Hammami Bouthaina, Kharrat Ines, Ayed Mariam Ben, Hbaieb Youssef, Souissi Basma, Mahfoudh Kheireddine Ben, Achour Imen, Mnejja Malek, Chaabouni Mohamed Amine, Charfeddine Ilhem
Department of otorhinolaryngology head and neck surgery, Habib Bourguiba University Hospital, Sfax, Tunisia.
Sfax Medical School, University of Sfax, Sfax, LR23ES01 Tunisia.
Indian J Otolaryngol Head Neck Surg. 2024 Aug;76(4):3051-3058. doi: 10.1007/s12070-024-04596-w. Epub 2024 Mar 16.
Congenital facial nerve (FN) malformations are uncommon. Our aim is to determine the clinical and radiological features of FN malformations along with the associated cochlear and vestibular malformations. We conducted a retrospective study including children with a profound sensorineural hearing loss who were candidates for cochlear implantation. We evaluated the presence of FN malformations through temporal bone computed tomography scan and magnetic resonance imaging. We recorded an aberrant FN course in five out of 165 patients in a total of 9 ears. They consisted of a bifurcation of the mastoid segment, an anterior or posterior displacement of the labyrinthine segment and a hypoplasia of the geniculate ganglion. Associated inner ear malformations included vestibular aqueduct dilation, cochlear hypoplasia and total labyrinthine aplasia. We noted a bilateral agenesis of the cochlear nerve in three patients. Facial nerve malformations should be suspected in patients presenting a congenital hearing loss especially in association with other temporal bone malformations. Their pre-operative discovery is helpful in planning the surgical procedure.
先天性面神经（FN）畸形并不常见。我们的目的是确定FN畸形的临床和放射学特征以及相关的耳蜗和前庭畸形。我们进行了一项回顾性研究，纳入了重度感音神经性听力损失且适合进行人工耳蜗植入的儿童。我们通过颞骨计算机断层扫描和磁共振成像评估FN畸形的存在情况。在165例患者中的9只耳中，我们记录到5例FN走行异常。它们包括乳突段分叉、迷路段向前或向后移位以及膝状神经节发育不全。相关的内耳畸形包括前庭导水管扩张、耳蜗发育不全和全迷路发育不全。我们注意到3例患者存在双侧耳蜗神经缺如。对于先天性听力损失尤其是合并其他颞骨畸形的患者，应怀疑存在面神经畸形。术前发现有助于手术方案的制定。
