耳硬化症

耳硬化症是一种原因不明的疾病，病理上是由于骨迷路原发性局限性骨质吸收，而代以血管丰富的海绵状骨质增生，故称“硬化”。当侵犯卵园窗时，可引起镫骨固定，失去传音功能，使听力进行性减退。别名：耳硬化英文名：otosclerosis发病部位：内耳就诊科室：耳鼻咽喉头颈科症状：耳聋耳鸣眩晕治疗手段：人工镫骨术助听器是否遗传：是是否传染：否

症状<pstyle="text-align:left;">1.听力下降：无明显诱因，双耳出现缓慢进行性、非对称性听力减退。由于自听增强，因此自语声小。另外，患者存在韦氏误听，即在嘈杂环境中患者感觉听力改善的现象。<pstyle="text-align:left;">2.耳鸣：约半数患者出现耳鸣，一般耳鸣程度较轻。<pstyle="text-align:left;">3.眩晕：耳蜗性耳硬化患者可出现眩晕，常表现为反复发作性眩晕或平衡障碍。

病因<pstyle="text-align:left;">尚不明确，可能与以下几种因素有关：<pstyle="text-align:left;">1.遗传性因素：部分耳硬化症患者有家族史，目前认为本病是常染色体显性或隐性遗传。<pstyle="text-align:left;">2.病毒因素：耳硬化症的发病可能与麻疹病毒有关。<pstyle="text-align:left;">3.内分泌因素：本病女性发病率高，且于妊娠期症状加重，因此认为本病发病与激素水平有关。<pstyle="text-align:left;">4.免疫因素：研究表明耳硬化症患者血清中自身抗体水平增高。

检查<!--[ifgtemso9]><xml><w:WordDocument><w:BrowserLevel>MicrosoftInternetExplorer4</w:BrowserLevel><w:DisplayHorizontalDrawingGridEvery>0</w:DisplayHorizontalDrawingGridEvery><w:DisplayVerticalDrawingGridEvery>2</w:DisplayVerticalDrawingGridEvery><w:DocumentKind>DocumentNotSpecified</w:DocumentKind><w:DrawingGridVerticalSpacing>7.8磅</w:DrawingGridVerticalSpacing><w:PunctuationKerning></w:PunctuationKerning><w:View>Normal</w:View><w:Compatibility><w:DontGrowAutofit></w:DontGrowAutofit><w:BalanceSingleByteDoubleByteWidth></w:BalanceSingleByteDoubleByteWidth><w:DoNotExpandShiftReturn></w:DoNotExpandShiftReturn><w:UseFELayout></w:UseFELayout></w:Compatibility><w:Zoom>0</w:Zoom></w:WordDocument></xml><![endif]--><pclass="MsoNormal"style="mso-pagination:widow-orphan;text-align:left;">1.耳镜检查：鼓膜完整，标志正常，部分患者可见后上象限透红区，为鼓岬区粘膜充血的反映，称为Schwartze征，提示耳硬化症处于活动期。<pclass="p"style="mso-pagination:widow-orphan;text-align:left;">2.听力学检查<pclass="p"style="mso-pagination:widow-orphan;text-align:left;">1）音叉检查：Weber试验偏向患侧或重的一侧，Rinne试验骨导大于气导。<pclass="p"style="mso-pagination:widow-orphan;text-align:left;">2）纯音测听：听力图表现为轻度至中度的传导性耳聋，听力下降的程度与耳硬化症的阶段有关。骨导在2kHz可有10-30dB的下降，称为卡哈切迹（Carhartnotch），系由镫骨固定所致。<pclass="p"style="mso-pagination:widow-orphan;text-align:left;">3）鼓室功能检查:鼓室图和声顺值正常，镫骨肌反射不能引出，咽鼓管功能良好。<pclass="p"style="mso-pagination:widow-orphan;text-align:left;">3.影像检查<pclass="p"style="mso-pagination:widow-orphan;text-align:left;">颞骨CT检查，应排除听小骨及内耳发育有无畸形，晚期耳硬化症病例，可以观察到镫骨底板增厚、迷路包囊骨影欠规则。<pclass="MsoNormal"><!--[ifgtemso9]><xml><w:LatentStylesDefLockedState="false"DefUnhideWhenUsed="true"DefSemiHidden="true"DefQFormat="false"DefPriority="99"LatentStyleCount="260"><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="Normal"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="heading1"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="heading2"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="heading3"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="heading4"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="heading5"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="heading6"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="heading7"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="heading8"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="heading9"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="index1"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="index2"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="index3"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="index4"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="index5"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="index6"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="index7"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="index8"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="index9"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="toc1"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="toc2"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="toc3"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="toc4"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="toc5"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="toc6"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="toc7"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="toc8"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="toc9"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="NormalIndent"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="footnotetext"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="annotationtext"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="header"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="footer"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="indexheading"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="caption"></w:LsdException><w:LsdExceptionLocked="false"Priority="99"SemiHidden="false"Name="tableoffigures"></w:LsdException><w:LsdExceptionLocked="false"Priori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诊断1.病因不清。常有家族史。表现为进行性听力减退，伴耳鸣。通常先发于一侧，孕期可加重。可有韦氏误听，部分患者有轻度眩晕。2.耳镜检查：鼓膜较薄或正常，部分患者有Schwartze征（透红征）。咽鼓管通畅，鼓室曲线呈As型。听功能检查：早期为传音聋，听力曲线以低频下降为主，中期曲线平坦，骨导曲线有Carhart切迹，晚期为混合性聋。Gelle氏试验阴性。声阻抗测听法显示声顺降低，镫骨肌反射消失。CT检查可见镫骨足板增厚，迷路骨影欠规则。

治疗1.手术为主。服用氟化钠、维生素D等据称可以停止病变。。2.手术治疗是提高听力最有效的措施，镫骨撼动术近期疗效较好，但硬化源不能控制，远期疗效差，目前已很少使用。内耳开窗术也能提高听力，但远期效果差，有可能出现感音神经性聋。目前普遍采用的是小窗镫骨术，安全性高，效果更好。3.不能手术者可配助听器。

预后耳硬化症为缓慢进行性侵及骨迷路的内耳病变，目前尚无有效药物阻止其发展，手术治疗仅改善声音的传导功能。

预防目前尚没有预防该病的方法

耳硬化患者镫骨的正常骨质被吸收且为富含血管的新生海绵状骨质所替代，继而导致镫骨固定并失去正常的传音、扩音功能，当病变进一步发展而侵及耳蜗时将影响感音功能。临床上病人常常表现为进行性传...