劉陽(yáng)

海軍總醫(yī)院耳鼻咽喉頭頸外科（北京100048）

鼓膜完整的傳導(dǎo)性耳聾是一組具有共同臨床特征的疾病，本文以病理和胚胎發(fā)生學(xué)為出發(fā)點(diǎn)，針對(duì)臨床影像診斷和外科治療策略進(jìn)行梳理總結(jié)。主要疾病譜包括：耳硬化癥、鼓室硬化、外傷性聽(tīng)骨鏈中斷、先天性膽脂瘤、先天性聽(tīng)骨鏈畸形。其共同臨床特征是：外耳道正常、鼓膜完整、傳導(dǎo)性耳聾、A型曲線（包括As型和Ad型）、蓋萊試驗(yàn)陰性。其它臨床罕見(jiàn)內(nèi)耳傳導(dǎo)性耳聾如大前庭導(dǎo)水管綜合征、上半規(guī)管裂綜合征、外后半規(guī)管裂綜合征、X-連鎖鐙井噴綜合征等[1]不在本文討論范圍。其它鼓膜完整的分泌性中耳炎、粘連性中耳炎、隱匿性中耳炎等也不屬于本文討論范圍。

傳統(tǒng)HRCT常規(guī)平片能夠很好的顯示鼓室、鼓竇、乳突病變狀態(tài)，即是否存在密度增高影及骨質(zhì)破壞，因?yàn)檫@涉及到是否開(kāi)放乳突等手術(shù)策略問(wèn)題，而本組以聽(tīng)骨鏈為主的疾病則鼓室乳突鼓竇基本正常；HRCT常規(guī)平片提供的另外一個(gè)重要信息是顳骨內(nèi)重要結(jié)構(gòu)如內(nèi)聽(tīng)道、耳蝸、半規(guī)管、乙狀竇、頸靜脈球等是否正常、畸形或破壞；但HRCT常規(guī)平片并不能很好的顯示聽(tīng)骨鏈的精細(xì)結(jié)構(gòu)，唐朝穎（2016）報(bào)告HRCT對(duì)先天性聽(tīng)骨鏈畸形的顯示僅為40%，外傷性聽(tīng)骨鏈中斷僅為50%[2]。但是，近年發(fā)展的多平面重組MPR（multi-planar ref?ormation）和三維容積重建技術(shù)3D-CTVR（three-dimensional volume rendered computer tomog?raphy）則能夠很好的顯示錘砧骨、鐙骨及其連接，在影像上能夠直觀的鑒別聽(tīng)骨鏈?zhǔn)欠裢暾⑵茐?、畸形、連接異常[34]。MPR與CTVR技術(shù)是應(yīng)用現(xiàn)代螺旋CT，通過(guò)“各向同性掃描”方式獲得組織結(jié)構(gòu)內(nèi)部的點(diǎn)陣信息（即容積數(shù)據(jù)）后，將數(shù)據(jù)通過(guò)后處理技術(shù)進(jìn)行重建獲得各種圖像。MPR圖像可以理解為將三維影像投影到同一平面，從而實(shí)現(xiàn)同層全程顯示；CTVR則是以不同閾值用不同顏色代表，對(duì)應(yīng)不同結(jié)構(gòu)，通過(guò)視覺(jué)色差在人眼視網(wǎng)膜獲得三維信息，通過(guò)旋轉(zhuǎn)、閾值濾過(guò)實(shí)現(xiàn)物體內(nèi)部的三維結(jié)構(gòu)顯示[4-6]。因此本文側(cè)重于從現(xiàn)代影像學(xué)角度論述上述具有共同特征的一組疾病的鑒別診斷，同時(shí)兼顧各疾病的胚胎發(fā)生學(xué)及病理解剖學(xué)特征，外科聽(tīng)力重建方法以2013年中華耳鼻咽喉頭頸外科雜志刊登《中耳炎臨床分類和手術(shù)分型指南（2012）》及解讀[7，8]中Ⅱ、Ⅲ型鼓室成形術(shù)為參照，介紹手術(shù)策略但不做詳細(xì)的技術(shù)描述。

1 耳硬化癥

1、耳硬化癥（otosclerosis）于1704年由Valsava首次發(fā)現(xiàn)，1872由 Anton von Tr?ltsch 首先命名[9]。主要病理改變是耳囊骨質(zhì)局灶性吸收與富含血管的海綿狀新骨形成，如果這種病理改變發(fā)生或發(fā)展至前庭窗周圍及環(huán)韌帶導(dǎo)致底板固定，即可引起傳導(dǎo)性耳聾。需要注意的是耳硬化癥底板固定和先天性底板骨化是二個(gè)概念，耳硬化癥主要是底板邊緣和前庭窗之間形成新生骨質(zhì)導(dǎo)致底板固定，而先天性底板骨化則是胚胎發(fā)育過(guò)程中，來(lái)自耳囊的環(huán)韌帶發(fā)育不全或發(fā)育后骨化形成[10]。

2、臨床診斷主要依靠病史和聽(tīng)力學(xué)檢查。影像診斷方面常規(guī)HRCT有時(shí)可在前庭窗周圍見(jiàn)蠶食樣改變。MPR和CTVR在耳硬化癥中可顯示完整的聽(tīng)骨鏈，其意義在于鑒別排除聽(tīng)骨鏈中斷和聽(tīng)骨鏈畸形，見(jiàn)圖1。

3、手術(shù)方法從最初的鐙骨撼動(dòng)逐步演變?yōu)殓嫻侨谐㈢嫻遣糠智谐?，直至目前底板造孔后Piston植入技術(shù)[11]。

圖1 耳硬化癥。A：HRCT常規(guī)平片軸位，可見(jiàn)錘骨頭（紅箭頭）和砧骨體（黃箭頭），耳蝸和前庭骨質(zhì)未見(jiàn)蠶食樣改變。B：MPR圖片，顯示完整鐙骨（白箭頭），可見(jiàn)鐙骨頭、前后弓、鐙骨底板，注意底板中央部分密度略低，在MPR圖片上提示可能為軟骨。C：CTVR圖像，聽(tīng)骨鏈三維容積重建后完整顯示聽(tīng)骨結(jié)構(gòu)及連接，錘骨（紅箭頭）、砧骨（黃箭頭）、鐙骨（白箭頭）及其前后弓和底板顯示良好，從而鑒別區(qū)分了聽(tīng)骨鏈中斷及畸形。Fig.1 Otosclerosis.A:Conventional axial section plane of HRCT image.Themalleus head(red arrow)and incus body(yellow arrow)is visible,the cochlear and vestibular bone shows no eroded sign.B:MPR image.The normal head,crus,and foot plane of staps can be seen.The central part of foot plane can be noted because of lower density,whichmay indicate cartilage.C:CTVR image.The whole ossicular structure and connection can be displayed well after three-dimensional reconstruction.This 3D image can exclude the ossicular chain disruption and deform ity.red arrow:malleus;yellow arrow:incus;white arrow:stapes.

2 鼓室硬化

1、鼓室硬化(tympanosclerosis)是中耳在長(zhǎng)期慢性炎癥愈合后所遺留的中耳結(jié)締組織退行性改變，1873 年由 Von Troltsh[12]最先提出，Ho KY.(2010)年報(bào)告在中耳炎疾病中的發(fā)生率為5.5%[13]。其主要病理表現(xiàn)是鈣鹽沉積在鼓膜纖維層、鼓岬粘膜、聽(tīng)骨鏈周圍，嚴(yán)重者可包裹整個(gè)聽(tīng)骨鏈或鐙骨區(qū)域，導(dǎo)致聽(tīng)骨鏈固定，甚至導(dǎo)致底板骨化。

2、在臨床診斷中除病史和聽(tīng)力學(xué)特征外，HRCT影像對(duì)鼓室硬化的特異影像檢出率極低，有報(bào)道僅為26.13%，對(duì)聽(tīng)骨鏈中斷的漏診率為51.61%[14]。而重建后的MPR圖像對(duì)鈣化、骨化灶則顯示良好，其鈣化灶密度較軟組織高但不均勻，鈣化灶可包裹鐙骨或與面神經(jīng)粘連,見(jiàn)圖2。在影像上單純從底板形態(tài)與耳硬化癥很難區(qū)別，但鈣化灶可間接提示鼓室硬化。

3、清除病灶后聽(tīng)骨鏈重建的方式可按以下思路完成手術(shù)：①全聽(tīng)骨鏈活動(dòng)完整，則保留聽(tīng)骨鏈；②底板活動(dòng)，鐙骨上結(jié)構(gòu)完整，可按Ⅱ型手術(shù)完成；③底板活動(dòng)，鐙骨上結(jié)構(gòu)缺如，可按Ⅲ型手術(shù)完成（圖2-3）；④底板固定骨化，可按耳硬化癥手術(shù)完成，如果底板開(kāi)窗后有內(nèi)耳感染風(fēng)險(xiǎn)，可待二期手術(shù)底板開(kāi)窗聽(tīng)力重建[15]。⑤震動(dòng)聲橋（VSB）植入：Pau HW(2010)年報(bào)告在二窗被鈣化灶嚴(yán)重阻塞的情況下，在鼓岬開(kāi)窗植入VSB振動(dòng)子[16]。

圖2 鼓室硬化.A：MPR圖片，顯示錘骨及融合的錘砧關(guān)節(jié)（白箭頭），砧骨（黃箭頭）長(zhǎng)腳遠(yuǎn)端與鈣化灶融合（紅箭頭），鐙骨區(qū)域?yàn)槊芏雀哂谲浗M織但低于骨組織的不均勻影像，為鈣化灶（紅箭頭），完全包裹融合鐙骨，同時(shí)與面神經(jīng)相粘連（黑箭頭）。B:黑箭頭為暴露的面神經(jīng)，紅箭頭顯示鐙骨區(qū)域廣泛鈣化灶。C:在剝離清除鈣化灶后確認(rèn)底板活動(dòng)良好，植入TORP聽(tīng)骨（白箭頭）。Fig.2 Tympanosclerosis.A:MPR image.The imaging showes the normal malleus and fused incudomalleolar joint(white arrow).The distal long processof incus(yellow arrow)confused w ith the calcification(red arrow).The density of ovalw indow area(red arrow)was higher than thatof the soft tissue but lower than that of the bone.This calcification surrounded the stapes and adhered to the facial nerve(black arrow).B:The black arrow indicate the naked facial nerve and the red arrow indicate the extensive calcification in the stapes area.C:The TORP(white arrow)was implanted after erdicating the calcification.

3 外傷性聽(tīng)骨鏈中斷

1、外傷性聽(tīng)骨鏈中斷可以是單純的聽(tīng)骨鏈損傷，也可是顳骨骨折的一部分，如果僅有聽(tīng)骨鏈損傷，可表現(xiàn)為鼓膜完整的傳導(dǎo)性耳聾。聽(tīng)骨鏈的病理?yè)p傷主要位于錘砧關(guān)節(jié)、砧鐙關(guān)節(jié)分離移位、鐙骨上結(jié)構(gòu)骨折，少見(jiàn)的有前庭底板分離、底板骨折等等。

2、明確的外傷病史、面神經(jīng)損傷、內(nèi)耳損傷有助于診斷。影像診斷中常規(guī)HRCT平片對(duì)聽(tīng)骨鏈損傷的精細(xì)診斷缺乏特異性[17]，但MPR圖像及CT?VR圖像則能夠很好的顯示聽(tīng)骨鏈損傷狀態(tài)，定位損傷部位，特別是病史久遠(yuǎn)的外傷，砧骨長(zhǎng)腳遠(yuǎn)端缺血骨質(zhì)吸收等，見(jiàn)圖3。

3、聽(tīng)骨鏈的重建是在顳骨骨折、內(nèi)耳損傷、面神經(jīng)損傷治療恢復(fù)后的治療，包括：①聽(tīng)骨鏈完整但關(guān)節(jié)離斷錯(cuò)位、與周圍組織粘連骨化等，可復(fù)位修復(fù)關(guān)節(jié)、分離粘連、或在過(guò)多分離的的關(guān)節(jié)間插入軟骨片以連接聽(tīng)骨鏈（圖3）；②聽(tīng)骨鏈其它部位的損傷可按Ⅱ型和Ⅲ型鼓室成形完成；③底板的骨折可先以軟組織覆蓋骨折處再按Ⅱ型和Ⅲ型鼓室成形完成手術(shù)。

圖3 外傷性聽(tīng)骨鏈中斷.A：MPR圖片，聽(tīng)骨周圍可見(jiàn)軟組織包裹，錘骨頭（紅箭頭）與砧骨體（黃箭頭）之間的錘砧關(guān)節(jié)（白箭頭）呈分離狀態(tài)，黑箭頭指示骨折線；B:CTVR重建圖像，濾除包裹聽(tīng)骨的軟組織，顯示聽(tīng)骨鏈狀態(tài)，白箭頭顯示分離的錘砧關(guān)節(jié)；C:術(shù)中見(jiàn)鼓竇側(cè)壁骨折線（黑箭頭），錘砧關(guān)節(jié)分離(白箭頭)，白色星號(hào)為軟骨，插入襯墊在錘砧關(guān)節(jié)之間，連接聽(tīng)骨鏈。Fig.3 Ttraumatic ossicular chain disruption.A:MPR image.The incudomalleolar joint was separated and the ossicular chain was surrounded by the soft tissue.red arrow:malleus head;yellow arrow:body of incus;black arrow:fracture line.B:CTVR image.After the soft tissue was filtered,the ossicular chain was displayed.White arrow:the disarticulated incudomalleolar joint.C:The broken line(black arrow)can be seen in the tympanic sinus wall.The white arrow indicate the separation of incudomalleolar joint.A small piece of cartilage(white asterisk)was inserted into the seperated incudomalleolar joint to connect the ossicular chain.

4 先天性膽脂瘤

1、先天性膽脂瘤是胚胎期外胚層上皮組織遺留于顳骨內(nèi)發(fā)展而成的角化上皮，通常沿聽(tīng)骨內(nèi)側(cè)向后生長(zhǎng)至中鼓室后上方，初期多破壞砧鐙關(guān)節(jié)、鐙骨上結(jié)構(gòu)，最后逐漸發(fā)展為顳骨廣泛膽脂瘤[18]。本文討論的是Derlacki(1965)及Levenson(1986)描述的特征為膽脂瘤位于完整鼓膜內(nèi)側(cè)、伴中耳炎病史[19,20]的一組疾病，即膽脂瘤局限于鼓室引起聽(tīng)骨破壞導(dǎo)致傳導(dǎo)性耳聾的病例。

2、常規(guī)HRCT對(duì)此病診斷并不困難，鼓竇鼓室內(nèi)包裹聽(tīng)骨、邊界清晰的軟組織密度增高影提示膽脂瘤，在MRI影像中，T1加權(quán)像上呈中等偏低信號(hào)，T2加權(quán)像呈高信號(hào)，增強(qiáng)掃描無(wú)強(qiáng)化[21]。但對(duì)聽(tīng)骨鏈破壞部位的診斷，MPR圖像及CTVR圖像則更顯優(yōu)勢(shì)，能夠清晰的顯示聽(tīng)骨破壞程度和缺失部位，見(jiàn)圖4。

3、清除病灶后的聽(tīng)力重建與鼓室硬化基本相同，但底板固定開(kāi)窗的少見(jiàn)。

圖4 先天性膽脂瘤。A：常規(guī)HRCT冠狀位，紅箭頭指示錘骨，但聽(tīng)骨鏈結(jié)構(gòu)不清，砧骨難以區(qū)分，聽(tīng)骨內(nèi)側(cè)軟組織影（星號(hào)）提示膽脂瘤，與面神經(jīng)緊鄰（黑箭頭），中鼓室及上鼓室內(nèi)為良好的含氣腔。B：MPR圖像，紅色箭頭指示錘骨，黃色箭頭指示砧骨體，未見(jiàn)砧骨長(zhǎng)腳，右下紅色方框內(nèi)為面神經(jīng)MPR圖像，可見(jiàn)與膽脂瘤軟組織影相粘連，面神經(jīng)骨管部分缺如；C：CTVR三維重建圖像，錘骨（紅箭頭）和砧骨體（黃箭頭）顯示清楚。濾除膽脂瘤軟組織后，未見(jiàn)砧骨長(zhǎng)腳和鐙骨上結(jié)構(gòu)。手術(shù)證實(shí)軟組織影為膽脂瘤，砧骨長(zhǎng)腳和鐙骨上結(jié)構(gòu)缺如，面神經(jīng)水平段骨管破壞，術(shù)中以鈦TORP聽(tīng)骨重建聽(tīng)骨鏈。Fig.4 Congenital cholesteatoma.A:Conventional coronal section plane of HRCT image.red arrow:malleus.The ossicular chain displayed unclearly and the incus could notbe identified well.The soft tissue(asterisk)inside the ossicles indicate the cholesteatoma contecting to the facial nerve(black arrow).The mesoty mpanum and attic was normal air cavity.B:MPR image.The red arrows indicate themalleus,the yellow arrow indicate the incus without long limb.The red box is the MPR image of facial nerve adhering to the cholesteatoma.C:CTVR image.The mallues(red arrow)and body of incus(yellow arrow)were displayed clearly.After the sofft tissue was filtered,the long limb of incus and stapes superstructure could not be seen.In the operation,the soft tissue was approved to be cholesteatoma,the long limb of incus and stapes superstructure absent,and the horizontal segmentof facial canal damaged.The titanium-TORPwas used to reconstruct the ossicular chain.

5 先天性聽(tīng)骨鏈畸形

廣義的外中耳畸形包括耳廓畸形、外耳道畸形、中耳畸形。本文所述內(nèi)容為非綜合征型中耳畸形，主要為外耳道鼓膜正常的單純聽(tīng)骨鏈畸形。1993年，Teunissen[22]較早的提出了中耳畸形的分類觀點(diǎn)，隨后 Nagao(1995)[23]、Okano（2003）[24]、袁虎（2005）[25]、Park K（2009）[26]等先后提出不同的分類觀點(diǎn)。其中文獻(xiàn)普遍引用的是Teunissen（1993）分類：Ⅰ類先天性鐙骨固定；Ⅱ類先天性鐙骨固定伴聽(tīng)骨鏈畸形；Ⅲ類先天性聽(tīng)骨鏈畸形但鐙骨足板活動(dòng)；Ⅳ類先天性前庭窗或蝸窗發(fā)育不全或重度發(fā)育異常。綜合以上各家分類，為便于理解，本文從胚胎發(fā)生學(xué)和聽(tīng)力重建角度，以鐙骨為中心分三個(gè)部分闡述：Ⅰ型-鐙骨底板活動(dòng)；Ⅱ型-鐙骨底板固定；Ⅲ型-前庭窗骨性閉鎖或未發(fā)育。

▲Ⅰ型-鐙骨底板活動(dòng)

1、胚胎發(fā)育第15周左右，第一腮弓內(nèi)的Michle軟骨發(fā)育成砧骨短腳、砧骨體、錘骨頭等上鼓室內(nèi)的聽(tīng)骨，如果上鼓室氣化不良或Michle軟骨不能完全吸收，則導(dǎo)致的聽(tīng)骨畸形主要表現(xiàn)為錘砧關(guān)節(jié)融合固定、砧骨體錘骨頭與上鼓室骨質(zhì)相融合連接[27,28]；第二腮弓的Reichert軟骨發(fā)育成錘骨柄、砧骨長(zhǎng)腳、鐙骨上結(jié)構(gòu)、鐙骨底板等中鼓室內(nèi)的聽(tīng)骨，如果Reichert軟骨發(fā)育不良導(dǎo)致的畸形主要表現(xiàn)以砧骨長(zhǎng)腳和鐙骨上結(jié)構(gòu)的缺失為主[29-31]。因此，由Michle軟骨和Reichert軟骨發(fā)育導(dǎo)致的畸形可歸納為Ⅰ型，共同特點(diǎn)是鐙骨底板活動(dòng)，聽(tīng)骨鏈各部位可出現(xiàn)單獨(dú)或合并畸形，包括錘骨頭固定、砧骨體固定、錘砧關(guān)節(jié)固定、砧骨長(zhǎng)腳缺如、砧鐙骨軟性連接、砧鐙關(guān)節(jié)中斷、孤立的豆?fàn)钔弧㈢嫻巧辖Y(jié)構(gòu)與鄰近結(jié)構(gòu)的骨性條索狀連接、鐙骨上結(jié)構(gòu)的缺失團(tuán)塊等。偶見(jiàn)面神經(jīng)鼓室段低垂，擠壓鐙骨上結(jié)構(gòu)[32]。考慮到現(xiàn)代外科重建技術(shù)，進(jìn)一步分型鐙骨上結(jié)構(gòu)正常可列為Ⅰa型，鐙骨上結(jié)構(gòu)畸形可列為Ⅰb型。

2、在影像學(xué)聽(tīng)骨CT顯示中，常規(guī)HRCT能夠提示聽(tīng)骨鏈畸形，但更多精細(xì)結(jié)構(gòu)并不能很好顯示。MPR圖像及CTVR則能夠很好的顯示畸形聽(tīng)骨精細(xì)結(jié)構(gòu)，特別是鐙骨前后弓在MPR圖像中顯示更為清晰，見(jiàn)圖5-1-1，圖5-1-2。外科聽(tīng)力重建中，Ⅰa型可按鼓室成形Ⅱ型完成，Ⅰb型則可按Ⅲ型完成。

圖5-1-1 聽(tīng)骨鏈畸形Ⅰa型，A、B為MPR圖片，C圖為CT?VR圖片。A圖見(jiàn)錘骨（紅箭頭）完整，砧骨長(zhǎng)腳缺如，砧骨體（黃箭頭）與錘骨頭融為一體；B圖見(jiàn)鐙骨結(jié)構(gòu)良好，清晰可見(jiàn)前后弓、鐙骨頭（黑箭頭）、底板；C圖見(jiàn)錘骨柄（紅箭頭）良好，鐙骨前后弓、鐙骨頭良好（黑箭頭），但未見(jiàn)砧骨長(zhǎng)腳。術(shù)中見(jiàn)鐙骨底板活動(dòng)，以PORP重建聽(tīng)骨鏈。Fig.5-1-1 Ossicular chain malformation type Ia.A&B：MPR image,C:CTVR images.A:The malleus(red arrow)was normal,the long process of incuswas absent,the body of in cus(yellow arrow)and the head of mallues was fused.B:The stapes was clear with the crus,head amd plate of stapes(black arrow)normaly,C:The hand of mallues(red arrow)together with crus and head of stapes(black arrow)was normal,,but the long limb of in cus wasabsent.The footplate of stapes wasmoved and the PORP was used to reconstruc the ossicular chain.

圖5-1-2 聽(tīng)骨鏈畸形Ⅰb型，A圖為MPR圖片，見(jiàn)錘骨（紅箭頭）完整，砧骨長(zhǎng)腳缺如，砧骨體（黃箭頭）與錘骨頭融為一體，紅色方框內(nèi)白色箭頭指示為鐙骨底板及底板上殘余的骨性結(jié)構(gòu)；B圖為CTVR圖片，紅色箭頭指示完整的錘骨柄，白色箭頭指示鐙骨底板及其上的殘余結(jié)構(gòu)，可能為前后弓發(fā)育過(guò)程中的殘跡，未見(jiàn)砧骨長(zhǎng)腳；C圖為術(shù)中所見(jiàn)，鐙骨上結(jié)構(gòu)不存在，面神經(jīng)（黑箭頭）裸露低垂，覆蓋前庭窗上緣，術(shù)中探查鐙骨底板活動(dòng)，以TORP（星號(hào)）重建聽(tīng)骨鏈。Fig.5-1-2 Ossicular chainmalformation type Ib.A:MPR image.Themalleus(red arrow)was normal,the long process of incus was absent,the body of incus(yellow arrow)and the head ofmallues was fused.The red box indicate the normal foot plate of stapesw ith the residual superstructure of stapes.B:CTVR image.The red arrow indicate the normal hand of mallus and thewhite arrow indicate the footplatew ith residual crus of stapes.The long limb of incuswas not seen.C:The surgical image.The stapes superstructure was absent,the facial nerve was dehiscent and cover the upper edge of oval w indow.The foot plate of stapes wasmoved and the TORP wasused to reconstruct the ossicular chain.

▲Ⅱ類-鐙骨底板固定

1、在胚胎第14-18周，第一、二腮弓軟骨發(fā)育成聽(tīng)骨鏈的同時(shí)，鐙骨底板同時(shí)發(fā)育成熟，與來(lái)自迷路包囊的前庭窗及環(huán)韌帶吻合[33]，這一過(guò)程可導(dǎo)致底板固定，其胚胎發(fā)生的病理狀態(tài)包括二種情況：其一是環(huán)韌帶在胚胎期即沒(méi)有完全發(fā)育，另外一種情況是雖已發(fā)育但出生后逐漸骨化[10]，是否進(jìn)一步發(fā)展成整個(gè)底板的骨化還缺乏足夠的胚胎學(xué)證據(jù)。如果僅有底板固定，Teunissen[22]將其分為class1，如果底板固定的同時(shí)伴有由Michle和Reichert軟骨分化而來(lái)的聽(tīng)骨鏈畸形，Teunissen[22]將其分為class2。但從現(xiàn)代手術(shù)方式角度看，無(wú)論底板以上聽(tīng)骨鏈結(jié)構(gòu)是否正常，主流手術(shù)方式均以底板手術(shù)為核心，故本文將其列為Ⅱ類一起闡述，進(jìn)一步分型僅有底板固定可列為Ⅱa型，合并底板上其他聽(tīng)骨鏈畸形可列為Ⅱb型，更有助于理解。

2、CT檢查圖像與Ⅰ型表現(xiàn)類似，如果鐙骨底板以上結(jié)構(gòu)正常，在CT圖像上與耳硬化癥不易區(qū)別，通常術(shù)中判斷。外科聽(tīng)力重建通常在底板開(kāi)窗處（底板全切除、部分切除、微孔技術(shù)）與砧骨長(zhǎng)腳/錘骨柄之間建立連接，選用的重建材料包括TORP和Piston，類似耳硬化癥手術(shù)（見(jiàn)圖5-2）。

圖5-2 聽(tīng)骨鏈畸形Ⅱ型術(shù)中圖像?；颊吣行裕?8歲，氣骨導(dǎo)間距語(yǔ)頻30-35dB，自幼聽(tīng)力較差，術(shù)前影像聽(tīng)骨結(jié)構(gòu)與耳硬化癥相同，術(shù)前診斷考慮耳硬化癥或先天性聽(tīng)骨鏈畸形。A圖:見(jiàn)錘骨、砧骨（黃箭頭）完整，錘砧關(guān)節(jié)活動(dòng)度差，砧鐙關(guān)節(jié)骨性融合，前后弓骨化，白色箭頭指示去除前后弓的鐙骨底板，呈骨化固定狀態(tài)。黑色箭頭指示面神經(jīng)，骨管缺損，神經(jīng)組織暴露但位置正常；B圖：去除前后弓后，磨薄骨化的底板至略呈藍(lán)色，以三棱針在底板中央偏后的位置鉆孔開(kāi)窗，見(jiàn)外淋巴液（紅箭頭）；C圖：以4.25mm Piston（星號(hào)）鈦聽(tīng)骨重建聽(tīng)骨鏈。Fig.5-2 Ossicular chain malformation typeⅡ.A patient of 28 years old,male,had poor hearing at his childhood with A-Bgap 30-35dB(0.5,1,2KHz).The preoperative diagnosis was otosclerosis or congenital ossicular chain malformation.A:The mallus and incus were normal,the incudomallular joint moved poorly amd the incudostapedia joint was fused.The white arrow indicate the fixed foot plate with crus eradicated.The black arrow indicate the dehiscent facialnerve with normal poisition.B:The footplate was drillout(red arrow)to a hole with three edged needle after the crus mass was removed and the ossified footplate was bured to a thin bone.C:Theossicular chainwas reconstructed with a4.25mm Piston.

▲Ⅲ類：前庭窗骨性閉鎖或未發(fā)育

在第一、二腮弓發(fā)育過(guò)程中的第8-10周，來(lái)自Reichert軟骨的面神經(jīng)組織與來(lái)自迷路包囊的骨管吻合；鐙骨前庭窗吻合與面神經(jīng)骨管吻合在時(shí)間與空間上高度相關(guān)，畸形相互影響[34]。所以在鐙骨前庭窗區(qū)域的畸形中，前庭窗可能沒(méi)有發(fā)育呈現(xiàn)骨性閉鎖板（Ⅲ型）、或底板雖然與前庭窗吻合但底板與前庭窗骨性融合（Ⅱ型）、或底板與前庭窗吻合活動(dòng)但鐙骨上結(jié)構(gòu)畸形（Ⅰ型），而面神經(jīng)則可以部分或全部覆蓋前庭窗[34-36]。因此前庭窗、鐙骨、面神經(jīng)胚胎發(fā)育互相影響所致畸形則列為單獨(dú)一種類型。主要表現(xiàn)為前庭窗未發(fā)育、較厚的骨性閉鎖板、前庭窗龕呈裂隙狀狹窄等；同時(shí)可伴有一、二腮弓發(fā)育異常導(dǎo)致的聽(tīng)骨鏈畸形，畸形的鐙骨或類似鐙骨的畸形結(jié)構(gòu)可以畸跨、擠壓、覆蓋面神經(jīng)；裸露畸形的面神經(jīng)走行在前庭窗上緣、正中或下緣或分叉走行，Su Y（2014）報(bào)告這一類型面神經(jīng)畸形發(fā)生率69.1%（67/97）[36],de Alarcon A（2008）報(bào)告畸形發(fā)生率58.8%（10/17）[37]。

2、在影像學(xué)聽(tīng)小骨CT顯示中，常規(guī)HRCT同樣能夠提示聽(tīng)骨鏈畸形，但MPR圖像及CTVR則能夠很好的顯示畸形聽(tīng)骨精細(xì)結(jié)構(gòu)。面神經(jīng)走行異常的診斷在這一類型中至關(guān)重要，常規(guī)HRCT冠狀位結(jié)合MPR圖像，以下三點(diǎn)征象可以幫助診斷面神經(jīng)畸形和前庭窗閉鎖[38-40]見(jiàn)圖5-3-1：①在HRCT冠狀位平行通過(guò)上半規(guī)管平面劃一條a線，再通過(guò)水平半規(guī)管基部劃一條b線垂直a線，十字交叉線的外上象限即為面神經(jīng)正常位置，如果面神經(jīng)位于十字線外下象限即為走行畸形；②前庭窗龕MPR圖像呈現(xiàn)“V”字形，說(shuō)明前庭窗可能沒(méi)有發(fā)育；③前庭窗閉鎖骨板增厚，呈骨性結(jié)構(gòu)，并非鐙骨底板的半透明軟骨征象。

圖5-3-1 面神經(jīng)走行異常定位診斷。A&B：HRCT圖像，a線平行通過(guò)上半規(guī)管平面，b線通過(guò)水平半規(guī)管根部垂直于a線，A圖顯示面神經(jīng)（紅箭頭）處于正常位置，位于ab紅十字交叉線外上象限，B圖為畸形位置，位于交叉線外下象限（紅箭頭）；B圖同時(shí)顯示前庭窗龕呈現(xiàn)橫“V”字形，未見(jiàn)鐙骨底板，提示前庭窗閉鎖；C圖為MPR圖像，白箭頭顯示前庭窗呈骨性閉鎖狀態(tài)，與鐙骨底板軟骨的影像不同，閉鎖板為骨性，紅箭頭指示面神經(jīng)位于閉鎖板下方。Fig.5-3-1 Localization of abnormal facial nerve in CT image.A&B:coronal secton plane of HRCT image.Line a:parallel to the plane of superior semicircular canal;Line b:crossing“l(fā)ine a”through the foot of horizontal semicircular canal;red arrow:facial nerve(A:normal position;B:abnormal position).The atretic oval window was displayed as“V”shape without foot plate in B image.C:MPR image.Thewhite arrow indicate the thick bone plate of oval window which is different from the cartilage of foot plate.The red arrow indicate the abnormal facial nerve inferior to the atretic plate of oval window.

3、外科治療：①面神經(jīng)位置正?；虿糠终趽跚巴ゴ埃好嫔窠?jīng)可能位于前庭窗上緣或下緣，只要有足夠的空間暴露前庭窗，即可將骨性閉鎖的前庭窗骨板磨薄至藍(lán)色菲薄骨質(zhì)，按耳硬化癥行全部/部分底板切除后在鼓膜底板之間以TORP連接，或應(yīng)用微孔技術(shù)植入Piston聽(tīng)骨[32，41]（圖5-3-2）。②面神經(jīng)完全覆蓋前庭窗區(qū)域：此時(shí)在原前庭窗處植入聽(tīng)骨進(jìn)行聽(tīng)力重建因面神經(jīng)畸形已喪失條件，多數(shù)放棄，而傳統(tǒng)的外半規(guī)管開(kāi)窗因聽(tīng)骨鏈重建困難并不適合。盡管如此，仍有極具挑戰(zhàn)性的重建技術(shù)供富有豐富經(jīng)驗(yàn)的耳科醫(yī)師選擇：技術(shù)一：鼓階開(kāi)窗TORP植入技術(shù)[39-42]（圖5-3-3）。這一技術(shù)的主要方法是在圓窗膜前下鼓階起始段磨除骨質(zhì)至骨內(nèi)膜，覆蓋筋膜后放置TORP聽(tīng)骨，通過(guò)鼓膜-TORP-鼓階外淋巴產(chǎn)生振動(dòng)。這一方法的最大風(fēng)險(xiǎn)是引起神經(jīng)性耳聾，關(guān)鍵是應(yīng)保持骨內(nèi)膜完整。技術(shù)二：震動(dòng)聲橋VSB植入[43-45]，VSB振子可放置在圓窗龕，通過(guò)振動(dòng)子-圓窗膜-鼓階外淋巴產(chǎn)生振動(dòng)，前提是圓窗龕沒(méi)有閉鎖，如果圓窗龕閉鎖，則可以借鑒技術(shù)一將振動(dòng)子放置在鼓階起始段通過(guò)振動(dòng)鼓階外淋巴產(chǎn)生聽(tīng)力。

圖5-3-2 先天性聽(tīng)骨鏈畸形Ⅲ型。A：HRCT圖像，白色虛箭頭指示閉鎖的前庭窗，前庭窗龕呈現(xiàn)橫“V”字形，未見(jiàn)鐙骨底板，黑箭頭指示面神經(jīng)軟組織影，無(wú)骨管，提示走行畸形，位于前庭窗龕下緣；B：CTVR圖像，紅箭頭指示錘骨，形態(tài)良好，黃箭頭指示砧骨長(zhǎng)腳，遠(yuǎn)端變細(xì)，與畸形鐙骨上結(jié)構(gòu)相連（白箭頭）；C圖：術(shù)中圖像，黃箭頭指示砧骨長(zhǎng)腳，遠(yuǎn)端與畸形鐙骨上結(jié)構(gòu)（白箭頭）融合連接，黑箭頭指示裸露的面神經(jīng)，位于閉鎖的前庭窗下緣，白色虛箭頭指示狹窄的前庭窗閉鎖板，C圖紅色方框顯示閉鎖板機(jī)械打孔后植入Piston聽(tīng)骨。Fig.5-3-2 Ossicular chain malformation typeⅢ.A:HRCT image.The white dotted arrow indicate the atretic bone plate of over window which displays as a“V”shape.The black arrow indicate the abnormal naked facial nerve located to the lower edge of the oval window.B:CTVR image.The red arrow indicte the normal mullues and the yellow arrow indicte the thiner long limb connecting with the stapes superstructure(white arrow).C:Surgical image.The yellow arrow indicte the long limb of incus confussing with the stapes superstructure.The black arrow indicate the naked facial nerve located at the lower edge of oval window.The white dotted arrow indicate the narrow atretic bone plate of oval window.The red box shows the implanted Piston.

圖5-3-3 先天性聽(tīng)骨鏈畸形Ⅲ型。A：HRCT圖像，白色箭頭指示閉鎖的前庭窗骨板，厚度接近0.8mm，黑箭頭指示面神經(jīng)軟組織，無(wú)骨管，提示走行畸形，位于前庭窗閉鎖板下緣；B：CTVR圖像，紅箭頭指示錘骨，形態(tài)良好，黃箭頭指示砧骨長(zhǎng)腳，遠(yuǎn)端變細(xì)，與畸形鐙骨上結(jié)構(gòu)相連，越過(guò)面神經(jīng)（黑箭頭）表面緊貼下方向前庭窗處走行；DEF為術(shù)中圖像，D：黃箭頭指示砧骨長(zhǎng)腳，遠(yuǎn)端與畸形鐙骨上結(jié)構(gòu)融合連接，畸跨于裸露的面神經(jīng)（黑箭頭）表面，面神經(jīng)完全覆蓋前庭窗，白色虛箭頭指示圓窗龕；E圖黑色虛箭頭指示在圓窗龕前方（圓窗膜的前下方）、面神經(jīng)（黑箭頭）下方磨除鼓岬骨質(zhì)，暴露鼓階起始段骨內(nèi)膜，黃箭頭指示砧鐙關(guān)節(jié)，位于面神經(jīng)表面，因角度關(guān)系鐙骨上畸形結(jié)構(gòu)被遮擋；F：顯示鼓階開(kāi)窗處覆蓋筋膜后植入TORP連接鼓膜；C圖為術(shù)后影像，與A圖同一部位，紅色虛箭頭指示植入的人工聽(tīng)骨，位于鼓階起始段骨質(zhì)內(nèi)，沒(méi)有進(jìn)入鼓階外淋巴腔，且位于面神經(jīng)（黑箭頭）下方，位置良好，與前庭窗開(kāi)窗（白箭頭處）具有相同的解剖學(xué)和傳音生理學(xué)基礎(chǔ)。Fig.5-3-3 Ossicular chain malformation typeⅢ.A:HRCT image.The white arrow indicates the atretic bone plate of oval w indow w ith 0.8mm thickness.The black arrow indicates the abnormal naked facial nerve located to the lower edge of oval w indow.B:CTVR Image.The red arrow indicates the normal mallus and the yellow arrow indicates the thiner long limb of crus that connected to the stapes superstructure,covered the naked facial nerve and arrived to the ovalw indow.D/E/F:Surgical image.D:The yellow arrow indicates the distal end of the long limb confused w ith the stapes superstructure and crossed naked facial nerve(black arrow).The facial nerval covered the ovalw indow fully.White dotted arrow:round w indow nich.E:The black dotted arrow indicates the burred position anterior to the round w indow nich and inferior to the facial nerve(black arrow).The bone of scala tympaniwas drilled out and the endosteum was exposed.The yellow arrow indicates the incudostapedia joint convering the facialnerve.F:The image displays the fenestration w ith the implanted TORP.C:Postoperative image.The red dottted arrow indicate the implanted TORP located at the initial segment of scal tympani.The TORP did not enter the perilymphatic cavity and located at the lower edge of facial nerve.The fenestration of scal tympani(white arrow)had the same anatomical and physiological theory comparing w ith the stepdectomy.

結(jié)語(yǔ)

本文所述內(nèi)容為一組鼓膜完整的傳導(dǎo)性耳聾病例的影像鑒別診斷與手術(shù)策略選擇。既往術(shù)前診斷主要依靠病史和各自聽(tīng)力學(xué)特征，間接診斷鑒別成分較多，而現(xiàn)代影像特別是近年發(fā)展的三維影像重組技術(shù)，為這組疾病的鑒別提供了直觀的影像，有利于術(shù)前更明確的診斷；本文手術(shù)策略的梳理總結(jié)則是根據(jù)現(xiàn)代聽(tīng)骨鏈重建技術(shù)的主流趨勢(shì)，以鐙骨病變及處理為中心進(jìn)行概要論述，包括了經(jīng)典手術(shù)，也有作者原創(chuàng)手術(shù)的經(jīng)驗(yàn)，希望對(duì)同道臨床工作有所幫助。

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