Bae Seong Hoon, Ikezono Tetsuo, Park Haeng Ran, Kim Hyoyeol, Matsumura Tomohiro, Saito Shiho, Maeda Yukihide, Matsuda Han, Jung Jinsei
Department of Otorhinolaryngology, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea.
Institute for Lee Won Sang Yonsei Ear Science, Seoul, Republic of Korea.
Front Neurol. 2025 Apr 2;16:1527311. doi: 10.3389/fneur.2025.1527311. eCollection 2025.
Cochlin is the most abundant protein in the inner ear. The cleaved N-terminal domain of cochlin, known as LCCL and referred to as CTP (cochlin tomoprotein) in clinical biomarker usage, is a perilymph-specific protein and is widely used as a biomarker to detect perilymph leakage. However, little is known about the secretion or presence of LCCL in the middle ear, even though it can result in false positives when using LCCL as a biomarker for perilymph leakage.
We conducted translational research in humans and mice. A retrospective observational study was conducted on human patients who underwent multiple CTP tests after tympanostomy. In parallel, an experimental study on cochlin and its cleaved product, LCCL, was performed in mice.
We found the exceptionally elevated level of CTP within 10 days after tympanostomy in humans regardless of the presence of definite perilymph leakage. In addition, we identified LCCL in the middle ear after tympanostomy in mice. The concentration of LCCL peaked at three days post-tympanic injury. Importantly, the origin of LCCL in the middle ear lavage was not from the inner ear but is secreted from the middle ear space especially the annular ligament, suggesting it functions as an innate immune response in the middle ear.
Tympanostomy for the CTP test results in a false positive when the sampling is delayed. While LCCL is a reliable biomarker for clinically detecting perilymph fistula, the timing of its application should be carefully considered to avoid false-positive results.
耳蜗蛋白是内耳中含量最丰富的蛋白质。耳蜗蛋白的裂解N端结构域,即LCCL,在临床生物标志物应用中被称为CTP(耳蜗蛋白断层蛋白),是一种外淋巴特异性蛋白,被广泛用作检测外淋巴漏的生物标志物。然而,尽管在将LCCL用作外淋巴漏的生物标志物时可能会导致假阳性,但关于LCCL在中耳中的分泌或存在情况却知之甚少。
我们在人类和小鼠中开展了转化研究。对鼓膜造口术后接受多次CTP检测的人类患者进行了一项回顾性观察研究。同时,在小鼠中对耳蜗蛋白及其裂解产物LCCL进行了实验研究。
我们发现,无论是否存在明确的外淋巴漏,人类鼓膜造口术后10天内CTP水平异常升高。此外,我们在小鼠鼓膜造口术后的中耳中鉴定出了LCCL。LCCL浓度在鼓膜损伤后三天达到峰值。重要的是,中耳灌洗中LCCL的来源并非内耳,而是从中耳间隙尤其是环状韧带分泌的,这表明它在中耳中发挥着先天性免疫反应的作用。
当采样延迟时,用于CTP检测的鼓膜造口术会导致假阳性结果。虽然LCCL是临床上检测外淋巴瘘的可靠生物标志物,但应仔细考虑其应用时机,以避免出现假阳性结果。