Dataset Information

TMPRSS3 expression is limited in spiral ganglion neurons: implication for successful cochlear implantation.

ABSTRACT:

Background

It is well established that biallelic mutations in transmembrane protease, serine 3 (TMPRSS3) cause hearing loss. Currently, there is controversy regarding the audiological outcomes after cochlear implantation (CI) for TMPRSS3-associated hearing loss. This controversy creates confusion among healthcare providers regarding the best treatment options for individuals with TMPRSS3-related hearing loss.

Methods

A literature review was performed to identify all published cases of patients with TMPRSS3-associated hearing loss who received a CI. CI outcomes of this cohort were compared with published adult CI cohorts using postoperative consonant-nucleus-consonant (CNC) word performance. TMPRSS3 expression in mouse cochlea and human auditory nerves (HAN) was determined by using hybridisation chain reaction and single-cell RNA-sequencing analysis.

Results

In aggregate, 27 patients (30 total CI ears) with TMPRSS3-associated hearing loss treated with CI, and 85% of patients reported favourable outcomes. Postoperative CNC word scores in patients with TMPRSS3-associated hearing loss were not significantly different than those seen in adult CI cohorts (8 studies). Robust Tmprss3 expression occurs throughout the mouse organ of Corti, the spindle and root cells of the lateral wall and faint staining within <5% of the HAN, representing type II spiral ganglion neurons. Adult HAN express negligible levels of TMPRSS3.

Conclusion

The clinical features after CI and physiological expression of TMPRSS3 suggest against a major role of TMPRSS3 in auditory neurons.

SUBMITTER: Chen YS

PROVIDER: S-EPMC10245338 | biostudies-literature | 2022 Dec

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

TMPRSS3 expression is limited in spiral ganglion neurons: implication for successful cochlear implantation.

Chen Yuan-Siao YS Cabrera Ernesto E Tucker Brady J BJ Shin Timothy J TJ Moawad Jasmine V JV Totten Douglas J DJ Booth Kevin T KT Nelson Rick F RF

Journal of medical genetics 20220812 12

<h4>Background</h4>It is well established that biallelic mutations in transmembrane protease, serine 3 (<i>TMPRSS3</i>) cause hearing loss. Currently, there is controversy regarding the audiological outcomes after cochlear implantation (CI) for <i>TMPRSS3</i>-associated hearing loss. This controversy creates confusion among healthcare providers regarding the best treatment options for individuals with <i>TMPRSS3</i>-related hearing loss.<h4>Methods</h4>A literature review was performed to identi ...[more]

PMID: 35961784

Similar Datasets

Project description:Conservation of a patient's residual hearing and prevention of fibrous tissue/new bone formation around an electrode array are some of the major challenges in cochlear implant (CI) surgery. Although it is well-known that fibrotic tissue formation around the electrode array can interfere with hearing performance in implanted patients, and that associated intracochlear inflammation can initiate loss of residual hearing, little is known about the molecular and cellular mechanisms that promote this response in the cochlea. In vitro studies in neonatal rats and in vivo studies in adult mice were performed to gain insight into the pro-inflammatory, proliferative, and remodeling phases of pathological wound healing that occur in the cochlea following an electrode analog insertion. Resident Schwann cells (SC), macrophages, and fibroblasts had a prominent role in the inflammatory process in the cochlea. Leukocytes were recruited to the cochlea following insertion of a nylon filament in adult mice, where contributed to the inflammatory response. The reparative stages in wound healing are characterized by persistent neuro-inflammation of spiral ganglion neurons (SGN) and expression of regenerative monocytes/macrophages in the cochlea. Accordingly, genes involved in extracellular matrix (ECM) deposition and remodeling were up-regulated in implanted cochleae. Maturation of scar tissue occurs in the remodeling phase of wound healing in the cochlea. Similar to other damaged peripheral nerves, M2 macrophages and de-differentiated SC were observed in damaged cochleae and may play a role in cell survival and axonal regeneration. In conclusion, the insertion of an electrode analog into the cochlea is associated with robust early and chronic inflammatory responses characterized by recruitment of leukocytes and expression of pro-inflammatory cytokines that promote intracochlear fibrosis and loss of the auditory hair cells (HC) and SGN important for hearing after CI surgery.

Dataset Information

TMPRSS3 expression is limited in spiral ganglion neurons: implication for successful cochlear implantation.

Background

Methods

Results

Conclusion

Publications

TMPRSS3 expression is limited in spiral ganglion neurons: implication for successful cochlear implantation.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets