陳彪 魏興梅 石穎 李永新

1首都醫(yī)科大學(xué)附屬北京同仁醫(yī)院耳鼻咽喉頭頸外科，

耳鼻咽喉頭頸科學(xué)教育部重點(diǎn)實(shí)驗(yàn)室（首都醫(yī)科大學(xué)）（北京100730）

面神經(jīng)畸形人工耳蝸植入圍手術(shù)期處理

陳彪1魏興梅1石穎1李永新1

1首都醫(yī)科大學(xué)附屬北京同仁醫(yī)院耳鼻咽喉頭頸外科，

耳鼻咽喉頭頸科學(xué)教育部重點(diǎn)實(shí)驗(yàn)室（首都醫(yī)科大學(xué)）（北京100730）

面神經(jīng)是耳科手術(shù)需重點(diǎn)保護(hù)的結(jié)構(gòu)之一，而面神經(jīng)發(fā)育畸形會(huì)顯著增加面神經(jīng)損傷的風(fēng)險(xiǎn)。人工耳蝸植入（cochlear implantation,CI）是治療重度感音神經(jīng)聾患者的重要手段之一，面神經(jīng)畸形(Facial nerve aberration,FNA)常影響其手術(shù)徑路的選擇，增加手術(shù)風(fēng)險(xiǎn)。本文就面神經(jīng)發(fā)育、FNA的分類、以及FNA患者行CI手術(shù)的圍手術(shù)期處理予以綜述，為臨床提供參考。

面神經(jīng)畸形;人工耳蝸植入;面神經(jīng)損傷

面神經(jīng)（facial nerve）是第VII對(duì)顱神經(jīng)，為人體內(nèi)穿過骨管最長(zhǎng)的腦神經(jīng)，走行復(fù)雜，在顳骨內(nèi)部分主要分為內(nèi)耳道段、迷路段、鼓室段和乳突段，術(shù)中對(duì)于面神經(jīng)的保護(hù)是耳顯微外科的重難點(diǎn)之一。面神經(jīng)損傷是人工耳蝸植入（cochlear implantation,CI）的嚴(yán)重并發(fā)癥之一，據(jù)文獻(xiàn)報(bào)道在CI術(shù)中面神經(jīng)損傷的發(fā)病率為0.4%—1.7%[1,2]。盡管近些年來隨著手術(shù)經(jīng)驗(yàn)的積累和科學(xué)技術(shù)水平的提高（如影像學(xué)的進(jìn)步以及術(shù)中面神經(jīng)探測(cè)儀的使用等）使得面神經(jīng)的損傷概率大大減低，但對(duì)于CI手術(shù)中面神經(jīng)的保護(hù)仍是研究熱點(diǎn)。

面神經(jīng)畸形（facial nerve aberrations，F(xiàn)NA）是導(dǎo)致術(shù)中面神經(jīng)損傷的重要因素，尤其是當(dāng)面神經(jīng)畸形合并中耳及內(nèi)耳畸形時(shí)，更增加了人工耳蝸的手術(shù)難度及術(shù)后面癱的風(fēng)險(xiǎn)。面神經(jīng)畸形分為面神經(jīng)管缺失、面神經(jīng)走行異常以及面神經(jīng)分叉畸形。據(jù)報(bào)道，面神經(jīng)畸形在所有CI患者中比例達(dá)5%，對(duì)于內(nèi)耳畸形CI患者而言面神經(jīng)畸形出現(xiàn)幾率則高達(dá)16%[3]。此外還有一些先天性的綜合征病變會(huì)出現(xiàn)面神經(jīng)異常，如心面綜合征、Moebius氏綜合征等。面神經(jīng)畸形不可避免地增加了CI的難度。因此本文將從面神經(jīng)的發(fā)育、面神經(jīng)畸形的形成、人工耳蝸植入與面神經(jīng)損傷，及預(yù)防與保護(hù)等方面進(jìn)行總結(jié)。

1 面神經(jīng)的發(fā)育

面神經(jīng)起源于面聽原基。在胚胎第四周，面神經(jīng)開始向位于第二腮弓背部的膝狀神經(jīng)節(jié)原始細(xì)胞延伸。胚胎第八周時(shí)，面神經(jīng)在顳骨內(nèi)的原始定位基本形成，但其最終位置與面神經(jīng)骨管的形成和面神經(jīng)周圍結(jié)構(gòu)的發(fā)育有關(guān)。面神經(jīng)骨管源于第二腮弓和耳囊，在妊娠第五月時(shí)開始骨化包繞面神經(jīng)，但是在妊娠期間部分面神經(jīng)管始終以軟骨形式存在，并會(huì)持續(xù)到出生后一年[4]。

2 面神經(jīng)畸形

2.1 面神經(jīng)骨管缺失

面神經(jīng)骨管缺失可以發(fā)生在面神經(jīng)管的任意位置，也可以是單個(gè)或多個(gè)位置[5]。1971年Baxter發(fā)現(xiàn)在正常顳骨中有55%存在面神經(jīng)管缺失，其中91%發(fā)生在面神經(jīng)水平段[6]。國(guó)內(nèi)外諸多文獻(xiàn)對(duì)于面神經(jīng)管缺失的發(fā)病率報(bào)道都是相似的高比例(55%-74%)，因此也有學(xué)者認(rèn)為面神經(jīng)管畸形應(yīng)屬于正常解剖變異，但是從其可能引起后果來看將其歸為面神經(jīng)畸形也許更合理[6,7]。尤其當(dāng)較大范圍的面神經(jīng)骨管缺失時(shí)，面神經(jīng)將會(huì)脫垂，對(duì)于中耳及內(nèi)耳手術(shù)有較大影響[8,9]。對(duì)于人工耳蝸植入而言，當(dāng)面神經(jīng)管缺失時(shí)難以將正常面神經(jīng)同其他軟組織分辨（如炎癥形成的肉芽）、同時(shí)缺少骨骼的遮擋也會(huì)增加電鉆熱傳導(dǎo)，這都會(huì)增加面神經(jīng)的損傷幾率。

2.2 面神經(jīng)走行異常

面神經(jīng)在顳骨內(nèi)的最終位置與面神經(jīng)骨管的形成和面神經(jīng)周圍結(jié)構(gòu)（包括鐙骨、迷路、顳骨乳突部、顳骨鼓部等）的發(fā)育有關(guān)[4]。面神經(jīng)走行異常發(fā)生在迷路段、水平段和垂直段，這種情況常伴隨著外、中、內(nèi)耳畸形。Hoffman等人發(fā)現(xiàn)內(nèi)耳畸形患者中有16%出現(xiàn)面神經(jīng)走行異常[10]。雖然內(nèi)耳畸形種類難以同面神經(jīng)畸形逐一對(duì)應(yīng)，但是可以確定是Modini畸形與面神經(jīng)迷路段內(nèi)移無對(duì)應(yīng)關(guān)系[11,12]。據(jù)文獻(xiàn)報(bào)道，面神經(jīng)水平走行異常與前庭窗閉鎖有關(guān)，面神經(jīng)垂直段前移常與外耳道閉鎖有關(guān)[8,13,14]。CI技術(shù)最常用的徑路是面隱窩徑路，而這兩種面神經(jīng)走行異常會(huì)影響人工耳蝸的圓窗暴露與電極植入，增加面神經(jīng)損傷的幾率。

2.3 面神經(jīng)分叉

面神經(jīng)分叉畸形發(fā)生于胚胎4-6周，具體原因未知，可能與顳骨的骨質(zhì)結(jié)構(gòu)的牽拉有關(guān)[4,15-17]。面神經(jīng)分叉畸形最常發(fā)生于面神經(jīng)水平段，可以分為兩支或者多支，也可以發(fā)生在面神經(jīng)管的任何一段[17]。當(dāng)面神經(jīng)分叉畸形發(fā)生于水平段或垂直段時(shí)，對(duì)于中耳及內(nèi)耳手術(shù)的影響最大。面神經(jīng)分叉畸形常伴隨中耳、內(nèi)耳畸形，正常的解剖標(biāo)志發(fā)生改變，從而增加面神經(jīng)損傷的風(fēng)險(xiǎn)。對(duì)于經(jīng)典的圓窗入路CI而言，這種情況的發(fā)生可能會(huì)導(dǎo)致面隱窩開放和圓窗暴露困難，增加面神經(jīng)損傷的風(fēng)險(xiǎn)[16,19]。

3 人工耳蝸植入與面神經(jīng)損傷

隨著手術(shù)技術(shù)進(jìn)步及科技發(fā)展，CI導(dǎo)致的面神經(jīng)損傷發(fā)病率已大幅度降低。1988年Cohen報(bào)告的發(fā)病率為1.74%[20]，2004年Bhatia等人報(bào)告遲發(fā)性面癱的發(fā)病率為0.7%[21]。伴面神經(jīng)畸形的CI手術(shù)更易損傷面神經(jīng)。由于面神經(jīng)功能擁有支配人面部表情肌的特殊功能，面神經(jīng)的保護(hù)仍然是CI的重點(diǎn)。面神經(jīng)的損傷根據(jù)其損傷結(jié)果可以分為永久性面神經(jīng)麻痹、暫時(shí)性面神經(jīng)麻痹、面神經(jīng)刺激，根據(jù)其發(fā)生時(shí)間可以分為即時(shí)性面神經(jīng)麻痹、遲發(fā)性面神經(jīng)麻痹。即時(shí)性面神經(jīng)麻痹發(fā)病率約為0.1%，預(yù)后一般較差；遲發(fā)性面神經(jīng)麻痹發(fā)病率約1%，預(yù)后較好，一般數(shù)月內(nèi)可以得到恢復(fù)[22-24]。即時(shí)性面神經(jīng)麻痹和早期遲發(fā)性面神經(jīng)麻痹的發(fā)生常與手術(shù)有關(guān)，術(shù)中的機(jī)械性損傷和熱損傷可以引起面神經(jīng)缺血、水腫和炎癥發(fā)生[22-24]。晚期遲發(fā)性面癱具體機(jī)制不明，目前提出的機(jī)制有熱損傷、潛伏病毒的激活（單純皰疹病毒、水痘-帶狀皰疹病毒）、血液分解產(chǎn)物的局部效應(yīng)及傷口的感染等[20,23-25]。面神經(jīng)刺激是指患者在正常使用人工耳蝸時(shí),面神經(jīng)受到電流刺激導(dǎo)致面肌抽搐的現(xiàn)象。據(jù)報(bào)道，其發(fā)病率從2%到14.6%不等[22-24]。面神經(jīng)刺激的原因包括電極插入時(shí)骨質(zhì)的損傷、組織阻抗的變化、從鼓階到面神經(jīng)的異常釋放的電流等[26]。

4 面神經(jīng)損傷的預(yù)防

4.1 術(shù)前影像評(píng)估：

目前高分辨CT層厚越來越薄，能夠更加清晰的顯示顳骨內(nèi)精細(xì)結(jié)構(gòu)，可以通過術(shù)前對(duì)于面神經(jīng)骨管的分析預(yù)測(cè)面神經(jīng)的走行及病變情況[27,28]。而顳骨CT平面重建技術(shù)的應(yīng)用可以更加準(zhǔn)確地提供面神經(jīng)每一段的形態(tài)特點(diǎn)[27]。內(nèi)耳MRI的T2加權(quán)像的薄層重建，可以呈現(xiàn)腦池和顱神經(jīng)的微細(xì)變化[29]。在內(nèi)聽道層面的內(nèi)耳MRI,可以顯現(xiàn)出面神經(jīng)是否缺失及其大小。雙側(cè)內(nèi)聽道的MRI重建對(duì)于診斷面神經(jīng)異常導(dǎo)致的面癱有顯著鑒別作用[30]。

4.2 面神經(jīng)監(jiān)護(hù)儀及術(shù)中影像導(dǎo)航技術(shù)的使用

術(shù)中使用面神經(jīng)監(jiān)護(hù)儀，可以對(duì)可疑面神經(jīng)結(jié)構(gòu)進(jìn)行探查。當(dāng)面神經(jīng)檢測(cè)儀置于面神經(jīng)或者面神經(jīng)骨管表面時(shí)將會(huì)給予警示。術(shù)中影像導(dǎo)航技術(shù)飛速發(fā)展，在諸多領(lǐng)域進(jìn)行應(yīng)用。2011年Stelter報(bào)道一例面神經(jīng)減壓術(shù)后的人工耳蝸植入患者應(yīng)用術(shù)中影像導(dǎo)航技術(shù)，對(duì)面神經(jīng)進(jìn)行標(biāo)記，成功避免面神經(jīng)損傷[35]。國(guó)內(nèi)也有報(bào)道影像導(dǎo)航技術(shù)應(yīng)用于面神經(jīng)畸形人工耳蝸植入患者，術(shù)后未出現(xiàn)并發(fā)癥[36]。

4.3 多種手術(shù)路徑選擇

人工耳蝸植入最常用的植入徑路是通過面隱窩經(jīng)圓窗植入電極。但是面神經(jīng)畸形的存在可能會(huì)影響面隱窩的開放及圓窗的暴露。Hoffman[10]教授報(bào)道8名存在面神經(jīng)畸形的人工耳蝸植入患者中，有一人遭受嚴(yán)重的面神經(jīng)損傷。對(duì)于這些患者，使用經(jīng)典的乳突面隱窩入路植入CI,會(huì)增加醫(yī)源性面神經(jīng)損傷的風(fēng)險(xiǎn)。這就需要術(shù)者在術(shù)前進(jìn)行規(guī)劃手術(shù)路徑，通過結(jié)合耳道入路、前庭窗植入及骨岬鉆孔植入，必要時(shí)結(jié)合內(nèi)鏡手術(shù)完成人工耳蝸植入，減少面神經(jīng)的損傷發(fā)生的幾率。2012年JAE-JIN[37]等人報(bào)道7例面神經(jīng)畸形人工耳蝸植入，其中兩例患者采用耳道徑路耳蝸造孔，然后經(jīng)過面隱窩成功植入電極。2001年Kronenberg[41]等人報(bào)道的外耳道上徑路，因避免面隱窩的開放，而減少了面神經(jīng)損傷機(jī)會(huì)。1998年Colletti[42]等人報(bào)道的顱中窩徑路，由于不再行乳突切除，而減少面神經(jīng)損傷的可能性。

4.4 術(shù)中精細(xì)的操作

當(dāng)面神經(jīng)畸形伴隨外、中、內(nèi)耳畸形時(shí)，手術(shù)醫(yī)生豐富的手術(shù)經(jīng)驗(yàn)、異常熟悉的顳骨解剖和精細(xì)的操作尤其重要，這些因素的存在可以大大減少面神經(jīng)損傷的幾率。其次術(shù)中充分的沖洗，也可以減少熱損傷的產(chǎn)生。

5 面神經(jīng)損傷后的評(píng)估

人工耳蝸術(shù)后出現(xiàn)面神經(jīng)麻痹時(shí)，首先應(yīng)對(duì)患者的一般生命體征進(jìn)行評(píng)估，其次評(píng)估面癱的程度，最常使用的是Brackmann于1985年發(fā)表的HB分級(jí)[31]以及面肌電圖檢查。據(jù)報(bào)道，遲發(fā)性面神經(jīng)麻痹患者血清中可發(fā)現(xiàn)HSV-1、HSV-2、VZV的Ig G、Ig E大量增加，因此有學(xué)者認(rèn)為術(shù)后遲發(fā)型面癱與病毒有關(guān)，當(dāng)出現(xiàn)延遲性面神經(jīng)麻痹時(shí)可以進(jìn)行免疫學(xué)檢查[32]。

6 面神經(jīng)損傷的處理

6.1 面癱患者：

（1）手術(shù)治療：手術(shù)方式包括面神經(jīng)減壓術(shù)、面神經(jīng)神經(jīng)吻合術(shù)或神經(jīng)移植術(shù)等[18]。Napoli[38]等人認(rèn)為傷后出現(xiàn)即時(shí)性面神經(jīng)損傷是外科的手術(shù)指征；Guntinas[39]等人認(rèn)為面神經(jīng)損傷的手術(shù)指征為：傷后即刻出現(xiàn)完全性面癱并伴有異常肌電圖。Fisch[40]認(rèn)為通過面神經(jīng)電圖判斷面神經(jīng)變性程度，并以神經(jīng)電圖減少大于90%作為手術(shù)標(biāo)準(zhǔn)。

（2）藥物治療：早期使用類固醇激素，對(duì)于面神經(jīng)損傷的治療效果是明確的，但是使用前應(yīng)當(dāng)排除禁忌[33]。對(duì)于病毒導(dǎo)致的面神經(jīng)麻痹應(yīng)當(dāng)選擇抗病毒藥物治療，但是不必預(yù)防性的使用。對(duì)于類固醇激素及抗病毒藥物的聯(lián)合使用存在爭(zhēng)議[33,34]。

6.2 面神經(jīng)刺激：

絕大多數(shù)病例通過調(diào)整刺激電流的參數(shù)或關(guān)閉相關(guān)電極的方法得到解決。對(duì)于面刺激癥狀嚴(yán)重,通過調(diào)機(jī)方法無法解決的病例,可考慮再次手術(shù),更換不同類型的電極。

總之，面神經(jīng)畸形患者行人工耳蝸植入是人工耳蝸植入的難點(diǎn)之一。對(duì)于面神經(jīng)的辨認(rèn)與保護(hù)，需要術(shù)者術(shù)前采取多手段的仔細(xì)評(píng)估、術(shù)中的謹(jǐn)慎操作及多種儀器的探查。而此類手術(shù)的開展，更需要術(shù)者長(zhǎng)期的經(jīng)驗(yàn)積累與豐富的解剖知識(shí)。術(shù)后應(yīng)仔細(xì)評(píng)估患者術(shù)后狀態(tài)，盡早發(fā)現(xiàn)可能存在的面神經(jīng)損傷，全面評(píng)估，積極治療。

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Perioperative Management of Cochlear Implantation in Patients with Facial NerveAberrations

Chen Biao1,Wei Xingmei1,Shi Ying1,Li Yongxin1
1 Department of Otolaryngology Head and Neck Surgery,Beijing Tongren Hospital,Capital Medical University,Key
Laboratory of Otolaryngology Head and Neck Surgery,Ministry of Education,Beijing,100730,China
Corresponding author：Li YongxinEmail：entlyx@sina.com

Facial nerve is one of the most essential anatomic structures which needs to be protected during otology surgeries,and facial nerve aberrations(FNA)may significantly increase the risk of facial nerve injury.Cochlear implantation(CI)is one of the most common treatment for patients with severe to profound sensorineural hearing loss.Facial nerve may impact the choice of surgical approach when performing CI.In this article,we will review embryonic development of the facial never,the classification of facial nerve aberrations and perioperative management of cochlear implantation in patients with facial nerve aberrations to facilitate clinical practice.

Facial NerveAberrations;Cochlear Implant;Facial Nerve Injury Project supported by The Natural Science Foundation of China.Numbers:81371088 and 81670923 The authors disclose no conflicts of interest.

R764

A

1672-2922（2017）05-571-4

10.3969/j.issn.1672-2922.2017.014.

陳彪，在讀碩士研究生，研究方向:耳科學(xué)

李永新，Email:entlyx@sina.com

2017-04-08審核人：馬芙蓉）