Rosanowski F, Tigges M, Eysholdt U
Abteilung für Phoniatrie und Pädaudiologie, Universitäts-HNO-Klinik Erlangen.
Laryngorhinootologie. 1995 Jun;74(6):343-7. doi: 10.1055/s-2007-997754.
The incidence of malformations of the ear is 1:5000, the incidence of stenosis and atresia is 1:12,000. 30% of the malformations are bilateral. In only 0.3% it is the only malformation. Inherited bilateral atresia of the external auditory canal associated with hypertelorism as the only additional malformation has not yet been described. Basing on a case report the way of interdisciplinary rehabilitation of children affected by bilateral hearing impairment is discussed. Special emphasis is on the discussion of the necessity of radiological examinations for the exclusion of cholesteatoma in an atretic ear which could be found in a 5-year old boy of the described family.
耳部畸形的发生率为1:5000，狭窄和闭锁的发生率为1:12000。30%的畸形为双侧性。仅0.3%的病例为单纯耳部畸形。遗传性双侧外耳道闭锁合并眼距过宽作为唯一额外畸形的情况尚未见报道。基于一例病例报告，讨论了双侧听力障碍患儿的多学科康复方法。特别强调了对一名来自所述家庭的5岁男孩进行放射学检查以排除闭锁耳中耳胆脂瘤的必要性。
