Startup Aims to Develop the First Gene Therapy for Hearing Loss

A Boston biotech startup is attempting to develop the first gene therapy treatment for hearing loss. If successful, it promises to offer new hope to the estimated 300,000 Americans who suffer from gene-based hearing loss—a number that includes more than 4,000 newborns.

The timing may not be ideal, but biotech companies have been largely immune from the effects of the COVID-19 pandemic and ensuing recession. Developing new drugs takes years, so these companies are better able to weather storms. And the efforts of biotechnology companies that are focusing on tools to prevent and treat potentially fatal health threats has resulted in soaring stock values for many of these companies, even in the face of the current crisis.

Akouos, the Boston startup, was founded in 2016. Its goal is to develop the first gene therapy to treat hearing loss and is targeting a specific form of deafness that results from mutations in a single gene. They are experimenting with adeno-associated viruses, using them as vectors to deliver DNA that encodes a functioning gene in certain target cells.

Around 7,000 people are afflicted with this type of genetic hearing loss. Adeno-associated viruses are considered viable candidates because they don’t usually cause disease and can be customized to treat a variety of genetic conditions. The company has partnered with two influential organizations: Massachusetts Eye and Ear and Lonza, a Swiss manufacturing company that holds contracts with various pharmaceutical makers.

Manny Simons, Akouos’ co-founder and chief executive, holds a degree in neuroscience from Harvard College, a Ph.D. in biomedical engineering from the Massachusetts Institute of Technology, and an MBA from Harvard Business School. His desire to help those with hearing loss stems from his passion for playing the piano.

“It’s easy to take hearing for granted,” said in a Boston Globe interview. “Maybe because music has been important to me, it’s something that I take a little less for granted.”[1]

[1] Saltzman, J. (2020, June 22). Akouos, a startup developing a gene therapy for hearing loss, raises IPO value to $125 million - The Boston Globe. Retrieved June 30, 2020, from https://www.bostonglobe.com/2020/06/22/business/ipo-upped-akouos-boston-startup-working-gene-therapy-hearing-loss/

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The Future of Hearing Devices: Deep Neural Networks & Artificial Intelligence

February 14, 2025

Hearing technology has come a long way over the years; however, one aspect of hearing loss that many hearing aids have difficulty addressing is trouble understanding speech in noise. Fortunately, some of today’s state-of-the-art devices can solve this problem utilizing deep neural networks (DNN) and artificial intelligence (AI). Sensorineural hearing loss causes difficulty understanding speech in background noise. This type of hearing loss is caused by damage to the hair cells of the inner ear that convert soundwaves into electrical energy, which are then interpreted by the brain. This causes the quality and quantity of the signal sent from the ears to the brain to diminish over time, resulting in the brain expending more energy and effort to make sense of it. DNNs may help improve the transmission of the neural code, making hearing easier on the brain. [1] It’s important to know what DNNs are to understand how they can help. DNNs are a subset of AI. They rapidly analyze large data sets so that they can teach hearing aids and other technological devices how to respond to data like a human would, all while verifying accuracy and making corrections. DNNs are beneficial in hearing technology because they can help with speech enhancement. DNNs can be applied as “time-frequency masks,” meaning select sounds—like speech—are allowed while other sounds are suppressed. DNNs can also identify and separate multiple voices, selecting a primary voice to emphasize and secondary voices to suppress. This feature can help hearing device wearers in a variety of settings. For example, at a restaurant or party, the hearing device can emphasize a conversation partner, allow other nearby voices at a reduced volume and suppress background noises such as dishes clinking. Schedule an appointment to talk to Center for Hearing about our vast state-of-the-art hearing solutions today— (239) 434-0086. [1] Published by Douglas L. Beck. (2021, January 15). Deep neural networks in hearing devices. Retrieved April 2, 2021 from https://www.hearingreview.com/hearing-products/hearing-aids/deep-neural-networks

Untreated Hearing Loss May Lead to Dementia

February 14, 2025

A recent study by the U.S. National Institute on Aging showed a link between hearing loss and dementia. It found that patients older than 60 had a 35% higher risk of developing dementia if they suffered from hearing loss. The study, conducted over a four-year period, monitored over 600 patients for signs of dementia. Data showed those with moderate to severe hearing loss developed degenerative cognitive disorders at a higher rate than others, concluding that for every additional loss of 10 decibels of hearing capacity, a patient’s risk for Alzheimer’s increased 20%. While several theories for this correlation exist, more research is needed to find a definitive association. “Hearing loss might result from damage to nerve cells,” Dr. Richard B. Lipton says. “That means damage to the hearing organ and the hair cells that pick up the pattern of vibration the sound produces in the ear. And if there’s damage to the neurons that mediate hearing, that may be a kind of marker for similar damage to nerve cells involved in memory and higher cognition.” Lipton also believes the social isolation that often accompanies hearing loss may lead to less cognitive engagement, crucial in preventing dementia. This in turn may increase the risk for Alzheimer’s. The bottom line is, dementia and Alzheimer’s have less to do with chronological age than previously believed. Researchers are focused instead on biological age and the overall health and lifestyle of those patients who exhibit early symptoms of cognitive decline.