Study Models for Non-Syndromic Hearing Loss

TL;DR

This review discusses models used to study non-syndromic hearing loss, aiming to improve therapies and understanding of inner ear physiology.

Contribution

The paper provides a detailed analysis of cellular, animal, and organoid models for studying non-syndromic hearing loss.

Findings

Approximately 150 genes are responsible for non-syndromic deafness.

Current hearing aids and cochlear implants have limited effectiveness in noisy environments.

Various models are used to study the inner ear and develop advanced therapies.

Abstract

Hearing loss is the most common sensory disorder; It is estimated that nearly 2.5 billion people will have some degree of hearing loss by 2050. Although the causes are diverse, a significant proportion of cases have a genetic origin, which is the main focus of the models discussed in this review. Many loci corresponding to deafness genes have already been identified, and approximately 150 genes are responsible for non-syndromic deafness, which is characterized by partial or total hearing loss that is not associated with other signs or symptoms. Although hearing aids and cochlear implants are widely available today, their effectiveness is often limited, especially in noisy environments, prompting the development of advanced therapies for hearing loss. To evaluate new therapies and improve our understanding of hearing physiology, various models, including cellular, animal, and organoid models, are used to study the inner ear. In this review, we present these different models in detail, with their respective strengths and limitations. This analysis will be particularly valuable in helping researchers to identify the most appropriate model for their specific research questions and to justify their choices from an ethical perspective.