Department of Otorhinolaryngology, Yonsei University College of Medicine, Seoul, Republic of Korea.
Department of Otorhinolaryngology, Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Republic of Korea.
Audiol Neurootol. 2021;26(2):111-120. doi: 10.1159/000508858. Epub 2020 Sep 2.
Fluctuating hearing loss is a distinctive feature caused by SLC26A4 variants. We investigated whether cochlear implantation had protective or deleterious effect on hearing fluctuation in patients with biallelic SLC26A4 variants.
Patients with biallelic SLC26A4 variants (N = 16; age = 10.24 ± 9.20 years) who had unilateral cochlear implantation and consecutive postsurgical, bilateral pure-tone audiograms more than 3 times were selected. We retrospectively reviewed the patients' medical records from 2008 to 2019 obtained from a tertiary medical center and used the auditory threshold change (Shift) over time as a marker of hearing fluctuation. Fluctuation events were counted, and the Shift of the implanted and contralateral ears was compared using logistic regression with a generalized estimating equation and linear mixed model. A total of 178 values were included.
The odds of fluctuating hearing frequency were 11.185-fold higher in the unimplanted ears than in the implanted ears postoperatively (p = 0.001). The extent of fluctuation at 250 and 500 Hz was also significantly lower in the implanted ears than in the unimplanted ears after adjusting for every other effect (p = 0.003 and p < 0.001, respectively). Notably, higher residual hearing was rather associated with lesser fluctuation in frequency and the extent of fluctuation at 500 Hz, indicating residual hearing function is not the positive predictor for hearing fluctuation.
In patients with biallelic SLC26A4 variants, cochlear implantation may reduce the frequency and extent of hearing fluctuations.
由 SLC26A4 变异引起的波动性听力损失是一个显著特征。我们研究了双侧 SLC26A4 变异患者的人工耳蜗植入是否对听力波动有保护或有害作用。
选择双侧 SLC26A4 变异（N=16；年龄=10.24±9.20 岁）且单侧植入人工耳蜗并在术后进行了 3 次以上连续双侧纯音测听的患者。我们回顾性分析了 2008 年至 2019 年在一家三级医疗中心从患者病历中获得的资料，并使用随时间变化的听阈变化（Shift）作为听力波动的标志物。计算波动事件的次数，并使用广义估计方程和线性混合模型的逻辑回归比较植入耳和对侧耳的 Shift。共纳入 178 个值。
术后未植入耳发生听力波动的频率比植入耳高 11.185 倍（p=0.001）。在调整其他所有因素后，植入耳的 250 Hz 和 500 Hz 的波动幅度也明显低于未植入耳（p=0.003 和 p<0.001）。值得注意的是，残余听力越高，频率波动和 500 Hz 波动幅度越低，表明残余听力功能并不是听力波动的正预测因子。
在双侧 SLC26A4 变异患者中，人工耳蜗植入可能会减少听力波动的频率和幅度。
