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双边先天性脚脚板固定,缺少脚肌

Eric Y Du¹, Carson K Gates², Rohan Patel¹

¹Department of Otolaryngology-Head and Neck Surgery, University of Missouri School of Medicine, Columbia, MO, USA.

The Permanente journal

|February 27, 2026

概括

带有缺少的足板固定是罕见的,特别是在双边时. 在患有这种独特病症的儿科患者身上,分阶段性管切除术成功恢复了功能性听力.

科学领域:

耳鼻喉科耳鼻喉科耳鼻喉科
儿科听力学儿科听力学
手术创新在外科创新.

背景情况:

先天性带脚板固定 (CSFF) 是儿科传导性听力损失的罕见原因.
双边CSFF与缺席的状肌并发是非常罕见的,以前没有报告过.
该案涉及一名15岁的男性,患有复杂的先天性异常.

研究的目的:

报告第一个双边CSFF病例,缺少状肌.
描述手术管理和结果.
突出复杂的先天性骨异常的听力恢复的潜力.

主要方法:

双边CSFF的外科检查,缺少状肌和形骨.
阶段性双边节节切除术手术.
术后听力测量评估气骨间隙和语音接收值.

主要成果:

空气与骨间隔显著改善 (从64dB到19dB右边,58dB到15dB左边).
功能性听力恢复与改善的语音接收值 (25dB右,15dB左).
尽管有复杂的先天性骨异常,但听力结果仍然很好.

结论:

这一案例代表了双边CSFF的第一个报告,其中缺少状肌.
在特定的儿科病例中,手术探索和管切除术对于恢复听力至关重要.
即使是复杂的先天性骨发育不良,Stapedotomy也可以提供有意义的听力改善.

关键词:

导电性听力损失是一种听力损失.遗传性骨质异常是先天的产生的先天性纹的固定.没有状肌的缺失.这是一次脚切除术 (Stepedotomy).

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