Autosomal dominant non-syndromic hearing loss (DFNA): a comprehensive narrative review

M Aldè, G Cantarella, D Zanetti, L Pignataro… - Biomedicines, 2023 - mdpi.com
Autosomal dominant non-syndromic hearing loss (HL) typically occurs when only one
dominant allele within the disease gene is sufficient to express the phenotype. Therefore …

[HTML][HTML] Age-related hearing loss: the link between inflammaging, immunosenescence, and gut dysbiosis

D Kociszewska, S Vlajkovic - International Journal of Molecular Sciences, 2022 - mdpi.com
This article provides a theoretical overview of the association between age-related hearing
loss (ARHL), immune system ageing (immunosenescence), and chronic inflammation …

Cochlear immune response in presbyacusis: a focus on dysregulation of macrophage activity

K Noble, LS Brown, P Elvis, H Lang - … of the Association for Research in …, 2022 - Springer
Age-related hearing loss, or presbyacusis, is a prominent chronic degenerative disorder that
affects many older people. Based on presbyacusis pathology, the degeneration occurs in …

NLRP3 inflammasome activation in human vestibular schwannoma: Implications for tumor-induced hearing loss

JE Sagers, MI Sahin, IS Moon, SG Ahmed… - Hearing research, 2019 - Elsevier
Vestibular schwannoma (VS) is the fourth most common intracranial tumor, arising from
neoplastic Schwann cells of the vestibular nerve and often causing debilitating …

Cochlear inflammaging in relation to ion channels and mitochondrial functions

P Bazard, J Pineros, RD Frisina, MA Bauer, AA Acosta… - Cells, 2021 - mdpi.com
The slow accumulation of inflammatory biomarker levels in the body—also known as
inflammaging—has been linked to a myriad of age-related diseases. Some of these include …

Gain of function mutation and inflammasome driven diseases in human and mouse models

MD Cordero, E Alcocer-Gómez, B Ryffel - Journal of autoimmunity, 2018 - Elsevier
Activation of the NLRP3 inflammasome, a multiprotein complex, leading to caspase
activation with production of proinflammatory IL-1β represents a major pathway of …

Oridonin ameliorates noise-induced hearing loss by blocking NLRP3-NEK7 mediated inflammasome activation

M Li, Y Zhang, S Qiu, W Zhuang, W Jiang… - International …, 2021 - Elsevier
Inflammation is involved in noise-induced hearing loss (NIHL), but the mechanism is still
unknown. The NOD-like receptor family pyrin domain containing 3 (NLRP3) inflammasome …

Auditory and vestibular characteristics of NLRP3 inflammasome related autoinflammatory disorders: Monogenic hearing loss can be improved by anti-interleukin-1 …

H Nakanishi, S Yamada, J Kita, D Shinmura… - Frontiers in …, 2022 - frontiersin.org
Inflammasomes are large multimeric protein complexes which regulate the activation of the
proinflammatory cytokines interleukins-1β and− 18 and inflammatory cell death called …

Hearing Loss as the Main Clinical Presentation in NLRP3-Associated Autoinflammatory Disease

D Oziębło, ML Leja, A Jeznach… - Frontiers in …, 2022 - frontiersin.org
The NLRP3 gene mutations are the cause of autosomal dominant autoinflammatory
disorders (NLRP3-AID). Recently, hearing loss (HL) has been found to be the sole or major …

Otological aspects of NLRP3-related autoinflammatory disorder focusing on the responsiveness to anakinra

BJ Kim, YH Kim, S Lee, JH Han, SY Lee… - …, 2021 - academic.oup.com
Objectives Gradually progressive sensorineural hearing loss (SNHL) is a prevalent sensory
defect. It is generally untreatable, making rehabilitation by hearing aid or cochlear …