FDA Expediting Review of Regeneron Drug to Treat Genetic Hearing Loss
Regeneron’s DB-OTO gene therapy for genetic hearing loss is one of nine drugs to receive expedited review under FDA's new CNPV program.
Category: Sensorineural
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Sensorineural
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Sensorion Announces Positive Data from Audiogene SENS-501 Clinical Trial
Sensorion’s Phase 1/2 Audiogene trial of the gene therapy SENS-501 for infants and toddlers with OTOF-related congenital deafness shows promising early safety and hearing improvement data, particularly in one patient, with a higher dose cohort now underway.
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Akouos’ AK-OTOF Receives Orphan Drug Designation
Akouos, Inc announced that the European Medicines Agency (EMA) Committee for Orphan Medicinal Products (COMP) issued a positive opinion on the company’s application for orphan drug designation for AK-OTOF, a gene therapy intended for the treatment of otoferlin gene-mediated hearing loss. The positive opinion was subsequently adopted by the European Commission.
Researchers Explain Link Between Hearing Loss, Dementia
Scientists consider explanations for how changes in brain activity due to hearing loss might directly promote the presence of abnormal proteins that cause Alzheimer’s disease, therefore triggering the disease.
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Plural Publishing Releases 2nd Edition of ‘Disorders of the Auditory System’
The book by Frank Musiek, Jennifer Shinn, Jane Baran, and Raleigh Jones provides the reader with both the audiologic and medical aspects of auditory dysfunction associated with disorders of the peripheral and central auditory system.
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Research Shows Hair Cell Damage May Be Main Cause of Age-related Hearing Loss
Researchers examined 120 inner ears collected at autopsy. They used multivariable statistical regression to compare data on the survival of hair cells, nerve fibers, and the stria vascularis with the patients’ audiograms to uncover the main predictor of the hearing loss in this aging population. They found that the degree and location of hair cell death predicted the severity and pattern of the hearing loss, while stria vascularis damage did not.
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What Is “Normal Hearing” for Older Adults and Can “Normal-hearing Older Adults” Benefit from Hearing Care Intervention?
Researcher and author Larry Humes, PhD, points out that large-scale studies have identified self-reported hearing difficulties as one of the strongest predictors of hearing aid uptake and use. He says this further reinforces the need for the older consumer and the hearing care professional to quantify the severity of hearing difficulties above and beyond those captured by the pure-tone audiogram.
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BBC Looks at How Loud Music Can Lead to Early Signs of Hearing Damage
The article examined a study from researchers at the University of Manchester, which suggests that although the damage observed is not enough to be diagnosed as a full-blown hearing loss, it could potentially have a cumulative effect on hearing later in life.
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Researchers Look at Potential Link between Coronavirus and Hearing Loss
The authors point out that coronaviruses, in some cases, can cause peripheral neuropathy, and, ”in theory..COVID-19 could cause auditory neuropathy, a hearing disorder where the cochlea is functioning but transmission along the auditory nerve to the brain is impaired.”
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The Musicians’ Clinics of Canada Launches New Website
MusiciansClinics.com is a resource for those seeking information on a variety of topics related to hearing and hearing loss in musicians. Developed by audiologist-musician Marshall Chasin, AuD, the website offers a large range of resources for guitarists, bass players, violinists, woodwind players, school band teachers, and more.
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USC Stem Cell Study Traces Evolution of Organ of Corti
By the time mice are born, the progenitor cells in the Organ of Corti have finished both proliferation and differentiation, and cannot regenerate if they are damaged—causing permanent hearing loss.
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Age-related Hearing Loss Genes Discovered in Fruit Flies
“Our twin discoveries that fruit flies experience age-related hearing loss and that their prior auditory health is controlled by a particular set of genes, is a significant breakthrough. The fact that these genes are conserved in humans will also help to focus future clinical research in humans and thereby accelerate the discovery of novel pharmacological or gene-therapeutic strategies,” says lead-author Joerg Albert.
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Age-related Hearing Loss May Reorganize Brain’s Sensory Areas
Researchers looked at mice engineered to have progressive hearing loss, and found that their neurotransmitter receptors—responsible for communication between brain cells—exhibited changes in sensory processing regions related to memory.
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Researchers Chart Development of Cells from Mouse Cochlea
In the future, scientists may be able to use the data to steer stem cells toward the hair cell lineage, helping to produce the specialized cells they need to test cell replacement approaches for reversing some forms of hearing loss.
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Sonova Announces New Brand Ambassador, Volleyball Player Natália Martins
The 35-year-old has had profound hearing loss since childhood and wears hearing aids from Sonova brand, Phonak. In Brazil, she is the “first volleyball player with hearing loss to play at professional level and to make it into her country’s national team.”
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Frequency Therapeutics Announces Clinical Data from Auditory Drug
FX-322 is Frequency’s lead product candidate, designed to regenerate auditory sensory hair cells in the cochlea and improve hearing in patients with sensorineural hearing loss (SNHL).
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Researchers Examine Link Between Cognitive Decline, Hearing Loss
In the hippocampus, synaptic plasticity was chronically impaired by progressive hearing loss. The distribution and density of neurotransmitter receptors in sensory and memory regions of the brain also changed constantly. The stronger the hearing impairment, the poorer were both synaptic plasticity and memory ability.
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How Might the Brain Change When We Reintroduce Sound? Interview with Anu Sharma, PhD
New research shows that after wearing professionally fit quality hearing aids, a patient’s brain may “re-organize” its auditory processing centers back towards its original state prior to the hearing loss—with corresponding gains in auditory speech perception abilities and improvements in global cognitive function, executive function, processing speed, and visual working memory performance. Anu Sharma discusses the research findings with Douglas Beck.
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