Department of Otolaryngology and Communication Enhancement, Children's Hospital Boston, Boston, Massachusetts, USA.
Laryngoscope. 2011 Jan;121(1):220-5. doi: 10.1002/lary.21184.
OBJECTIVE/HYPOTHESIS: To explore the characteristics of vestibular evoked myogenic potential (VEMP) in children with enlarged vestibular aqueduct (EVA) and to determine the diagnostic value of VEMP testing for this particular inner ear structural anomaly.
Retrospective cohort study in a pediatric tertiary care facility.
A total of 25 pediatric cases (37 ears) of EVA were identified with complete records, including otologic evaluation, CT scan of the temporal bone, and audiologic assessment. Results of audiometry, tympanometry, and VEMP testing were analyzed.
Hearing loss was found in 97% (36/37) of the ears with EVA. Airbone gaps (conductive components) were found in all hearing losses with normal middle ear pressure and mobility. Abnormally low threshold VEMP responses were found in 92% (34/37) of the ears with EVA. VEMP responses were absent unilaterally in three EVA patients who had vestibular complaints. No clear correlation was found between the size of EVA and the audiologic findings.
The presence of airbone gaps in children with EVA was found without apparent middle ear pathology. Characteristics of VEMP in EVA were lower thresholds and higher amplitudes despite of the presence of airbone gaps. The abnormally low threshold VEMP responses suggested a "third" window effect in the pathologic condition of EVA. Unilateral absence of VEMP may implicate peripheral vestibular impairment. The findings from our study are helpful in clinical evaluation of young children who usually give limited and ambiguous input regarding their hearing and vestibular problems.
目的/假设：探讨儿童前庭导水管扩大（EVA）患者前庭诱发肌源性电位（VEMP）的特点，并确定 VEMP 测试对此种特定内耳结构异常的诊断价值。
在一家儿科三级保健机构进行的回顾性队列研究。
共确定了 25 例（37 耳）EVA 儿科病例，这些病例均有完整的记录，包括耳科评估、颞骨 CT 扫描和听力学评估。分析了听力测试、鼓室图和 VEMP 测试的结果。
发现 97%（36/37）的 EVA 耳有听力损失。中耳压力和活动正常的所有听力损失中均发现气骨间隙（传导成分）。92%（34/37）的 EVA 耳的 VEMP 反应异常低阈值。3 例有前庭症状的 EVA 患者单侧 VEMP 缺失。EVA 的大小与听力结果之间未发现明显相关性。
在 EVA 患儿中发现了气骨间隙的存在，而没有明显的中耳病理。尽管存在气骨间隙，但 EVA 中的 VEMP 特征为较低的阈值和较高的振幅。异常低阈值 VEMP 反应提示 EVA 病理状况下存在“第三”窗口效应。单侧 VEMP 缺失可能提示周围性前庭功能障碍。我们的研究结果有助于对通常对其听力和前庭问题提供有限和模糊信息的幼儿进行临床评估。
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