Chorda tympani neuroma.
Chorda tympani neuroma.
复制标题
鼓索神经瘤。
DOI：
10.1097/mao.0b013e3181b4e79b
复制
发表时间：
2010
期刊：
影响因子：
--
通讯作者：
Steven W Cheung
中科院分区：
文献类型：
--
作者：
Kevin C Huoh;Steven W Cheung
一名患有 2 型神经纤维瘤病的 24 岁女性来诊所评估和讨论其双侧前庭神经鞘瘤 (VSs) 的治疗方案。患者报告双侧渐进性听力损失已有十多年。两年前，右耳几乎失聪。左耳是她听力较好的耳朵。音调型耳鸣涉及双耳。童年早期就出现右面部无力，可能是由于产伤。病人没有提供清晰的叙述。没有引发味觉障碍投诉。耳镜检查值得注意的是，右鼓膜完整，鼓索神经肿瘤相当厚，其横截面直径是预期横截面直径的三倍多（图 1A）。颅神经检查显示 House-Brackmann 4/6 级右侧面瘫。术前头部和脊柱磁共振成像研究显示预期存在多个轴外肿块，包括双侧 VS（图 1，BYD）。左侧 VS 尺寸为 1.6 × 1.4 2 厘米，右侧尺寸为 1.4 × 1.4 2 厘米。沿着小脑半球前外侧的右侧乙状结肠周围 1 厘米肿块与脑膜瘤一致（图 1D）。其他轴外肿块存在于颞叶前部、颈髓交界处、视交叉和小脑池。左侧 C1Y2 外侧也有一个大的分叶状增强病变。在 T1 钆图像上发现了一些意想不到的发现：鼓索和面神经肿瘤。冠状图像显示面神经右侧垂直段（垂直面神经[vFN]；图 1B）增强。轴向图像显示膝状神经节（图 1C）和 vFN（图 1D）增强。鼓索神经瘤增强在 vFN 前面也很明显（图 1D），并且代表与图 1A 中的临床照片相关的成像。对她进行了右侧乙状结肠后开颅手术，几乎完全切除了她的前庭和乙状窦周围脑膜瘤。术前诊断通过肿瘤标本的病理检查进行验证。术后钆磁共振成像显示右侧膝状神经节、颞内面部和鼓索肿瘤增强，与 4 个月前获得的术前图像相比没有变化。患者术后面神经功能仍维持House-Brackmann 4/6级。尽管面神经神经鞘瘤是有据可查的病变，但鼓索神经鞘瘤却相当罕见。英文文献中仅报道了 7 例（1Y4）。大多数病例为孤立性脊索鼓室神经瘤，仅1例Nager于1969年报道的1型神经纤维瘤病患者中存在多发性神经瘤（2）。孤立性鼓索神经瘤的主要症状是听力丧失、耳鸣和面瘫 (2)。有趣的是，在之前的任何病例中都没有记录到味觉障碍（3），这与该患者的情况一致。据我们所知，这是文献中第一张显示鼓索神经瘤的临床照片。患者接受了颅内右侧 VS 的简单切除术。由于她没有症状，因此没有尝试切除所代表的鼓索肿瘤。尽管如此，一些作者还是主张经乳突或经根管切除腱索。地址通信和重印请求请发送给 Kevin C. Huoh, M.D., Department of OtolaryngologyYHead and Neck Surfing, 400 Parnassus Avenue, ACC 7th Floor, San Francisco CA 94143；电子邮件：khuoh@ohns.ucsf.edu 耳科与神经科 31:1172Y1173 2010，耳科与神经科，Inc.
A 24-year-old woman with neurofibromatosis Type 2 presented to clinic for evaluation and discussion of management options for her bilateral vestibular schwannomas (VSs). The patient reported bilateral progressive hearing loss for more than a decade. The right ear became mostly deaf 2 years ago. The left ear was her better hearing ear. Tonal-type tinnitus was referable to both ears. Right facial weakness was present early in childhood, possibly from birth trauma. The patient did not offer a lucid account. No taste disturbance complaint was elicited. Otoscopic examination was notable for an intact right tympanic membrane with a rather thick chorda tympani nerve tumor that was more than thrice the expected cross-sectional diameter (Fig. 1A). Cranial nerve examination revealed right facial paresis at House-Brackmann Grade 4/6. Preoperative head and spine magnetic resonance imaging studies showed expected multiple extra-axial masses, including bilateral VSs (Fig. 1, BYD). The left VS measured 1.6 1.4 2 cm, and the right measured 1.4 1.4 2 cm. A right 1-cm perisigmoid mass along the anterolateral aspect of the cerebellar hemisphere was consistent with meningioma (Fig. 1D). Other extra-axial masses were present at the anterior aspect of the temporal lobe, cervicomedullary junction, optic chiasm, and cisterna magna. There was also a large lobulated enhancing lesion at the lateral aspect of left C1Y2. Some unexpected findings were identified on T1 with gadolinium images: chorda tympani and facial nerve tumors. A coronal image showed enhancement of the right vertical segment of the facial nerve (vertical facial nerve [vFN]; Fig. 1B). Axial images demonstrated enhancement of the geniculate ganglion (Fig. 1C) and vFN (Fig. 1D). Chorda tympani neuroma enhancement was also evident just anterior to the vFN (Fig. 1D) and represented the imaging correlate to the clinical photograph in Figure 1A. A right retrosigmoid craniotomy for near-total excision of her VS and perisigmoid meningioma was performed. The preoperative diagnoses were verified by pathologic review of tumor specimens. A postoperative magnetic resonance imaging with gadolinium demonstrated enhancement of the right geniculate ganglion, and intratemporal facial and chorda tympani tumors, which were unchanged compared with preoperative images that were obtained 4 months ago. The patient’s postoperative facial nerve function remained at House-Brackmann Grade 4/6. Although facial nerve schwannomas are welldocumented lesions, chorda tympani schwannomas are quite rare. There are only 7 cases reported in the English literature (1Y4). Most of the cases were isolated chordatympani neuromas, with only 1 case reporting multiple neuromas in a patient with neurofibromatosis Type 1 reported by Nager in 1969 (2). The presenting symptoms of isolated chorda tympani neuromas were hearing loss, tinnitus, and facial palsy (2). Interestingly, taste disturbance was not documented in any previous case (3), which is in accordance with this patient. To our knowledge, this is the first clinical photograph in the literature showing a chorda tympani neuroma. The patient underwent uncomplicated excision of her intracranial right VS. No attempt was made to resect the represented chorda tympani tumor because she was symptom free. Notwithstanding, some authors have advocated transmastoid or transcanal excision of chorda Address correspondence and reprint requests to Kevin C. Huoh, M.D., Department of OtolaryngologyYHead and Neck Surgery, 400 Parnassus Avenue, ACC 7th Floor, San Francisco CA 94143; E-mail: khuoh@ohns.ucsf.edu Otology & Neurotology 31:1172Y1173 2010, Otology & Neurotology, Inc.