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先天性聽小骨異常之分類與手術結果

Classification and Surgical Outcome of Congenital Ossicular Anomalies

摘要


背景:臨床上單純先天性聽小骨異常相當少見,病人通常自幼年時期開始有非進行性傳導性聽障,沒有頭部外傷或中耳炎的病史,而先天性聽小骨異常早期診斷非常重要,經由外科手術治療,其術後聽力改善是可預期的。本研究針對此類患者的臨床分類和手術結果作分析探討,作為診斷及治療的參考。方法:以回溯性方式收集2007年7月至2014年3月間在臺大醫院因傳音性聽力障礙接受鼓室探查術的病人,術後診斷為先天性聽小骨異常為研究對象,總計18名20耳。研究資料包含術前純音聽力檢查、術中發現、手術方法、以及術後純音聽力檢查;其中純音聽力檢查,氣導與骨導閾值的記錄採用500, 1000, 2000, 4000Hz四頻率平均值。並根據Teunissen & Cremers分類方法將病人分類。術後聽力改善程度以兩種方式評估:一.聽力獲得(Hearing gain, AC gain)。二.手術前後氣骨導差閉合程度(Air-Bone Gap closure)。結果:合於本研究條件的患者共18名,男女比為10:8,平均手術年齡26.2歲。聽小骨異常根據Teunissen & Cremers分類:Class I:4耳;Class II:1耳;Class III:13耳;Class IV:2耳。Class I中4耳及Class II中1耳均接受人工鐙骨置換術;Class III中有5耳接受第三型鼓室成形術,8耳接受第四型鼓室成形術;Class IV中2耳沒有進行聽小骨重建手術。總計手術18耳中,術前平均AC是58.4dB,術前平均ABG是42.5dB;術後平均聽力增加24.7dB,術後平均ABG進步18.6dB。結論:先天性聽小骨異常的表現非常多樣,根據Teunissen & Cremers分類可以幫助我們預測手術的成效,而外科手術治療先天性聽小骨異常,其術後聽力改善是可預期的,尤其是第一型與第三型進步程度最為明顯。

並列摘要


BACKGROUND: Congenital ossicular anomalies are presented as non-progressive conductive hearing loss without history of head trauma or chronic infection. Early diagnosis is very important because most cases can be corrected by appropriate surgical treatment. The main purpose of this study was to analyze the types of congenital ossicular anomalies and compare the postoperative hearing outcomes based on their classification. METHODS: From July 2007 to March 2014 in National Taiwan University Hospital, exploratory tympanotomies were performed on 20 ears of 18 patients with nonprogressive conductive hearing loss but without external ear malformations. The preoperative and postoperative audiological findings, operative findings, types of ossicular chain reconstruction were reviewed. Mean air conduction (MAC) and bone conduction (MBC) thresholds were recorded at 0.5, 1, 2 and 4 k Hz. The types of congenital ossicular anomalies were classified by Teunissen & Cremers system. The evaluation of hearing outcome was based on the MAC gain and mean Air-Bone Gap closure.RESULTS: The preoperative MAC was 58.4 dB with 42.5dB mean Air-Bone Gap. According to Teunissen & Cremers classification system, there were 4 ears in class I, 1 ear in class II, 13 ears in class III and 2 ears in class IV. We performed stapes surgery in 5 ears, type III tympanoplasty in 5 ears and type IV tympanoplasty in 8 ears. Ossicular chain reconstruction was not conducted in 2 ears (class IV). Postoperative hearing improvement revealed MAC gain of 24.7dB and mean air-bone gap closure of 18.6dB. CONCLUSIONS: Congenital ossicular anomalies are variable, but they could be classified into four types. The classification helps in predicting the surgical outcome. Surgical treatment of congenital ossicular anomalies allows good hearing results, especially in cases of Teunissen & Cremers class I and III.

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