Office of the Clinical Director and Otolaryngology Branch, National Institute on Deafness and Other Communication Disorders, Georgetown University Medical Center, Washington, DC, USA.
Laryngoscope. 2013 Feb;123(2):477-83. doi: 10.1002/lary.23646. Epub 2012 Oct 15.
OBJECTIVES/HYPOTHESIS: To define the surgical treatment and outcomes of von Hippel-Lindau (VHL) disease-associated endolymphatic sac tumors (ELSTs), we analyzed consecutive VHL patients who underwent ELST resection.
Retrospective investigation of consecutive VHL patients who underwent resection of ELSTs at a clinical research center between 1999 and 2010.
Analysis of serial clinical examinations, audiograms, imaging studies, and operative findings were analyzed.
Thirty-one consecutive patients with ELSTs (15 males, 16 females) underwent resection of 33 tumors (mean follow-up, 49.9 ± 48.0 months; range, 1.0-116 months). One patient had bilateral ELST resections and one patient underwent reoperation for recurrence. Mean age at surgery was 38.2 ± 10.2 years (range, 12-67 years). Whereas 29 ears (88%) had direct radiographic evidence of an ELST, four ears (12%) did not. Mean tumor size was 1.3 ± 1.1 cm (range, 0.2-5.2 cm). Whereas two patients (two ears, 6%) were asymptomatic, 29 patients (31 ears, 94% of ears) had associated audiovestibular symptoms, including sensorineural hearing loss (28 ears, 84%), tinnitus (24 ears,73%), and vertigo (21 patients, 68%). Postoperatively, hearing was stabilized (27) or improved (three) in 97% of 31 ears. Complete tumor resection was achieved in 30 ears (91% of 33 ears). Complications included cerebrospinal fluid leak in two ears (6%) and transient lower cranial nerve palsy in one ear (3%).
Surgical resection of ELSTs can be performed with hearing preservation and a reduction in audiovestibular dysfunction. Early surgical resection can prevent or decrease disabling audiovestibular symptoms, enhance the opportunity for complete resection, and preserve hearing.
目的/假设：为了定义与 von Hippel-Lindau(VHL)病相关的内淋巴囊肿瘤(ELST)的手术治疗和结果，我们分析了在临床研究中心连续接受 ELST 切除术的 VHL 患者。
对 1999 年至 2010 年间在临床研究中心接受 ELST 切除术的连续 VHL 患者进行回顾性调查。
分析了连续的临床检查、听力图、影像学研究和手术结果。
31 例连续的 ELST 患者(15 例男性，16 例女性)接受了 33 个肿瘤切除术(平均随访 49.9±48.0 个月;范围，1.0-116 个月)。1 例患者行双侧 ELST 切除术，1 例患者因复发而行再次手术。手术时的平均年龄为 38.2±10.2 岁(范围，12-67 岁)。29 耳(88%)有直接放射影像学证据的 ELST，4 耳(12%)没有。肿瘤平均大小为 1.3±1.1cm(范围，0.2-5.2cm)。2 例患者(2 耳，6%)无症状，29 例患者(31 耳，94%的耳)有相关的听觉-前庭症状，包括感音神经性听力损失(28 耳，84%)、耳鸣(24 耳，73%)和眩晕(21 例，68%)。术后，31 耳中有 27 耳(97%)听力稳定，3 耳(10%)听力改善。33 个肿瘤中有 30 个(91%)实现了完全切除。并发症包括 2 耳(6%)的脑脊液漏和 1 耳(3%)的短暂颅神经麻痹。
ELST 的手术切除可以在保留听力和减少听觉-前庭功能障碍的情况下进行。早期手术切除可以预防或减少致残性听觉-前庭症状，增加完全切除的机会，保留听力。
