Kaga Kimitaka, Nakamura Masako, Takayama Yoshihiro, Momose Hiromitsu
Department of Otolaryngology, Graduate School of Medicine, University of Tokyo, Japan.
Acta Otolaryngol. 2004 Mar;124(2):202-5. doi: 10.1080/00016480310015975.
Generally, cortical deafness is not complicated by anarthria and cortical anarthria does not affect auditory perception. We report a case of simultaneous progressive cortical deafness and anarthria. At the age of 70 years, the patient, a woman, noticed hearing problems when using the telephone, which worsened rapidly over the next 2 years. She was then referred to our hospital for further examinations of her hearing problems. Auditory tests revealed threshold elevation in the low and middle frequencies on pure-tone audiometry, a maximum speech discrimination of 25% and normal otoacoustic emissions and auditory brainstem, middle- and long-latency responses. An articulation test revealed abnormal pronunciation. Because of these problems only written and not verbal communication was possible; her ability to read and write was unimpaired. She showed no other neurological problems. Brain MRI demonstrated atrophic changes of the auditory cortex and Wernicke's language center and PET suggested low uptake of (18F) 2-fluoro-2-deoxy-d-glucose around the Sylvian fissures in both hemispheres. Neurologically, the patient was suspected of having progressive aphasia or frontotemporal dementia. Her cortical deafness and anarthria are believed to be early signs of this entity.
一般来说，皮质聋不会并发构音障碍，而皮质性构音障碍也不会影响听觉感知。我们报告一例同时出现进行性皮质聋和构音障碍的病例。患者为一名70岁女性，70岁时使用电话时注意到听力问题，在接下来的2年里迅速恶化。随后她被转诊至我院进一步检查听力问题。听力测试显示纯音听力测定中低频和中频阈值升高，最大言语辨别率为25%，耳声发射及听觉脑干、中潜伏期和长潜伏期反应正常。构音测试显示发音异常。由于这些问题，只能进行书面而非口头交流；她的读写能力未受损。她没有其他神经系统问题。脑部MRI显示听觉皮层和韦尼克语言中枢萎缩性改变，PET显示双侧大脑半球外侧裂周围（18F）2-氟-2-脱氧-D-葡萄糖摄取降低。神经学上，该患者被怀疑患有进行性失语或额颞叶痴呆。她的皮质聋和构音障碍被认为是该疾病的早期迹象。
