From Genetics to the Outside World: Hearing Dysfunction from Two Perspectives

  Hearing is not just sound entering your ears and going to your brain. It involves a process called “transduction,” which transforms stimuli into electrical signals that your brain can understand. Sometimes hearing loss or interference can be caused by damage to the many working parts of the ear that contribute to this process. In a recent study by Yang et al. (2025) titled, “Critical role of hepsin/TMPRSS1 in hearing and tectorial membrane morphogenesis: insights from transgenic mouse models,” the gene Hepsin was studied for its function in building the inner ear. Additionally, an article written by Melinda Moyer titled, “What Can You Do About Tinnitus?” covers a specific form of hearing interference and the multiple causes and treatments for it. With both of these resources, we get perspectives on both the structural biology of the ear, how hearing loss can affect daily life, and the clinical implications for it.

           Recently, Yang et al. (2025) examined the function of the gene Hepsin (TMPRSS1) in hearing abilities. The Hepsin gene is responsible for creating proteins with the ability to splice, which allows for an essential part of the inner ear, the tectorial membrane (TM), to develop correctly. When born without it, the TM forms to be more rigid so that it cannot carry sound waves properly, decreasing hearing sensitivity significantly. Yang et al. (2025) sought to study the direct effects of the gene by genetically mutating several groups of mice. They studied four groups: mice born without any hepsin, mice born with a version of hepsin that did not have splicing abilities, mice born with hepsin that was barely expressed (undetectable), and mice born with hepsin that was moderately expressed. The amount of expression of hepsin was largely beyond the control of the researchers, as modern science has not yet allowed for this level of precision in genomics. The results suggested that the absence of Hepsin, specifically its ability to perform splicing activity in the formation of the TM, is directly responsible for some instances of genetic hearing loss in mice and humans. Both the mice without any hepsin and with non-splicing hepsin recorded complete functional hearing loss, while the mice with moderately and low expressed Hepsin recorded respective levels of hearing. 

Melinda Moyer goes further to discuss a symptom of hearing damage or dysfunction, tinnitus. Tinnitus is characterized as the perception of ringing without any external stimuli. It can be caused by environmental factors such as excessive volume, bone damage, or tumors or could be a result of genetic factors as well. When certain genes or the absence of them lead to structural abnormalities in the ear, sound waves do not travel the way they are supposed to. When tinnitus is constant, it can cause significant disruption to ways of life, such as hindering sleep or focus. Because it has so many possible causes, figuring out the root cause is important to find the proper treatment for it. Some people remedy the ringing simply by playing white noise, which can be enough interference for the perception to disappear. Other treatments include cognitive behavioral therapy to help with any distress that comes with it and tinnitus retraining therapy to help with adjustment.

These two articles complement each other to give an encompassing analysis of the levels of hearing dysfunction. The Hepsin paper guides readers through how genetic alterations can affect hearing throughout all stages of life, while the tinnitus article gives us insight into one way hearing dysfunction is experienced, as well as some of the less-invasive treatments for it. However, these pieces both point to the underlying question of: Can we treat the root of the problem rather than the symptoms? While the causes of tinnitus vary, most vulnerabilities contain genetic components. Treating the root problem can look a lot of different ways. Genetically mutating embryos that do not have Hepsin would raise ethical concerns, even if modern science evolves to that point.  However, it would be valuable knowledge for families that might be expecting a child with hearing loss, or for the medical field to start thinking about interventions that might mitigate the predisposition to hearing loss without genetic manipulation. Are there medical interventions or lifestyle changes that could be made to augment the structure of the ear to be more resilient? Or rather, could we use an intervention like viral vectors to produce similar effects as necessary genes? What further insights can we take from the experiences of those with hearing loss?

H., Hsu, Y.-C., Yeh, P., Hung, C.-J., Tsai, Y.-F., Fang, M.-C., ... & Lin, S.-W. (2025). Critical role of hepsin/TMPRSS1 in hearing and tectorial membrane morphogenesis: insights from transgenic mouse models. Hearing Research. https://doi.org/10.1016/j.heares.2025.108956
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Moyer, M. W. (2025, April 29). What Can You Do About Tinnitus? The New York Times. https://www.nytimes.com/2025/04/29/well/live/tinnitus-treatment-options.html?searchResultPosition=1