目的：了解人工耳蜗植入法康复大前庭水管综合征(LVAS)重度和极重度听力损失的效果。
Objective: to know the rehabilitation fors ev ere to profound hearing loss of large vestibular aqueduct syndrome (LVAS) with cochlear implants.
目的分析伴前庭水管扩大(eva)的遗传性耳聋患者SLC26 A 4基因的突变特点，探讨该病行人工耳蜗植入时应注意的问题。
Objective to probe the problems that should be paid attention to in cochlea implantation for inherited deafness patients with EVA and to detect the SLC26A4 gene mutation underlying the disease.
目的：探讨大前庭导水管综合征的诊断、听力学表现及治疗。
Objective: To explore the diagnosis, hearing and treatment of large vestibular aqueduct syndrome.
CT能准确地显示前庭导水管扩大，对指导治疗有帮助。
Ct can accurately show the large vestibular aqueduct, and is helpful to its therapy.
目的评价横轴位高分辨率CT扫描对前庭导水管扩大畸形的诊断价值。
Objective To assess the value of axial high resolution computed tomography(HRCT)scans in the diagnosis of the large vestibular aqueduct malformation.
方法：对18例经高分辨率CT证实为双侧大前庭导水管综合征患者的诊疗过程进行回顾性分析。
Methods: The clinical process of diagnosis and treatment of 18 patients with LVAS confirmed by high resolution ct were retrospectively analyzed.
目的探讨儿童大前庭导水管综合征的听力学特点。
ObjectiveTo investigate the audiological characteristics of children with large vestibular aqueduct syndrome (LVAS).
目的：探讨大前庭导水管综合征(LVAS)的诊断、听力学表现及治疗。
Objective: To explore the diagnosis, hearing and treatment of large vestibular aqueduct syndrome (LVAS).
大前庭导水管综合征纯音听阈可以有低范围的气骨导差，但原因不明。
An air-bone gap might exist in ATPT of LVAS at low frequencies, while the underlying cause remains unclear.
结论大前庭导水管综合征病儿初次发病时一般为双侧中重度以上感音神经性听力损失，随后波动性听力逐渐下降直至全聋。
ConclusionGenerally, moderate or severe neurosensory hearing loss of both ears occurs at the initial episode of LVAS and aggravates gradually to total deafness.
结论大前庭导水管综合征病儿初次发病时一般为双侧中重度以上感音神经性听力损失，随后波动性听力逐渐下降直至全聋。
ConclusionGenerally, moderate or severe neurosensory hearing loss of both ears occurs at the initial episode of LVAS and aggravates gradually to total deafness.
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