Cochlin-tomoprotein (CTP) detection test identifies traumatic perilymphatic fistula due to penetrating middle ear injury

Ikezono, Tetsuo; Shindo, Susumu; Sekine, Kuwon; Shiiba, Kyoko; Matsuda, Han; Kusama, Kaoru; Koizumi, Yasuo; Sugizaki, Kazuki; Sekiguchi, Satomi; Kataoka, Ryohei; Pawankar, Ruby; Baba, Shunkichi; Yagi, Toshiaki; Okubo, Kimihiro

doi:10.3109/00016489.2011.575795

Cited by 18 publications

(14 citation statements)

References 18 publications

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“…Considering that the LCCL domain is thought to have innate immune function in the spleen, these results suggest that the uncleaved LCCL domain may not properly function in response to inflammation in the inner ear. Given that a low molecular weight cochlin isoform p16 representing the LCCL domain is abundant in the perilymph of the healthy inner ear, patients harboring COCH mutations may not produce enough LCCL peptides in the perilymph [Ikezono et al., , , ]. The consequence of this process may be the accumulation of endotoxin in the inner ear over several decades, which would attack and slowly destroy the cochlea and other structures, eventually resulting in hearing loss.…”

Section: Discussionmentioning

confidence: 99%

NovelCOCHp.V123E Mutation, Causative of DFNA9 Sensorineural Hearing Loss and Vestibular Disorder, Shows Impaired Cochlin Post-Translational Cleavage and Secretion

et al. 2015

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DFNA9 is an autosomal dominant disorder characterized by late-onset, non-syndromic hearing loss, and vestibular dysfunction. Mutations in the COCH (coagulation factor C homology) gene encoding cochlin are etiologically linked to DFNA9. Previous studies have shown that cochlin is cleaved by aggrecanase-1 during inflammation in the spleen and that the cleaved LCCL domain functions as an innate immune mediator. However, the physiological role of cochlin in the inner ear is not completely understood. Here, we report that cochlins containing DFNA9-linked mutations (p.P51S, p.V66G, p.G88E, p.I109T, p.W117R, p.V123E, and p.C162Y) demonstrate reduced cleavage by aggrecanase. Notably, in families affected with DFNA9, we found a novel COCH mutation causing p.V123E substitution in cochlin, which significantly reduced protein susceptibility to cleavage by aggrecanase (to about 20.5% of the wild-type). These results suggest that the impaired post-translational cleavage of cochlin mutants may be associated with pathological mechanisms underlying DFNA9-related sensorineural hearing loss.

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Section: Discussionmentioning

confidence: 99%

NovelCOCHp.V123E Mutation, Causative of DFNA9 Sensorineural Hearing Loss and Vestibular Disorder, Shows Impaired Cochlin Post-Translational Cleavage and Secretion

et al. 2015

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“…Cochlin-tomoprotein (CTP) is a novel perilymph-specific protein, not found in CSF, saliva, or serum [56] and found in a perilymph leak in a patient with a penetrating ear drum injury [57] and in some cases of PLF [58]. …”

Section: Operative Confirmation Of Plfmentioning

confidence: 99%

Perilymph Fistula: Fifty Years of Controversy

Hornibrook

2012

ISRN Otolaryngology

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Perilymph fistula (PLF) is defined as a leak of perilymph at the oval or round window. It excludes other conditions with “fistula” tests due to a dehiscent semi circular canal from cholesteatoma and the superior canal dehiscence syndrome. It was first recognized in the early days of stapedectomy as causing disequilibrium and balance problems before sealing of the stapedectomy with natural tissue became routine. It then became apparent that head trauma and barotraumatic trauma from flying or diving could be a cause of PLF. Descriptions of “spontaneous” PLF with no trauma history followed. A large literature on PLF from all causes accumulated. It became an almost emotional issue in Otolaryngology with “believers” and “nonbelievers.” The main criticisms are a lack of reliable symptoms and diagnostic tests and operative traps in reliably distinguishing a perilymph leak from local anaesthetic. There are extensive reviews on the whole topic, invariably conveying the authors' own experiences and their confirmed views on various aspects. However, a close examination reveals a disparity of definitions and assumptions on symptoms, particularly, vestibular. This is an intentionally provocative paper with suggestions on where some progress might be made.

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“…Among these cases, each of these causes should be discussed separately; e.g., 50 of 128 cases were related to cholesteatoma, where the CTP detection test can be utilized for the staging of fistula depth induced by chronic inflammation (in preparation). In the case of poststapes surgery hearing loss due to dislocation of the piston, the CTP detection test could be used to identify perilymph leakage prior to exploratory tympanotomy [11], while in the case of direct middle ear trauma, the CTP test was useful in deciding the surgical treatment [3].…”

Section: Fistula Signmentioning

confidence: 99%

“…Its characteristics have already been reported in detail [1][2][3]8]. We initially developed a Western blotting-based CTP detection test for PLF diagnosis, and improved the throughput and sensitivity of this test by using an ELISA-based CTP detection kit.…”

Section: Fistula Signmentioning

confidence: 99%

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A nationwide multicenter study of the Cochlin tomo-protein detection test: clinical characteristics of perilymphatic fistula cases

Matsuda

Sakamoto

Matsumura

et al. 2017

Acta Oto-Laryngologica

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In addition to the CTP-positive rate, audio-vestibular symptoms were compared between CTP-positive and -negative cases.Results: 8-50% of patients in category 1 (trauma, middle and inner ear disease cases), and about 20% of those in categories 2, 3 and 4 (external origin antecedent events, internal origin antecedent events, and without antecedent event, respectively) were positive for CTP. In category 1 cases, the earlier tested samples showed a higher CTP-positive rate, whereas no differences were observed in categories 2, 3 or 4. The characteristic clinical features in the earlier tested cases were nystagmus and fistula sign in CTP test-positive cases in category 1, and streaming water-like tinnitus in those in categories 2, 3 and 4.Conclusion: The present study clarified that CTP detection test-positive patients exist at considerable rates among patients with inner ear-related manifestations.ARTICLE HISTORY

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Cochlin-tomoprotein (CTP) detection test identifies traumatic perilymphatic fistula due to penetrating middle ear injury

Cited by 18 publications

References 18 publications

NovelCOCHp.V123E Mutation, Causative of DFNA9 Sensorineural Hearing Loss and Vestibular Disorder, Shows Impaired Cochlin Post-Translational Cleavage and Secretion

NovelCOCHp.V123E Mutation, Causative of DFNA9 Sensorineural Hearing Loss and Vestibular Disorder, Shows Impaired Cochlin Post-Translational Cleavage and Secretion

Perilymph Fistula: Fifty Years of Controversy

A nationwide multicenter study of the Cochlin tomo-protein detection test: clinical characteristics of perilymphatic fistula cases

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