Bassila M K, Goldberg R
Department of Otolaryngology, Montefiore Medical Center, Bronx, NY 10467.
Cleft Palate J. 1989 Oct;26(4):287-91.
Hemifacial microsomia (HFM) is a common craniofacial disorder that is known to be etiologically heterogenous. Phenotypic differentiation of the various subgroups remains unresolved. A review of 50 patients with HFM has yielded data that may help explain different pathogenetic processes. Of particular interest is the association of facial nerve palsy, sensorineural hearing loss (SNHL), or both in a higher percentage of patients than expected. Twenty-two percent had evidence of facial palsy of varying degree. Thirty-three cases had microtia or anotia, and all instances of facial palsy were associated with auricular malformation. Sensorineural hearing loss was found in 16 percent. All patients with microtia and sensorineural hearing loss had facial palsy. Ear tags or pits were found in 21 patients, only two of whom had facial palsy. In all but one case the palsy was found on the more hypoplastic side of the face. In the single exception, both sides of the face were hypoplastic.
半侧颜面短小畸形（HFM）是一种常见的颅面疾病，已知其病因具有异质性。各亚组的表型分化仍未明确。对50例HFM患者的回顾研究得出了一些数据，可能有助于解释不同的致病过程。特别值得关注的是，面神经麻痹、感音神经性听力损失（SNHL）或两者同时出现的患者比例高于预期。22%的患者有不同程度面神经麻痹的证据。33例患者有小耳畸形或无耳畸形，所有面神经麻痹病例均与耳部畸形相关。感音神经性听力损失的发生率为16%。所有小耳畸形且有感音神经性听力损失的患者均有面神经麻痹。21例患者有附耳或耳前瘘管，其中只有2例有面神经麻痹。除1例例外情况外，所有病例的面神经麻痹均出现在面部发育较差的一侧。在这一例外情况中，面部两侧均发育不良。
