Saito R, Jurado A B, Inokuchi I, Koide I, Tomotsu T, Mohammed M B, Ogura Y
Acta Med Okayama. 1987 Jun;41(3):125-31. doi: 10.18926/AMO/31763.
Temporal bone histopathological findings of two patients with trisomy 18 syndrome are described. Many of the abnormalities previously described were seen in the present cases; namely, atresia of the external auditory canal, aberrant course of the tensor tympani muscle, malformed stapes, aberrant course of the facial nerve with an obtuse angulation at the first genu and displacement of geniculate ganglion cells into the internal auditory canal, shortened cochlea with decreased spiral ganglion cell population, and vestibular anomalies, such as bony and membranous blockage of the superior semicircular canal. Moreover, an extremely underdeveloped malleus and incus continuous with a persistent Meckel's cartilage were observed.
描述了两名18三体综合征患者的颞骨组织病理学发现。本病例中可见许多先前描述的异常情况,即外耳道闭锁、鼓膜张肌走行异常、镫骨畸形、面神经走行异常,在第一膝部呈钝角且膝状神经节细胞向内耳道移位、耳蜗缩短且螺旋神经节细胞数量减少,以及前庭异常,如上前庭半规管的骨性和膜性阻塞。此外,还观察到锤骨和砧骨极度发育不全,并与持续存在的Meckel软骨相连。