Response to Letter to the Editor Regarding "Hearing Preservation and Spatial Hearing Outcomes after Cochlear Implantation in Children with TMPRSS3 Mutations".
Response to Letter to the Editor Regarding "Hearing Preservation and Spatial Hearing Outcomes after Cochlear Implantation in Children with TMPRSS3 Mutations".
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回复关于“TMPRSS3 突变儿童人工耳蜗植入后的听力保护和空间听力结果”的致编辑信。
DOI：
10.1097/mao.0000000000003946
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发表时间：
2023
期刊：
影响因子：
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通讯作者：
Litovsky,RuthY
中科院分区：
文献类型：
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作者：
Peng,ZEllen;Garcia,Alejandro;Godar,ShellyP;Holt,JeffreyR;Lee,DanielJ;Litovsky,RuthY
致编辑：我们感谢博士。 Carlson 和 Rubinstein 对我们关于两名 TMPRSS3 突变儿童的人工耳蜗植入结果的临床胶囊报告做出了富有见识的评论。我们感谢他们慷慨分享额外的纵向听力数据，这些数据显示了 5 名患有 TMPRSS3 的儿童在接受单侧人工耳蜗植入前后的情况。我们对胶囊报告中关于推荐双侧人工耳蜗植入作为 TMPRSS3 突变儿童干预措施的解释感到担忧。在报告中，我们得出的结论是：“未来的工作有必要研究更大样本的 TMPRSS3 突变（DFNB8 和 DFNB10）儿科患者，为具有听力保留的双侧人工耳蜗植入类似治疗的长期疗效提供额外支持。”信中显示的五个儿童在 500 Hz 处的纵向听力阈值提供了对低频范围渐进变化的有用见解。他们的儿童群体在植入前的较长时间内似乎患有轻度至中度感音神经性听力损失，因此需要更仔细地监测进展情况。在我们对两兄弟的报告中，其中一人在该频率下已达到严重听力损失。我们主张对两名患有与 DFNB8 相关的 TMPRSS3 突变的儿童的临床胶囊报告结果进行更仔细的解释。双侧人工耳蜗植入是一种可行的但不是唯一的治疗选择，因为我们研究中报告的两名儿童都显示出使用双侧人工耳蜗的益处。在儿科患者 TMPRSS3 治疗的现阶段，人工耳蜗植入后低频听力可能进行性恶化的情况下仍然存在两个相关问题：
To the Editor: We thank Drs. Carlson and Rubinstein for their knowledgeable comments on our clinical capsule report on cochlear implantation outcomes from two children with TMPRSS3 mutations. We are grateful for their generous sharing of additional longitudinal audiometric data showing five children with TMPRSS3 before and after receiving unilateral cochlear implantation. We are concerned about the interpretation of our capsule report on recommending bilateral cochlear implantation as intervention for children with TMPRSS3 mutations. In the Report, we concluded that “Future work is warranted to study a larger sample of pediatric patients with TMPRSS3 mutations (both DFNB8 and DFNB10) to provide additional support on the long-term efficacy of similar treatments of bilateral cochlear implants with hearing preservation.” The five children's longitudinal audiometric thresholds at 500 Hz shown in the Letter provided useful insights into the progressive change in the low-frequency range. Their cohort of children seemed to have mild to moderate sensorineural hearing loss over a longer period of time before implantation to warrant more careful monitoring of progression. In our Report of the two siblings, one had reached profound hearing loss at that frequency. We advocate for a more careful interpretation of our clinical capsule report results on two children with TMPRSS3 mutation associated with DFNB8. Bilateral cochlear implantation is a viable, but not the only, treatment option as both children reported in our study showed benefit of using bilateral cochlear implants. At this stage of TMPRSS3 treatment for pediatric patients, two relevant questions remain under the potential progressive deterioration of low-frequency acoustic hearing after cochlear implantation: