Quaranta A, Zini C, Gandolfi A, Piazza F, De Thomasis G, Frisina A, Mercante G, Quaranta N, Scaringi A, Uccelli M
Clinica Otorinolaringoiatrica I, Dipartimento di Oftalmologia e Otorinolaringoiatria, Università di Bari.
Acta Otorhinolaryngol Ital. 2001 Oct;21(5 Suppl 68):1-20.
The present work provides clinical-functional findings, results and surgical complications observed in a consecutive series of 100 subjects with acoustic neuroma (AN). Analysis of the data has made it possible to draw some important conclusions. Compromised hearing is found in 90% of the ears affected by AN. Indeed the percentage of normal hearing in such cases does not exceed 5%. There is, however, no clear correlation between degree of hearing and tumor size. The symptoms of AN do not always present unilateral or asymmetrical hearing loss, unilateral tinnitus and/or dizziness. At times AN presents atypical symptoms and can even be asymptomatic. Sudden onset of unilateral hearing loss, acute vertigo, persistent monolateral tinnitus and even isolated symptoms of the V or VI cranial nerve should lead one to suspect AN. Only by applying the diagnosis of suspected AN in a large number of cases is it possible to lower the time gap between the onset of symptoms and the definitive diagnosis of AN, increasing the number of cases diagnosed while the AN is still small. Auditory brainstem responses (ABR) are still the means of choice for screening and following up subjects where AN is suspected. Reduced ABR sensitivity reported in the literature for intracanal ANs must induce further testing with magnetic resonance imaging with gadolinium in all subjects where an AN is suspected, even when the ABR is normal. Recording of transient evoked otoacoustic emissions in the presence and in the absence of contralateral white noise has proved to be a simple, inexpensive, non-invasive test for the diagnosis of suspected retrocochlear pathologies. A deficit in vestibular function is most frequently encountered when the AN is already quite large and an alteration in the smooth pursuit test is only found when the AN involves the brainstem. These data have led us to conclude that vestibular reflex studies do not play any role in early diagnosis of AN. Surgical exeresis is the treatment of choice in those cases where "watch and scan" (only hearing ear in the absence of neurological complications; AN < 0.5 cm in the ponto-cerebellar angle, particularly in elderly patients) is not indicated. The enlarged translabyrinthine approach is indicated in all cases of AN, no matter what the tumor size and extent of pre-operative hearing. Promptly and correctly treating intra and postoperative complications, most frequently encountered in patients with AN > 2 cm, reduces the mortality and morbidity to a minimum. Modern otological microsurgery and monitoring techniques make it possible to preserve the VIIth facial nerve in more than 90% of the ears, consequently preserving or nearly preserving normal VIIth nerve function 1 year after surgery in at least three out of four patients. No matter what approach is used, hearing can be preserved measurably in approximately 50% of the ears undergoing surgery and to a socially useful or nearly useful level in a significantly lower proportion of patients. In this regard the most satisfactory results are obtained when preoperative hearing is normal and the AN is < 2 cm.
本研究提供了对连续100例听神经瘤(AN)患者的临床功能检查结果、手术结果及手术并发症的观察情况。对数据的分析使我们能够得出一些重要结论。在受AN影响的耳朵中,90%存在听力受损。实际上,此类病例中听力正常的比例不超过5%。然而,听力程度与肿瘤大小之间并无明确关联。AN的症状并不总是表现为单侧或不对称听力损失、单侧耳鸣和/或头晕。有时AN会呈现非典型症状,甚至可能无症状。单侧听力突然丧失、急性眩晕、持续性单侧耳鸣,甚至孤立的Ⅴ或Ⅵ颅神经症状都应使人怀疑患有AN。只有在大量病例中应用疑似AN的诊断,才有可能缩短症状出现与AN最终确诊之间的时间间隔,增加在AN仍较小时被诊断出的病例数量。听觉脑干反应(ABR)仍是疑似AN患者筛查和随访的首选方法。文献中报道的内耳道AN的ABR敏感性降低,这就必须对所有疑似AN的患者进行钆增强磁共振成像的进一步检查,即使ABR正常时也应如此。在有和没有对侧白噪声的情况下记录瞬态诱发耳声发射,已被证明是一种用于诊断疑似蜗后病变的简单、廉价且非侵入性的测试。当AN已经相当大时,前庭功能障碍最为常见,而只有当AN累及脑干时,平稳跟踪试验才会出现异常。这些数据使我们得出结论,前庭反射研究在AN的早期诊断中不起任何作用。在不适合“观察与扫描”(仅针对无神经并发症的单耳听力;桥小脑角处AN<0.5 cm,特别是老年患者)的情况下,手术切除是首选治疗方法。无论肿瘤大小和术前听力程度如何,所有AN病例均应采用扩大经迷路入路。及时、正确地处理手术中和术后并发症(这些并发症在AN>2 cm的患者中最为常见),可将死亡率和发病率降至最低。现代耳科显微手术和监测技术使90%以上的耳朵能够保留Ⅶ面神经,从而在至少四分之三的患者中,术后1年保留或几乎保留正常的Ⅶ神经功能。无论采用何种入路,在接受手术的耳朵中,约50%的听力可得到一定程度的保留,而能保留到对社交有用或接近有用水平的患者比例则显著更低。在这方面,当术前听力正常且AN<2 cm时,可获得最满意的结果。