PIETROPAOLO Susanna

Project status: active

At the Aquitaine Institute of Cognitive and Integrative Neuroscience in Pessac (France), Susanna Pietropaolo is attempting to find a treatment for hyperacusis and tinnitus, abnormalities of auditory perception that can be severely debilitating in everyday life.

Hyperacusis – the heightened perception of sounds – and tinnitus – the perception of “phantom” sounds causing permanent background noise – are acoustic dysfunctions that can have a major impact on patients’ quality of life. Currently, no effective treatment is available. However, these are real public health concerns: 20 million French people suffer from tinnitus, according to a JNA-IFOP survey in 2020. With her team, Susanna Pietropaolo, a CNRS neuroscientist, is trying to find a pharmacological solution to these pathologies.

In patients with rare genetic diseases and hyperacusis or tinnitus, a malfunction has been observed in certain structures required for communication between nerve cells. These are the ion channels, so called because they allow the entry and exit of ions in the cell. It is these ion flows that initiate nerve signal propagation.a“Fondation Pour l’Audition’s support is critical to this very complex project. We’ve been able to recruit a PhD student and a technician to work 100% on the project for three years. We’ve also been able to purchase the equipment required for the behavioral tests on our models.”Repositioning drugs

Based on these findings, Susanna Pietropaolo summarizes her approach: “To verify that ion channel dysfunction is involved in these patients’ hearing anomalies, we want to test the effect of molecules targeting these channels to counterbalance their deficiency.” At this stage, the team will work in mouse models that mimic these rare genetic diseases, Fragile X syndrome* and Williams-Beuren syndrome**. “We’ve started to develop a murine model of tinnitus,” she says. “We also need to develop a protocol for assessing auditory symptoms in our animals. This will include behavioral tests.” The team will inject molecules already used in certain diseases. In the event of a positive result, the use of immediately available drugs will speed up the development of treatment. “In addition, our studies should shed light on the mechanisms involved in hyperacusis and tinnitus,” concludes the researcher.

Doctor Susanna Pietropaolo
Investigator
Aquitaine Institute for Cognitive and Integrative Neuroscience, University of Bordeaux, France

*Fragile X syndrome: a rare genetic disease associated with hyperacusis and characterized by delayed developmental impairment, mental impairment of varying severity and behavioral disorders often related to autism spectrum disorders.

**Williams-Beuren syndrome: a rare genetic disease characterized by a set of abnormalities such as cardiac malformations, cognitive impairment and hyperacusis.