Lalwani A K, Abaza M M, Makariou E V, Armstrong M
Department of Otolaryngology-Head & Neck Surgery, University of California San Francisco, 94143-0342, USA.
Am J Otol. 1998 May;19(3):352-7.
The audiologic presentation of vestibular schwannoma (VS) associated with neurofibromatosis type 2 (NF2) has not been well characterized. The goal of this study was to investigate the audiologic features of NF2-associated VS and to determine their relationship to the size of the tumor.
A retrospective case review.
Quaternary governmental medical research institute evaluating patients fitting specific criteria for ongoing clinical studies.
Audiologic and magnetic resonance imaging data were available for 40 patients (25 males, 15 females), with an average age of 32 years, who had been recruited for ongoing clinical and genetic studies of NF2.
The audiologic profile and magnetic resonance imaging characteristic of tumor were retrospectively reviewed.
The average size of the tumor at presentation was 7.26 +/- 16.58 cm3 and measured 1.2, 1.6, and 1.1 cm in the anterior/posterior, lateral/medial, and superior/inferior dimensions, respectively. An increase in lateral/medial size of the tumor most significantly correlated with deterioration in mid- (1,000-2,000 Hz) and high- (4,000-8,000 Hz) frequency hearing levels, elevated speech reception threshold, and prolonged auditory brain stem response waves III and V latency.
Patients with NF2 demonstrate a more predictable audiologic profile for a given size tumor than has been previously described with spontaneous or sporadic VS.
与2型神经纤维瘤病（NF2）相关的前庭神经鞘瘤（VS）的听力学表现尚未得到充分描述。本研究的目的是调查NF2相关VS的听力学特征，并确定它们与肿瘤大小的关系。
回顾性病例分析。
四级政府医学研究所，对符合特定标准的患者进行正在进行的临床研究评估。
40例患者（25例男性，15例女性）有听力学和磁共振成像数据，平均年龄32岁，这些患者已被纳入NF2正在进行的临床和基因研究。
回顾性分析肿瘤的听力学特征和磁共振成像特征。
肿瘤出现时的平均大小为7.26±16.58 cm³，前后径、左右径和上下径分别为1.2 cm、1.6 cm和1.1 cm。肿瘤左右径的增加与中高频（1000 - 2000 Hz和4000 - 8000 Hz）听力水平下降、言语接受阈升高以及听觉脑干反应波III和V潜伏期延长最显著相关。
对于给定大小的肿瘤，与之前描述的散发或散发性VS相比，NF2患者表现出更可预测的听力学特征。
