Abstract：
OBJECTIVE: To examine the clinical features and surgical outcomes in patients with congenital absence of the oval window (CAOW), and to investigate the potential factors that affect audiologic results.
METHODS: A retrospective chart review.
METHODS: A tertiary academic center.
METHODS: A total of 17 ears among 16 patients were confirmed to have CAOW. Among them, 13 ears underwent vestibulotomy for hearing reconstruction. Clinical parameters associated with the hearing outcomes were analyzed.
METHODS: A mean air-bone gap (ABG) after 6-month and long-term follow-up was compared with preoperative measurements.
RESULTS: Intraoperative findings showed that anomalies of the malleus or incus were observed in 11 ears (64.7%), stapes anomalies were present in all ears (100%), and facial nerve anomalies were present in 10 ears (58.8%). Because of unfavorable facial nerve anomalies, hearing reconstruction was aborted in four cases (23.5%). In the hearing reconstruction group, the mean ABG at 6 months postoperation was significantly reduced after compared with the preoperative value (44.0 ± 8.4 dB versus 58.8 ± 9.1 dB, p = 0.006). After dividing ears into a success subgroup (ABG ≤ 30 dB, seven ears) and non-success subgroup (ABG > 30 dB, six ears), the use of a drill during vestibulotomy was significantly related to a poor hearing outcome (100% versus 16.7%, p = 0.015). The long-term follow-up result (mean, 60 mo) revealed no deterioration compared with the 6-month postoperative result. Five ears (29.4%) underwent revision surgery, and three of them showed ABG improvements. No serious complications were reported.
CONCLUSIONS: Vestibulotomy is an effective and safe option for hearing restoration in patients with CAOW, particularly when the use of a drill is not required. The long-term audiologic outcome is also reliable.
METHODS: A retrospective chart review.
METHODS: A tertiary academic center.
METHODS: A total of 17 ears among 16 patients were confirmed to have CAOW. Among them, 13 ears underwent vestibulotomy for hearing reconstruction. Clinical parameters associated with the hearing outcomes were analyzed.
METHODS: A mean air-bone gap (ABG) after 6-month and long-term follow-up was compared with preoperative measurements.
RESULTS: Intraoperative findings showed that anomalies of the malleus or incus were observed in 11 ears (64.7%), stapes anomalies were present in all ears (100%), and facial nerve anomalies were present in 10 ears (58.8%). Because of unfavorable facial nerve anomalies, hearing reconstruction was aborted in four cases (23.5%). In the hearing reconstruction group, the mean ABG at 6 months postoperation was significantly reduced after compared with the preoperative value (44.0 ± 8.4 dB versus 58.8 ± 9.1 dB, p = 0.006). After dividing ears into a success subgroup (ABG ≤ 30 dB, seven ears) and non-success subgroup (ABG > 30 dB, six ears), the use of a drill during vestibulotomy was significantly related to a poor hearing outcome (100% versus 16.7%, p = 0.015). The long-term follow-up result (mean, 60 mo) revealed no deterioration compared with the 6-month postoperative result. Five ears (29.4%) underwent revision surgery, and three of them showed ABG improvements. No serious complications were reported.
CONCLUSIONS: Vestibulotomy is an effective and safe option for hearing restoration in patients with CAOW, particularly when the use of a drill is not required. The long-term audiologic outcome is also reliable.
摘要：
目的：为了检查先天性卵圆窗缺失（CAOW）患者的临床特征和手术结果，并探讨影响听力学结果的潜在因素。
方法：回顾性图表回顾。
方法：三级学术中心。
方法：16例患者共17只耳被证实为CAOW。其中,13耳前庭切开术进行听力重建。分析与听力结果相关的临床参数。
方法：将6个月和长期随访后的平均气-骨间隙（ABG）与术前测量值进行比较。
结果：术中发现，在11只耳朵中观察到锤骨或砧骨异常（64.7％），stapes骨异常存在于所有耳朵（100％），10耳(58.8%)出现面神经异常。由于不利的面神经异常，4例(23.5%)听力重建失败。在听力重建小组中，术后6个月平均ABG较术前明显降低（44.0±8.4dBvs58.8±9.1dB，p=0.006)。将耳朵分为成功亚组（ABG≤30dB，七耳)和非成功亚组(ABG>30dB，六只耳朵)，前庭切开术期间使用钻头与听力不良结果显着相关（100％对16.7％，p=0.015)。长期随访结果(平均，60mo）与术后6个月的结果相比没有恶化。五只耳朵(29.4%)接受了翻修手术,其中3例显示ABG改善。无严重并发症报告。
结论：前庭切开术是CAOW患者听力恢复的一种有效且安全的选择，特别是当不需要使用钻头时。长期的听力学结果也是可靠的。
方法：回顾性图表回顾。
方法：三级学术中心。
方法：16例患者共17只耳被证实为CAOW。其中,13耳前庭切开术进行听力重建。分析与听力结果相关的临床参数。
方法：将6个月和长期随访后的平均气-骨间隙（ABG）与术前测量值进行比较。
结果：术中发现，在11只耳朵中观察到锤骨或砧骨异常（64.7％），stapes骨异常存在于所有耳朵（100％），10耳(58.8%)出现面神经异常。由于不利的面神经异常，4例(23.5%)听力重建失败。在听力重建小组中，术后6个月平均ABG较术前明显降低（44.0±8.4dBvs58.8±9.1dB，p=0.006)。将耳朵分为成功亚组（ABG≤30dB，七耳)和非成功亚组(ABG>30dB，六只耳朵)，前庭切开术期间使用钻头与听力不良结果显着相关（100％对16.7％，p=0.015)。长期随访结果(平均，60mo）与术后6个月的结果相比没有恶化。五只耳朵(29.4%)接受了翻修手术,其中3例显示ABG改善。无严重并发症报告。
结论：前庭切开术是CAOW患者听力恢复的一种有效且安全的选择，特别是当不需要使用钻头时。长期的听力学结果也是可靠的。
