Blood-labyrinth barrier damage mediated by granzymes from cytotoxic lymphocytes results in hearing loss in systemic lupus erythematosus.

Hearing loss (HL) in patients with systemic lupus erythematosus (SLE) has been widely reported, though the underlying mechanisms remain unclear. In this study, we demonstrate significant HL in an imiquimod-induced mouse model of SLE, accompanied by blood-labyrinth barrier (BLB) damage in the stria vascularis (SV). We found that cytotoxic CD8 T lymphocytes and natural killer cells mediate BLB disruption, with granzyme b (Gzmb) acting as the primary factor inducing cell death of the cochlear capillary endothelial cells.

Atomically dispersed copper(I) on tungstosilicic acid for catalytic protection against cisplatin-induced hearing loss.

The employment of platinum-based drugs for cancer chemotherapy, which might yield oxidative stress, is regarded as one main factor leading to hearing loss. The exact molecular mechanisms for cisplatin-induced hearing loss require further clarification, thus limiting the development of FDA-approved therapies. Herein, we mimicked the molecular structure of natural antioxidative enzymes to fabricate a four-oxygen-coordinating copper single-atom nanozyme (Cu SAN) exhibiting good superoxide dismutase and catalase activity, to alleviate the oxidative stress induced by platinum-based drugs.

A Novel Intragenic Deletion Identified by NGS in a BHDS Family and Literature Review.

Birt-Hogg-Dubé syndrome (BHDS, MIM #135150), caused by germline mutations of gene, is a rare autosomal dominant inherited disorder characterized by skin fibrofolliculomas, renal cancer, pulmonary cysts and spontaneous pneumothorax. The syndrome is considered to be under-diagnosed due to variable and atypical manifestations. Herein we present a BHDS family.