特纳综合征镫骨异常伴镫骨肌腱骨化的罕见病例

IF 0.2 Q4 OTORHINOLARYNGOLOGY Indian Journal of Otology Pub Date : 2023-01-01 DOI:10.4103/indianjotol.indianjotol_42_23
Nurul Anis Mohd Fauzi, Ahmad Aizudin Mohd Jamali, Juhara Haron, Nik Adilah Nik Othman, Mohd Khairi Md. Daud
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引用次数: 0

摘要

摘要先天性中耳小听骨异常非常罕见。我们提出了一个患有特纳综合征的患者,他表现为听力损失。纯音听力测定显示双侧中重度听力损失,低频为传导性听力损失,高频为混合性听力损失。两侧的骨阈值在2千赫时下降。患者被诊断为双侧耳硬化,并接受了左中耳手术。术中发现镫骨肌腱骨化,镫骨单足固定,闭孔闭塞。镫骨肌腱分裂,使镫骨足板活动。这导致听力改善,气骨间隙关闭约40-50分贝。镫骨异常和镫骨肌腱骨化导致镫骨固定可导致纯音听力学Carhart缺口听力损失,误诊为耳硬化。分离骨化的镫骨肌腱的手术可能有助于改善患者的听力。
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A Rare Case of Stapes Anomaly with Ossified Stapedius Tendon in a Patient with Turner Syndrome
Congenital anomaly of ossicles in the middle ear is rare. We present a patient with Turner syndrome who presented with hearing loss. Pure-tone audiometry revealed bilateral moderate-to-severe hearing loss with conductive type at the lower and mixed type at the higher frequencies. There was a bone threshold dip at 2 kHz on both sides. The patient was diagnosed as having bilateral otosclerosis and underwent left middle ear surgery. Intraoperative findings showed an ossified stapedius tendon and a fixed monopod stapes with obliterated obturator foramen. The stapedius tendon was divided causing the footplate of the stapes to be mobile. This resulted in improved hearing with the closure of air-bone gap by around 40–50 dB. Stapes anomaly and ossified stapedius tendon causing stapes fixation may results in hearing loss with Carhart’s notch on pure-tone audiometry and misdiagnosed as otosclerosis. Surgery in which the ossified stapedius tendon is divided may help improve the patient’s hearing.
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来源期刊
Indian Journal of Otology
Indian Journal of Otology OTORHINOLARYNGOLOGY-
CiteScore
0.40
自引率
0.00%
发文量
21
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