Parnes L S, Chernoff W G
Department of Otolaryngology, University of Western Ontario, London, Canada.
Ann Otol Rhinol Laryngol. 1990 Dec;99(12):957-9. doi: 10.1177/000348949009901205.
Congenital malformations of the vestibular labyrinth (pars superior) are rare. We present two patients with computed tomographic findings of bilateral semicircular canal aplasia with normal or near-normal cochleas. Initial bone conduction thresholds were within normal limits, although both patients had significant conductive hearing losses due to congenital middle ear malformations. Bithermal caloric responses were absent in both. To our knowledge these are the first reports of vestibular aplasia concomitant with normal or near-normal cochlear development. These findings conflict with conventional hypotheses that state that inner ear malformations result from arrested development during the normal stages of inner ear embryogenesis.
前庭迷路（上半规管）的先天性畸形较为罕见。我们报告了两名患者，其计算机断层扫描结果显示双侧半规管发育不全，而耳蜗正常或接近正常。尽管两名患者均因先天性中耳畸形而存在明显的传导性听力损失，但初始骨导阈值仍在正常范围内。两人的冷热试验均无反应。据我们所知，这些是前庭发育不全合并正常或接近正常耳蜗发育的首例报告。这些发现与传统假说相矛盾，传统假说认为内耳畸形是内耳胚胎发育正常阶段发育停滞所致。
