而，在大白熊犬中观察到的病变不太典型。这是首次对马尔济斯犬和大白熊犬品种的蜗球囊变性进行组织病理学描述。在这两只幼犬中，缺陷可能是先天性的。
