Nager G T, Kennedy D W, Kopstein E
Ann Otol Rhinol Laryngol Suppl. 1982 May-Jun;92:1-52.
Fibrous dysplasia is a fairly common, localized misdifferentiation of the bone-forming mesenchyme affecting a single or many bones, in which skeletal aberrations represent the cardinal feature, but in which certain endocrinopathies, abnormal pigmentation of skin and mucous membrane, and occasionally other abnormalities form part of the entire disease process. The craniofacial skeleton is one of its predilective sites and therefore the temporal bone may become involved. In such instances the disease manifests itself with 1) progressive loss of hearing, 2) increasing obliteration of the external ear canal, and 3) enlargement and distortion of the temporal bone. The first part of this communication is concerned with a review of the clinical and pathological aspects of the disease in general. The second part is concerned with a discussion of the clinical manifestations in the temporal bone, based upon analysis of the entire literature and the authors' personal experience. The indications for surgical intervention include 1) maintenance of a normal outer ear canal, 2) preservation of cochlear and vestibular function, and 3) prevention of secondary complications.
骨纤维异常增殖症是一种相当常见的、局限性的骨形成间充质错向分化疾病，可累及单骨或多骨，骨骼畸变是其主要特征，但某些内分泌病、皮肤和黏膜色素沉着异常以及偶尔的其他异常也是整个疾病过程的一部分。颅面骨骼是其好发部位之一，因此颞骨可能受累。在这种情况下，疾病表现为：1）听力进行性丧失；2）外耳道闭锁加重；3）颞骨增大和变形。本文第一部分总体回顾了该疾病的临床和病理方面。第二部分基于对全部文献和作者个人经验的分析，讨论了颞骨的临床表现。手术干预的指征包括：1）维持外耳道正常；2）保留耳蜗和前庭功能；3）预防继发性并发症。
