Khetarpal U, Schuknecht H F
Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts.
Ann Otol Rhinol Laryngol Suppl. 1990 Mar;145:1-16.
Mixed sensorineural and conductive hearing loss is a common clinical manifestation of Paget's disease of the temporal bone, and while there are numerous clinical and pathologic reports on the condition, none have identified a consistent pathologic explanation for the hearing loss. We performed histologic studies on 26 temporal bones exhibiting Paget's disease from 16 persons, of whom 7 had audiometric testing performed. Contrary to common opinion, the conductive hearing loss is not caused by ossicular fixation; in fact, no cause could be found in the seven ears with documented conductive hearing losses. While the sensorineural hearing losses were greater than normal for age, we could not identify cochlear disorders that could be attributed to Paget's disease. It is concluded that the hearing losses in Paget's disease are caused by changes in bone density, mass, and form that serve to dampen the finely tuned motion mechanics of the middle and inner ears.
混合性感音神经性和传导性听力损失是颞骨佩吉特病的常见临床表现，虽然有许多关于该病症的临床和病理报告，但尚无一份报告能确定听力损失一致的病理学解释。我们对来自16人的26块呈现佩吉特病的颞骨进行了组织学研究，其中7人接受了听力测试。与普遍观点相反，传导性听力损失并非由听骨固定所致；事实上，在有记录的传导性听力损失的7只耳朵中未发现病因。虽然感音神经性听力损失高于同龄人正常水平，但我们无法确定可归因于佩吉特病的耳蜗疾病。结论是，佩吉特病中的听力损失是由骨密度、质量和形态的变化引起的，这些变化会抑制中耳和内耳精细调节的运动机制。
