Kitamura K, Senba T, Komatsuzaki A
Department of Otolaryngology, University of Tokyo, Japan.
Neurofibromatosis. 1989;2(1):47-52.
The common (and definitive) intracranial tumors in patients with one form of neurofibromatosis (NF), bilateral acoustic NF (NF-2), are bilateral acoustic neuromas. In this study, we present 2 cases with what appears to be another form of NF, namely, NF-1, who showed bilateral enlargement of the internal auditory canals. Bilateral caloric responses were remarkably impaired in both cases; a unilateral sensorineural hearing loss was observed in 1. No tumor was demonstrated in the enlarged internal auditory canals in either case. Although the pathophysiology remains uncertain, some patients with NF-1 show abnormal cochleovestibular function in association with bilateral internal auditory canal enlargement without an acoustic tumor.
患有某种神经纤维瘤病（NF）即双侧听神经型神经纤维瘤病（NF - 2）的患者常见（且典型）的颅内肿瘤是双侧听神经瘤。在本研究中，我们报告了2例似乎是另一种形式的NF即NF - 1的患者，他们表现为双侧内耳道扩大。两例患者的双侧冷热试验反应均明显受损；其中1例观察到单侧感音神经性听力损失。两例患者扩大的内耳道中均未发现肿瘤。尽管病理生理学尚不确定，但一些NF - 1患者表现出与双侧内耳道扩大相关的异常耳蜗前庭功能，且无听神经瘤。
