Shulman Abraham, Goldstein Barbara
Health Science Center at Brooklyn, State University of New York, Downstate Medical Center, 450 Clarkson Avenue, Box 1239, Brooklyn, NY 11203, USA.
Int Tinnitus J. 2006;12(1):75-81.
Secondary endolymphatic hydrops (SEH) has clinically been found to have a significant incidence of occurrence in patients with subjective idiopathic tinnitus (SIT) of a severe disabling type. The diagnosis is made clinically and has been established by integration in a medical audiological tinnitus patient protocol of the clinical history with results of electrodiagnostic cochleovestibular testing that fulfill the diagnostic criteria of inner-ear disease consistent with Ménière's disease. SEH is hypothesized to be a factor, not an etiology, influencing the clinical course of SIT. Alterations over time (i.e., delay in the homeostatic mechanisms in normnal function of the fluid compartments of the inner-ear perilymph, endolymph, or brain cerebrospinal fluid) result in endolymphatic hydrops and interference in normal function of the inner ear, with resultant inner-ear complaints that can be highlighted by tinnitus rather than by vertigo. The endolymphatic hydrops may be either localized or diffuse within the cochlear or vestibular labyrinth. The etiologies and mechanisms of cochleovestibular-type tinnitus are multiple and are influenced by the SEH. Classically, the tetrad of symptoms--episodic vertigo, fluctuating sensorineural hearing loss, tinnitus, and ear blockage--associated with the histopathological correlate endolymphatic hydrops has been diagnosed as Ménière's disease. Specifically, key etiological agents that have been identified as playing a role in the clinical course of tinnitus (e.g., noise exposure, stress) may serve as "triggers" or stressors (or both), resulting in interference in normal biochemical and physiological function of sensorineural structures in the inner ear or in neural structures in the brain. In both conditions, the alterations over time (i.e., delay) in the clinical manifestation of the tetrad of symptoms of inner-ear dysfunction, when highlighted by SIT rather than vertigo, otherwise fulfill the criteria for diagnosing SEH. The chief complaint of SIT, when presenting as one of the tetrad of inner-ear symptoms and otherwise diagnosed as Ménière's disease, has also been associated clinically with perfusion asymmetries in brain, identified by nuclear medicine brain imaging (single-photon emission computed tomography [SPECT] of brain), and reflects an interference in homeostasis in the blood-brain labyrinth or blood-brain barriers, with a resulting SEH. The medical significance of the SIT in some patients may be a gradual, progressive sensorineural hearing loss. The inclusion of SPECT of brain in SIT patients demonstrates a global approach for improving the accuracy of diagnosing the SIT symptom, for focusing on the contribution of central nervous system dysfunction to the development of SEH, and for understanding and influencing the clinical course of SIT.
临床上已发现,继发性内淋巴积水(SEH)在重度致残型主观性特发性耳鸣(SIT)患者中的发生率颇高。该诊断基于临床判断,并通过将临床病史与电诊断性耳蜗前庭测试结果整合到医学听力学耳鸣患者方案中得以确立,这些测试结果符合与梅尼埃病相符的内耳疾病诊断标准。据推测,SEH是影响SIT临床病程的一个因素,而非病因。随着时间推移发生的改变(即内耳外淋巴、内淋巴或脑脑脊液液腔正常功能的稳态机制延迟)会导致内淋巴积水,并干扰内耳正常功能,进而引发内耳不适,耳鸣可能比眩晕更突出。内淋巴积水可能局限于耳蜗或前庭迷路内,也可能呈弥漫性。耳蜗前庭型耳鸣的病因和机制多种多样,且受SEH影响。传统上,与组织病理学相关的内淋巴积水相关的症状四联征(发作性眩晕、波动性感音神经性听力损失、耳鸣和耳堵塞)被诊断为梅尼埃病。具体而言,已确定在耳鸣临床病程中起作用的关键病因(如噪声暴露、压力)可能作为“触发因素”或应激源(或两者皆是),导致内耳感觉神经结构或大脑神经结构的正常生化和生理功能受到干扰。在这两种情况下,当以SIT而非眩晕突出显示时,内耳功能障碍症状四联征临床表现随时间的改变(即延迟),否则符合SEH的诊断标准。当SIT作为内耳症状四联征之一出现且被诊断为梅尼埃病时,其主要诉求在临床上也与通过核医学脑成像(脑单光子发射计算机断层扫描[SPECT])确定的脑灌注不对称有关,这反映了血脑迷路或血脑屏障稳态的干扰,进而导致SEH。部分患者中SIT的医学意义可能是逐渐进展的感音神经性听力损失。对SIT患者进行脑SPECT检查体现了一种全面的方法,有助于提高SIT症状诊断的准确性,关注中枢神经系统功能障碍对SEH发展的影响,并理解和影响SIT的临床病程。