Tapping Into the Immune System to Diagnose and Treat Hearing Loss

Woman cupping her ear, clearly not hearing, in front of a blue background

The Department of Otolaryngology-Head & Neck Surgery at the University of Pittsburgh has started an immunological hearing division.

“There are a lot of conditions in hearing that we don’t have a reason for,” said Dr. Peter Santa Maria, MD, PhD, Chief, Division of Otology/Neurotology and Co-Leader of this new division. “We call them idiopathic.”

For example, there are conditions like idiopathic sudden sensorineural hearing loss, Meniere’s disease, and fluctuating hearing loss. A lot of different ear conditions do not really have any good treatments. A group seem to be steroid responsive, but none of the mechanisms are known. “Steroids themselves are very dirty drugs with a lot of horrible side effects that prevent people from taking them long term,” Dr. Santa Maria added.

So, the idea is to have an “immune mediated inner ear center that is a world leader where we can start to unlock some of the mechanisms that cause these things,” Dr. Santa Maria explained. “We can start to understand and diagnose patients correctly according to immune system issues and potentially offer new immune treatments that might be better than steroids for helping patients with hearing loss.”

This division will house discovery, translational, and clinical research. Animal models will be used to study the mechanisms and how the immune system in the ear works, so potential therapies can be better identified. A couple of different targets that act on macrophages or the innate immune system are already being worked on. The innate immune system seems to underlie a lot of why hearing loss occurs, such as noise-induced hearing loss. Some people get a large degree of hearing loss when they are exposed to a little noise, yet others don’t end up with much hearing loss despite a lot of noise exposure. One of the things underlying that is the immune response to that noise and the protective mechanisms that do or do not kick in that can prevent that. There are also potential treatments for hearing loss associated with vestibular schwannoma.

A goal is to go beyond what people traditionally think as immune conditions and look into associated things where the immune system plays a role. This includes hearing loss associated with aging, noise, and tumors.

On the translational side, the division is also looking at potentially doing some drug discovery and drug development for these targets. On the clinical side, “we want to make sure that we’re not only delivering the best level of care to all these patients with these conditions, but also working them up, seeing them appropriately, and giving them the best available therapies,” Dr. Santa Maria said. In addition, they are looking to test their samples to look for immune mediated markers that can drive future research on the translational side.

A large population of people with Meniere’s disease lives in the area and is underserved. There are a lot of misdiagnoses, and a huge number of sudden sensorineural hearing loss patients that can potentially be helped.

A research assistant professor with a strong background in immunology, particularly in the innate immune system, was just hired to co-lead the division. The division has just identified and is hoping to recruit a clinical trials coordinator.

The team has already started looking at immune mechanisms of hearing loss in chronic ear infections and has an expedited pathway for patients with sudden sensorineural hearing loss. They are beginning to investigate their immune markers within the boundaries of insurance. Things like genetic testing, immune media panel screens, and biomarker investigation are typically not covered by insurance for the average person but can be achieved through this division.

“We’re aiming to be the first division that is complete from discovery research through translation into clinical research,” Dr. Santa Maria said.

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