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Progression of hearing loss and cochlear implantation in large vestibular aqueduct syndrome.
International Journal of Pediatric Otorhinolaryngology ( IF 1.5 ) Pub Date : 2020-05-23 , DOI: 10.1016/j.ijporl.2020.110133
Nanki Hura 1 , Matthew Stewart 1 , Jonathan Walsh 1
Affiliation  

Objectives

Large vestibular aqueduct syndrome (LVAS) is a congenital inner ear malformation that commonly results in progressive sensorineural hearing loss (SNHL) and cochlear implantation (CI). Though LVAS accounts for approximately 15% of pediatric SNHL, little is known regarding the rate and severity of SNHL in these patients. We sought to characterize the timing of SNHL progression to CI in patients with LVAS.

Methods

We performed a retrospective chart review at our institution from 2000 to 2018 using ICD-10 “large vestibular aqueduct syndrome,” and through identifying patients with CI who had LVAS. Demographic, surgical, and audiometric data were collected. Theoretical CI candidacy was approximated using a pure tone average (PTA) HL threshold of 70 dB.

Results

Of 103 patients, 96 had bilateral LVAS, and 7 had unilateral LVAS. Forty-one patients had bilateral implants, 52 had unilateral implants, and 10 were not implanted. The mean age at first implant was 8.62 years old [95%CI = 6.75,10.49], the mean age at second implant was 12.24 years old [95%CI = 8.33,16.15], and the mean time between implants was 4.37 years [95%CI = 3.02,5.73]. LVAS patients reached HL threshold of 70 dB at a mean age of 5.16 years old (SD = 3.04) for the “worse ear” and 9.08 years old (SD = 4.96) for the “better ear.”

Conclusions

LVAS patients are a heterogenous population of patients, in which some may undergo progression of HL and some may not. Further, there may be a discrepancy in the timing between patients’ theoretical CI candidacy and when they undergo CI. In order to optimize timing of CI, individual monitoring and close observation of LVAS patients is recommended.



中文翻译:

大型前庭导水管综合征的听力损失和耳蜗植入进展。

目标

大型前庭导水管综合征(LVAS)是先天性内耳畸形,通常会导致进行性感觉神经性听力减退(SNHL)和人工耳蜗(CI)。尽管LVAS约占小儿SNHL的15%,但对于这些患者中SNHL的发生率和严重程度知之甚少。我们试图描述LVAS患者SNHL进展为CI的时机。

方法

我们在2000年至2018年间使用ICD-10“大前庭水管综合征”并通过鉴定患有LVAS的CI患者,对我们的机构进行了回顾性图表审查。收集了人口统计,外科手术和听力测验数据。理论CI候选资格使用70 dB的纯音平均(PTA)HL阈值近似。

结果

在103例患者中,有96例患有双侧LVAS,有7例患有单侧LVAS。41例患者有双侧植入物,52例患者有单侧植入物,10例未植入。第一次植入的平均年龄为8.62岁[95%CI = 6.75,10.49],第二次植入的平均年龄为12.24岁[95%CI = 8.33,16.15],两次植入之间的平均时间为4.37年[ 95%CI = 3.02,5.73]。LVAS患者的“耳朵较差”的平均年龄为5.16岁(SD = 3.04),而“更好的耳朵”的平均年龄为HL阈值70 dB(SD = 4.96)。

结论

LVAS患者是异质性患者群体,其中一些可能经历HL的进展而另一些则没有。此外,患者的理论CI候选资格与接受CI的时间之间可能存在差异。为了优化CI的时机,建议对LVAS患者进行单独监测和密切观察。

更新日期:2020-05-23
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