Potential Treatment to Safeguard Inner Ear Cells

André Hoelz (Photo – caltech.edu)

In 2015, Andre Hoelz, a chemistry and biochemistry professor at UC San Diego, was diagnosed with a rare brain tumor pressing on his acoustic nerve. As a scientist who normally studies protein structures, Hoelz delved into understanding the auditory system. He soon partnered with his surgeon, Rick Friedman, an expert in ear disorders, to research methods for protecting the inner ear from hearing loss. With a new grant, they’re expanding their efforts.

By Cynthia Eller, Caltech

The inner ear, which converts sound into signals for the brain, relies on delicate hair-like cells. Unfortunately, these cells can’t regenerate once damaged, leading to permanent hearing loss. Hoelz explains that only 16,000 of the trillions of cells in the human body are involved in hearing. Once they die, hearing is gone, often due to causes like aging, infections, or noise exposure. A particularly harmful culprit is cisplatin chemotherapy, which, though life-saving, can cause severe hearing loss in up to 70% of patients, especially children.

Currently, the only FDA-approved treatment to prevent hearing loss from cisplatin works by inactivating the chemotherapy, weakening its cancer-fighting effects. As Hoelz himself experienced hearing loss in 2015 due to his tumor, he began collaborating with Friedman on potential solutions. They identified a gene called Prkag2, which regulates a key enzyme, AMPK, involved in hearing. By activating AMPK, they can protect the hair cells and preserve hearing, especially during cisplatin treatment.

With support from Curebound, Hoelz and Friedman are testing this approach in animal models and plan to move to human trials. Their long-term goal is to develop a treatment that could act like a “vitamin” to protect ear cells from damage throughout life, potentially preventing hearing loss from various causes.

While some animals can regenerate hearing cells, humans cannot. However, this ongoing research could open the door to treatments that protect hearing, possibly for Hoelz and others like him.

This article has been edited for brevity. Read the full article at caltech.edu.

(Photo – André Hoelz)