Jagannathan Jay, Lonser Russell R, Stanger Richard A, Butman John A, Vortmeyer Alexander O, Zalewski Christopher K, Brewer Carmen, Surowicz Christal, Kim H Jeffrey
Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1414, USA.
Otol Neurotol. 2007 Oct;28(7):927-30. doi: 10.1097/MAO.0b013e31805c7506.
Bilateral endolymphatic sac tumors (ELSTs) are associated with von Hippel-Lindau disease and often underlie significant audiovestibular morbidity, including hearing loss.
This 44-year-old female von Hippel-Lindau disease patient presented with tinnitus, vertigo, and binaural hearing loss. Magnetic resonance and computed tomography imaging demonstrated bilateral ELSTs, and audiometry confirmed bilateral hearing loss.
The patient underwent staged resection of the ELSTs (left then right). After resection of the left ELST and during the same operation, a cochlear implant was placed.
Clinical, audiometric, and imaging data.
Postoperatively, the patient had resolution of tinnitus and vertigo with a significant implant-aided improvement in hearing.
Because of their unique anatomic and biologic features, resection of bilateral tumors and cochlear implantation in deaf ELST patients is a potential option to improve hearing and quality of life.
双侧内淋巴囊肿瘤（ELSTs）与冯·希佩尔－林道病相关，常导致严重的听觉前庭功能障碍，包括听力损失。
这位44岁的冯·希佩尔－林道病女性患者出现耳鸣、眩晕和双耳听力损失。磁共振成像和计算机断层扫描显示双侧ELSTs，听力测定证实双侧听力损失。
患者接受了分期切除ELSTs（先左后右）。在切除左侧ELST期间并在同一手术中，植入了人工耳蜗。
临床、听力测定和影像学数据。
术后，患者耳鸣和眩晕症状消失，人工耳蜗辅助听力有显著改善。
由于其独特的解剖和生物学特征，对于失聪的ELST患者，切除双侧肿瘤并植入人工耳蜗是改善听力和生活质量的一种潜在选择。
