Cochlear Nerve Atrophy in Postlingual ANSD: Diagnostic Clue and Implications for Cochlear Implantation.

IF 1.9 3区医学 Q3 CLINICAL NEUROLOGY

Otology & Neurotology Pub Date : 2025-06-02 DOI:10.1097/MAO.0000000000004567

Kyu Ha Shin, Kherlen Altanbayar, Hye-Rim Park, Jin Hee Han, Jiyeon Yang, Pil Geum Jang, Ngoc-Trinh Tran, Ju Ang Kim, Bong Jik Kim, Byung Yoon Choi

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引用次数: 0

Abstract

Objective: To evaluate cochlear nerve (CN) atrophy in postlingual auditory neuropathy spectrum disorder (post-ANSD) versus simple sensorineural hearing loss (sSNHL) and its implications for cochlear implantation (CI).

Design: A retrospective study of 61 CI recipients (15 post-ANSD, 46 sSNHL) assessed CN size through imaging, analyzed auditory performance, and compared outcomes based on etiology, age, and molecular subtypes.

Results: Post-ANSD exhibited more severe CN atrophy despite better pure tone averages than sSNHL. Postsynaptic post-ANSD showed significantly greater CN atrophy than presynaptic cases and sSNHL. Although CN atrophy was pronounced, it did not predict poor CI outcomes within the observed thresholds. Speech scores improved markedly post-CI, with no correlation to CN size.

Conclusion: CN atrophy severity is etiology-dependent and most pronounced in postsynaptic post-ANSD. Severe atrophy could serve as a diagnostic marker of postsynaptic post-ANSD without auditory test clues. Early CI is recommended for optimal outcomes, emphasizing timely intervention in post-ANSD patients.

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舌后ANSD的耳蜗神经萎缩：诊断线索及对人工耳蜗植入的意义。

目的：评价舌后听神经病变谱障碍（后ansd）与单纯感音神经性听力损失（sSNHL）的耳蜗神经萎缩及其对人工耳蜗植入（CI）的影响。设计：对61例CI受者（15例ansd后，46例sSNHL）进行回顾性研究，通过影像学评估CN大小，分析听觉表现，并根据病因、年龄和分子亚型比较结果。结果：与sSNHL相比，ansd后的CN萎缩更严重，尽管纯音平均值更好。与突触前病例和sSNHL相比，突触后ansd的CN萎缩明显更大。虽然CN萎缩很明显，但在观察到的阈值内，它并不能预测较差的CI结果。言语评分在ci后显著提高，与CN大小无关。结论：神经网络萎缩严重程度与病因有关，在突触后ansd中最为明显。在没有听觉测试线索的情况下，严重萎缩可作为突触后ansd的诊断标志。推荐早期CI以获得最佳结果，强调ansd后患者的及时干预。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Otology & Neurotology 医学-耳鼻喉科学

CiteScore

3.80

自引率

14.30%

发文量

509

审稿时长

3-6 weeks

期刊介绍： Otology & Neurotology publishes original articles relating to both clinical and basic science aspects of otology, neurotology, and cranial base surgery. As the foremost journal in its field, it has become the favored place for publishing the best of new science relating to the human ear and its diseases. The broadly international character of its contributing authors, editorial board, and readership provides the Journal its decidedly global perspective.