先天性聽小骨異常

背景：先天性聽小骨異常包括單一聽小骨異常或者合併多個聽小骨異常。因為此類病例稀少，故欲了解此類病患的手術發現以及其手術後聽力情形。方法：採病歷回顧方式，蒐集光田醫院2002年至2012年出院診斷為先天性聽小骨異常的病患，並且排除非單純先天性聽小骨異常的病患（如：Branchio-oto-renal syndrome）以及再次手術的病患。根據Teunissen & Cremers分類方法，將病人分類，並記錄病人的術前純音聽力檢查、術中發現、手術方法、以及術後6個月純音聽力檢查，其中純音聽力檢查，氣導與骨導閾值的記錄頻率採5百，1千及2千赫茲之平均值，術前純音聽力檢查採最靠近手術日的聽力檢查，術後純音聽力檢查定為術後6個月之追蹤結果。術後純音聽力檢查之氣骨導差在20 dB以內視為手術成功。結果：總共18個病人，21個耳朵，男：女=12：6，左耳：右耳=10：11，平均手術年齡為19.8歲。根據Teunissen & Cremers分類，病人的分佈為：Class Ⅰ: 7耳（33.3%）；Class Ⅱ: 2耳（9.5%）；Class Ⅲ: 9耳（42.8%）；Class Ⅳ: 3耳（14%）。4耳於術中沒有進行聽小骨重建手術，其中1耳為Class Ⅲ；3耳為Class Ⅳ，其他有9耳接受鐙骨鑿窗手術（stapedotomy），8耳接受聽小骨成形手術（ossiculoplasty）。平均術後氣骨導差（ABG）以及手術前後的氣骨導差間距（ΔABG）為：Class Ⅰ: 16.4dB/18.8 dB；Class Ⅱ: 30.8 dB/17.5 dB；Class Ⅲ: 16.4 dB/30.2 dB，成功率：Class Ⅰ: 71.4% (5/7)；Class Ⅱ:50% (1/2)；Class Ⅲ: 62.5% (5/8)；整體成功率：64.7% (11/17)。Class Ⅳ因為病人皆無進行重建手術，所以不討論術後的ABG以及成功率。結論：綜合台灣本土研究，先天性聽小骨異常在Teunissen & Cremers分類中，第三型所佔比例最多為46.6%，其次為第一型佔24.1%。第四型屬於比較無法進行聽力重建的類型，建議配戴骨傳導助聽器，其所佔比例為13.8%。本研究中最常見的中耳異常部位則是鐙骨足板固定，約佔42.8%。而在第一型到第三型患者中整體平均術後氣骨導差可改善約24 dB，其中又以第三型患者改善最多平均約為30 dB。

關鍵字

先天性聽小骨異常；人工聽小骨

並列摘要

BACKGROUND: Congenital ossicular anomaly is a rare disease entity which comprises both isolated and multiple ossicular chain anomalies. We collected patients from our hospital and analyzed their surgical outcome. METHODS: Retrospective cohort was developed from congenital ossicular anomaly patients in our hospital between 2002 and 2012. Patients with other congenital syndrome or having previous surgeries were excluded from this study. The types of congenital ossicular anomaly were classified by Teunissen & Cremers classification. Preoperative pure tone audiometry (last exam before operation), surgical findings, surgical procedures, and postoperative pure tone audiometry (6 month after operation) were reviewed. Mean air conduction and bone conduction thresholds were recorded at 0.5, 1, 2 KHz. A closure of the ABG (air-bone gap) to within 20 dB was considered a successful outcome. RESULTS: Eighteen patients (12 male and 6 female) with total twenty-one ears were recruited in this study. The average age of subjects is 19.8 years (range 7~53 years). According to Teunissen & Cremers classification, there were 7 ears in class I, 2 ears in class II, 9 ears in class III and 3 ears in class IV. There were 4 ears without ossicular chain reconstruction: 1 class III and 3 class IV. Stapedotomy was performed in 9 ears and ossiculoplasty in 8 ears. Mean postoperative ABG and ΔABG (the improvement ABG) were class I: 16.4 dB/18.8 dB; Class II: 30.8 dB/17.5 dB; Class III: 16.4 dB/30.2 dB. Success rates were 71.4%; 50%; 62.5%, respectively. Total success rate was 64.7% (11/17). CONCLUSIONS: Based on recent Taiwan studies, most of Taiwan cases are class III (46.6%), followed by class I (24.1%). Class IV (13.8%) is very difficult for ossicular reconstruction so bone conduction hearing aid is recommended for them. Based on our study, the most common middle ear anomaly is footplate fixation (42.8%). The average ΔABG in class I, II, III is around 24 dB, while the most favorable outcome is class III (ΔABG, 30 dB).

並列關鍵字

congenitall ossicular anomaly ； ossicular replacement prosthesis

國際替代計量

先天性聽小骨異常

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