王正敏

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·院士筆談·

內(nèi)耳疾病診治的不同認識

王正敏

內(nèi)耳深居顳骨巖部，未能被直接觀察到，也甚難取得其病變組織作病理學(xué)檢查，故內(nèi)耳疾病至今仍是未被清楚查明的一類病變。雖然影像學(xué)、功能檢測和生物化學(xué)檢查所得的依據(jù)可用于診斷內(nèi)耳畸形、遺傳、藥物中毒和外傷等病因可查得的內(nèi)耳疾病，但發(fā)病率高的梅尼埃病和良性陣發(fā)性位置性眩暈在著名的《Schuknecht’s耳病理學(xué)》(Merchant SN, Nadol JB Jr, 2010，第3版)一書中仍被列在病因不明或多因性的一章中。其他如影像學(xué)明確可見的上半規(guī)管裂也被質(zhì)疑是否負責(zé)同耳發(fā)生的癥狀。由于這類內(nèi)耳疾病病因不明或可能是多因性的，因此目前對其的臨床診斷和治療仍多是推理和探索性的。

1 梅尼埃病

本病經(jīng)典的病理依據(jù)是在尸解顳骨組織學(xué)表現(xiàn)的膜迷路積水。因其初始病因不知，診斷用名為“原發(fā)性內(nèi)淋巴積水”。破密“原發(fā)性”是最終闡明梅尼埃病病因和機制的任務(wù)。單純皰疹病毒1型(herpes simplex virus 1, HSV-1)常在感染者三叉神經(jīng)節(jié)內(nèi)終身潛伏，并隨時可被激活而引起復(fù)發(fā)性唇皰疹，這一事實令人猜想HSV-1也可能潛伏感染前庭神經(jīng)節(jié)和螺旋神經(jīng)節(jié)，并被隨時激活造成內(nèi)耳損害，例如具有反復(fù)突發(fā)的梅尼埃病。國外多篇文獻報告，梅尼埃病前庭神經(jīng)超微結(jié)構(gòu)顯示神經(jīng)變性，而神經(jīng)變性有可能是炎癥的結(jié)局。已有多篇國外專家報道梅尼埃病與HSV感染有關(guān)。如在梅尼埃病患者血清中檢查到病毒特異性免疫球蛋白E(IgE)；外淋巴中有高水平HSV抗體；在人顳骨尸檢中雖可檢測到HSV潛伏感染，但其中患有梅尼埃病的HSV感染率高于一般群體[1-9]。相關(guān)文獻報告有：環(huán)氧合酶 2(cyclooxygenase 2, COX-2)可在炎性和變性腦病中生成，COX抑制藥物可降低鼠眼HSV-1激活等。HSV-1能誘導(dǎo)體外培養(yǎng)的前庭神經(jīng)元高表達COX-2，而COX抑制劑非甾體抗炎藥能抑制前庭神經(jīng)元培養(yǎng)體系中HSV-1的繁殖和再激活。此項實驗為非甾體抗炎藥臨床治療梅尼埃病的應(yīng)用提供一定的依據(jù)[10-18]。

2 良性陣發(fā)性位置性眩暈

又名嵴帽沉石病和管沉石病。本病的主要臨床表現(xiàn)是發(fā)作于頭部位置改變時的眩暈，是由半規(guī)管壺腹嵴帽內(nèi)有沉石在頭位變化時誘發(fā)。文獻報道在566例1 031只顳骨病理檢驗中，嵴帽內(nèi)有嗜堿性沉積物的可達22%，可見于各半規(guī)管，沉積物大小也不等。對于沉積物是否含鈣物，仍未有結(jié)論。至于嵴帽內(nèi)的沉積物是來自橢圓囊斑或球囊斑的砂膜，還是嵴帽蛋白的變性(嵴帽是支持細胞產(chǎn)生的糖蛋白)還有待進一步判定。對頭位施以加速度變動可消除癥狀的原理被認為是，貼附在嵴帽上從砂膜移位過來的“耳石”得到復(fù)位。另一種解釋是，嵴帽內(nèi)沉積物在加速度運動時，因其慣性而脫出嵴帽。其自然脫落可資解釋本病為何可不治自愈。管沉石病的重要依據(jù)是有專家報告其在術(shù)中后半規(guī)管開放時發(fā)現(xiàn)白色離散顆粒，但尸檢顳骨病理學(xué)研究從未有管內(nèi)“結(jié)石”的報道。迄今為止，尚未在顳骨病理檢查中發(fā)現(xiàn)有橢圓囊斑和球囊斑砂膜脫落的證明[19-25]。

3 上半規(guī)管裂

有文獻報告596例1 000只顳骨組織學(xué)檢查中有上半規(guī)管裂的發(fā)生率可達2%，并認為嬰兒上半規(guī)管在弓狀隆起處會相當(dāng)薄，隨著發(fā)育長大才漸變厚。成人上半規(guī)管變薄或成裂與其管壁發(fā)育受阻有關(guān)。上半規(guī)管在弓狀隆起弓背處管壁厚度若<0.1 mm，在CT上就不易顯示，可能被誤診為管壁全層缺失。上半規(guī)管骨壁完全缺失仍可有完整骨膜。骨膜及覆蓋其上的硬膜和充滿腦脊液的蛛網(wǎng)膜下隙可產(chǎn)生足夠抗力防止骨膜破裂和骨迷路開放?？沽蓽p少或消除外淋巴液流側(cè)向壓力導(dǎo)致的順應(yīng)變動而保護膜迷路。有專家認為，上半規(guī)管裂成為產(chǎn)生臨床癥狀和體征的“第三窗”，應(yīng)有“第二事件”存在。第二事件可能是顱外傷或顱內(nèi)壓升高。所以，診斷上半規(guī)管裂綜合征應(yīng)該綜合分析CT、誘發(fā)癥狀及體征的聲強大小、耳內(nèi)壓變化和既往史等[26-31]。

內(nèi)耳疾病的病因、發(fā)病機制和轉(zhuǎn)歸及診治至今仍是富有挑戰(zhàn)性和亟待揭秘。隨著相關(guān)基礎(chǔ)研究的不斷發(fā)現(xiàn)和臨診科學(xué)技術(shù)水平的提高，內(nèi)耳疾病的診療會踏上新的臺階。

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(本文編輯 楊美琴)

復(fù)旦大學(xué)附屬眼耳鼻喉科醫(yī)院耳鼻喉科 衛(wèi)生部聽覺醫(yī)學(xué)重點實驗室 上海 200031

王正敏(Email：fjswzm2015@126.com)

10.14166/j.issn.1671-2420.2015.01.001

2014-12-22)