Modugno G C, Brandolini C
Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna.
Acta Otorhinolaryngol Ital. 2014 Feb;34(1):71-4.
Hyperacusis can be a prominent and disabling symptom of superior semicircular canal dehiscence associated with autophony and the Tullio phenomenon. We report three clinical cases characterized by disabling hyperacusis in which semicircular canals dehiscence was excluded by temporal bone high-resolution computed tomography. The images disclosed lateral semicircular canal dysplasia, characterized by a small bony island, and dilatation of both the anterior and the posterior arms of the lateral semicircular canal. Cochleo-vestibular examinations (pure tone audiometry, infra-red videonystagmoscopy, vibration-induced nystagmus test, vestibular evoked myogenic potentials) will also be described. To verify the transtympanic ventilation tube effect, bilateral myringotomies tubes were performed in one patient but no long lasting subjective benefit was noted. Concerning the pathophysiology of this condition, we hypothesized that the increased volume of inner ear liquid can modify the micromechanical function of the cochlea and the labyrinthine hydrodynamics. In conclusion, in the case of specific symptoms, such as hyperacusis, it is important to consider the possibility of an inner ear morphological alteration involving the lateral canal and vestibule structures, as well as the existence of bony semicircular canal dehiscence.
听觉过敏可能是上半规管裂的突出且致残性症状,与自听增强和图利奥现象相关。我们报告了三例以致残性听觉过敏为特征的临床病例,颞骨高分辨率计算机断层扫描排除了半规管裂。图像显示外侧半规管发育异常,其特征为一个小骨岛,以及外侧半规管前后臂扩张。还将描述耳蜗 - 前庭检查(纯音听力测定、红外视频眼震图、振动诱发眼震试验、前庭诱发肌源性电位)。为验证鼓膜置管效果,对一名患者进行了双侧鼓膜切开置管,但未观察到长期主观获益。关于这种情况的病理生理学,我们推测内耳液体量增加可改变耳蜗的微机械功能和迷路流体动力学。总之,对于听觉过敏等特定症状,重要的是要考虑内耳形态改变累及外侧半规管和前庭结构的可能性,以及骨半规管裂的存在。