Mechanisms of morbid hearing loss associated with tumors of the endolymphatic sac in von Hippel-Lindau disease
Mechanisms of morbid hearing loss associated with tumors of the endolymphatic sac in von Hippel-Lindau disease
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DOI：
10.1001/jama.298.1.41
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发表时间：
2007-07-04
影响因子：
120.7
通讯作者：
Lonser, Russell R.
中科院分区：
文献类型：
--
作者：
Butman, John A.;Kim, H. Jeffrey;Lonser, Russell R.
内淋巴囊肿瘤（ELST）与von Hippel-Lindau病有关，可引起不可逆的感音神经性听力损失（SNHL）和前庭病变。听前庭发病的潜在机制仍不清楚，治疗的最佳时机尚不清楚。目的明确与ELSTs相关的听前庭病理生理学机制。设计，设置，对von Hippel患者的前瞻性和系列评价-1990年5月至2006年12月期间，美国国立卫生研究院对林道病和ELST进行了研究。主要结果测量临床发现和听力学结果35例vonHippel-Lindau病合并ELST患者38耳（3例双侧ELST）。肿瘤侵犯耳囊与较大肿瘤相关（P = 0.01），发生在7耳（18%）中，导致SNHL（100%）。其余31耳（82%）无耳囊侵犯的证据。31耳中有27耳（87%）突然（14耳; 52%）或逐渐（13耳; 48%）发生SNHL，4耳听力正常。14耳突发性SNHL中有11耳出现脑干内出血（79%; P <0.001），而17耳渐进性SNHL或听力正常的患者中无一例出现脑干内出血。肿瘤大小与SNHL（P = .23）或前庭病变（P = .83）无关。结论ELST相关SNHL和前庭病变可能是由于肿瘤相关的淋巴结内出血突然发生，也可能是与内淋巴积水一致的隐匿性发生。这两种病理生理机制都发生在与耳囊浸润无关的小肿瘤中。
Context Endolymphatic sac tumors (ELSTs) are associated with von Hippel-Lindau disease and cause irreversible sensorineural hearing loss (SNHL) and vestibulopathy. The underlying mechanisms of audiovestibular morbidity remain unclear and optimal timing of treatment is not known.Objective To define the mechanisms underlying audiovestibular pathophysiology associated with ELSTs.Design, Setting, and Patients Prospective and serial evaluation of patients with von Hippel-Lindau disease and ELSTs at the National Institutes of Health between May 1990 and December 2006.Main Outcome Measures Clinical findings and audiologic data were correlated with serial magnetic resonance imaging and computed tomography imaging studies to determine mechanisms underlying audiovestibular dysfunction.Results Thirty-five patients with von Hippel-Lindau disease and ELSTs in 38 ears (3 bilateral ELSTs) were identified. Tumor invasion of the otic capsule was associated with larger tumors (P = .01) and occurred in 7 ears (18%) causing SNHL (100%). No evidence of otic capsule invasion was present in the remaining 31 ears (82%). SNHL developed in 27 of these 31 ears (87%) either suddenly (14 ears; 52%) or gradually (13 ears; 48%) and 4 ears had normal hearing. Intralabyrinthine hemorrhage was found in 11 of 14 ears with sudden SNHL (79%; P < .001) but occurred in none of the 17 ears with gradual SNHL or normal hearing. Tumor size was not related to SNHL (P = .23) or vestibulopathy (P = .83).Conclusions ELST-associated SNHL and vestibulopathy may occur suddenly due to tumor-associated intralabyrinthine hemorrhage, or insidiously, consistent with endolymphatic hydrops. Both of these pathophysiologic mechanisms occur with small tumors that are not associated with otic capsule invasion.