Vestibulocochlear Nerve Diseases

Researchers have uncovered that tinnitus, an incurable persistent ringing or other sounds in the ears, might result from underlying auditory nerve damage that can't be detected on conventional hearing tests. The work builds upon previous research into cochlear synaptopathy or 'hidden hearing loss,' a difficulty hearing in noisy environments despite showing normal results on hearing tests. The researchers hope these new findings into the mechanisms underlying tinnitus could lead to treatment options.  A new study from Mass Eye and Ear investigators shows that individuals who report tinnitus, which present as a ringing in the ears in more than one out of ten adults worldwide, are experiencing auditory nerve loss that is not picked up by conventional hearing tests. This work is part of a P50 grant awarded by the National Institutes of Health (NIH) to Mass Eye and Ear researchers within the Eaton-Peabody Laboratories (EPL) for their work on cochlear synaptopathy, which is commonly referred to as "hidden hearing loss." The results from this study provide a better understanding on the origins of tinnitus and are published November 30th in Scientific Reports. "Beyond the nuisance of having persistent ringing or other sounds in the ears, tinnitus symptoms are debilitating in many patients, causing sleep deprivation, social isolation, anxiety and depression, adversely affecting work performance, and reducing significantly their quality of life," said senior author Stéphane F. Maison, PhD, CCC-A, a principal investigator at Mass Eye and Ear, a member of Mass General Brigham, and clinical director of the Mass Eye and Ear Tinnitus Clinic. "We won't be able to cure tinnitus until we fully understand the mechanisms underlying its genesis. This work is a first step toward our ultimate goal of silencing tinnitus." Many individuals with hearing loss report a buzzing, humming, ringing or even roaring sound in their ears. It's been a longstanding idea that these symptoms, known as tinnitus, arise as a result of a maladaptive plasticity of the brain. In other words, the brain tries to compensate for the loss of hearing by increasing its activity, resulting in the perception of a phantom sound, tinnitus. Until recently though, this idea was disputed as some tinnitus sufferers have normal hearing tests. However, the discovery of cochlear synaptopathy back in 2009 by Mass Eye and Ear investigators brought back to life this hypothesis as it was evidenced that patients with a normal hearing test can have a significant loss to the auditory nerve. In view of this paradigm shift in the way researchers and clinicians think about hearing loss, Maison and his team sought to determine if such hidden damage could be associated with the tinnitus symptoms experienced by a cohort of normal hearing participants. By measuring the response of their auditory nerve and brainstem, the researchers found that chronic tinnitus was not only associated with a loss of auditory nerve but that participants showed hyperactivity in the brainstem. "Our work reconciles the idea that tinnitus may be triggered by a loss of auditory nerve, including in people with normal hearing," said Maison. In terms of future directions, the investigators aim to capitalize on recent work geared toward the regeneration of auditory nerve via the use of drugs called neurotrophins. "The idea that, one day, researchers might be able to bring back the missing sound to the brain and, perhaps, reduce its hyperactivity in conjunction with retraining, definitely brings the hope of a cure closer to reality," Maison added. Disclosures: The authors declare no competing interests. Funding: This work was supported by a grant from the NIDCD (P50 DC015857) and the Lauer Tinnitus Research Center at the Mass Eye and Ear.

This is the longest Niagen NR clinical trial to date spanning two years and showcases that long-term Niagen NR is safe and well tolerated LOS ANGELES--(BUSINESS WIRE)-- ChromaDex, Inc. (NASDAQ:CDXC), one of the global authorities on Nicotinamide Adenine Dinucleotide (NAD+) and healthy aging research, shares results from a phase II clinical study published in the peer-reviewed journal, Movement Disorders, by a team of researchers led by Dr. Hilde Nilsen, University of Oslo, Arkershus University Hospital, Norway. The clinical trial was part of the ChromaDex External Research Program (CERP™), which donated ChromaDex’s patented nicotinamide riboside (NR) ingredient, Niagen®, one of the most efficient and superior NAD+ precursors available, for the advancement of this research. The promising results demonstrate that long-term supplementation with Niagen NR effectively increased whole blood NAD+ levels up to fourfold, improved coordination, and enhanced eye movement while maintaining biomarkers of stable liver and kidney function in Ataxia Telangiectasia (AT) patients. AT is a rare, inherited neurodegenerative disorder characterized by premature aging, cerebellar degeneration, immunodeficiency, and cancer predisposition. This press release features multimedia. View the full release here: Newly published phase II clinical study demonstrates that supplementation with Niagen, patented nicotinamide riboside (NR), elevates NAD+ up to fourfold, improving motor coordination and eye movement in Ataxia Telangiectasia (AT) patients (Graphic: Business Wire) Building on a growing body of research, this is the second published study (Veenhuis et al. in 2021) analyzing the therapeutic effects of Niagen NR supplementation in AT patients and marks a milestone as it is the longest Niagen NR clinical trial to date, spanning two years, with results showcasing that long-term supplementation is safe and well tolerated. "NR supplementation has shown promising potential in supporting brain health and cognition,” said Dr. Andrew Shao the Vice President of Scientific & Regulatory Affairs at ChromaDex. “Independent research through CERP suggests that NR may play a role in maintaining neuronal function and promoting cognitive well-being by supporting NAD+ levels in brain cells." Individuals diagnosed with AT frequently experience profound challenges in motor function, characterized by neurodegenerative conditions like cerebellar ataxia, resulting in impaired movement and coordination and they may exhibit oculomotor apraxia, difficulties controlling eye movements. These factors often result in a loss of independent walking ability, typically necessitating the use of a wheelchair by the age of ten. Dr. Nilsen remarks, “While the exact mechanisms underlying cerebellar degeneration and neurodegeneration in AT are not fully understood, research indicates inability to repair and respond appropriately to DNA damage. AT research has shown that DNA damage depletes NAD+, the crucial cellular coenzyme that plays an essential role in mitochondrial function, DNA repair and cellular energy production. This growing body of research is promising as it demonstrates that NR supplementation may be a potential therapeutic strategy for AT patients.” About the study This two year phase II clinical study was a single-arm open-label intervention trial investigating the safety and efficacy of long-term NR supplementation in 10 patients with AT (6 were female, 4 were male, of which 7 were children, 3 were adults), between the ages of 3 to 27 years. The patients were given oral NR as an intervention starting with an initial dose of 150 mg/day, gradually increasing to 300 mg/day, and reaching a final dose of 500 mg/day. Study highlights NR supplementation was well tolerated, with no serious adverse events. NR supplementation increased whole blood NAD+ levels up to fourfold. Serum levels of AFP, serum alpha-fetoprotein and Nfl, neurofilament light chain, biomarkers of AT progression, remained stable for most participants over two years of NR supplementation, suggesting that NR may slow or delay progression of AT. Long-term NR supplementation reduced ataxia symptoms and improved motor coordination, eye movements, and overall neurological functioning: Positive correlations were observed between NR supplementation and improved AT Neurological Examination Toolkit (NEST) total score, eye movements, SARA coordination scores, ICARS coordination scores, and oculomotor scores. Biomarkers related to liver and kidney function did not show clinically significant changes during the two-year treatment period, indicating the NR supplementation was safe. Long-term blood glucose regulation, measured as HbA1c level, remained stable in all participants. NR supplementation did not cause changes in hematological or immunological parameters. Relevance Reaching a milestone in NR clinical research as the longest published clinical study to date, results of this study demonstrate that NR effectively increased whole blood NAD+ levels up to fourfold, improved coordination, and enhanced eye movement in both adults and children with AT, while maintaining biomarkers of stable liver and kidney function. The outcomes of this study strongly support the inclusion of NR supplementation as a standard clinical approach for AT, however more research needs to be conducted to confirm the impact of NR on motor skills such as standing and walking. For additional information on the science supporting Niagen® visit . About ChromaDex: ChromaDex Corp. is a global bioscience company dedicated to healthy aging. The ChromaDex team, which includes world-renowned scientists, is pioneering research on nicotinamide adenine dinucleotide (NAD+), levels of which decline with age. ChromaDex is the innovator behind NAD+ precursor nicotinamide riboside (NR), commercialized as the flagship ingredient Niagen®. Nicotinamide riboside and other NAD+ precursors are protected by ChromaDex’s patent portfolio. ChromaDex maintains a website at to which ChromaDex regularly posts copies of its press releases as well as additional and financial information about the Company. Forward-Looking Statements: This release contains forward-looking statements within the meaning of Section 27A of the Securities Act of 1933, as amended, and Section 21E of the Securities and Exchange Act of 1934, as amended, including statements related to whether the results demonstrate that long-term supplementation with Niagen NR effectively increased whole blood NAD+ levels up to fourfold, improved coordination, and enhanced eye movement while maintaining biomarkers of stable liver and kidney function in Ataxia Telangiectasia (AT) patients, whether the results showcase that long-term supplementation is safe and well tolerated, whether NR supplementation has shown promising potential in supporting brain health and cognition, and whether this growing body of research is promising or if it demonstrates that NR supplementation may be a potential therapeutic strategy for AT patients. Statements that are not a description of historical facts constitute forward-looking statements and may often, but not always, be identified by the use of such words as "expects," "anticipates," "intends," "estimates," "plans," "potential," "possible," "probable," "believes," "seeks," "may," "will," "should," "could" or the negative of such terms or other similar expressions. Risks that contribute to the uncertain nature of these forward-looking statements include the impact of the COVID-19 pandemic on our business and the global economy; our history of operating losses and need to obtain additional financing; the growth and profitability of our product sales; our ability to maintain sales, marketing and distribution capabilities; changing consumer perceptions of our products; our reliance on a single or limited number of third-party suppliers; and the risks and uncertainties associated with our business and financial condition. More detailed information about ChromaDex and the risk factors that may affect the realization of forward-looking statements is set forth in ChromaDex's Annual Report on Form 10-K for the fiscal year ended December 31, 2021, ChromaDex's Quarterly Reports on Form 10-Q and other filings submitted by ChromaDex to the SEC, copies of which may be obtained from the SEC's website at . Readers are cautioned not to place undue reliance on these forward-looking statements, which speak only as of the date hereof, and actual results may differ materially from those suggested by these forward-looking statements. All forward-looking statements are qualified in their entirety by this cautionary statement and ChromaDex undertakes no obligation to revise or update this release to reflect events or circumstances after the date hereof.

IRVINE, Calif.--(BUSINESS WIRE)-- Viseon Inc. announces the company’s first use of the MaxView® 4K Advanced Digital Visualization (4K-ADV™) system during a neurosurgical tumor resection procedure. The procedure was performed by neurosurgeon Dr. Farrokh Farrokhi, Virginia Mason Medical Center, Seattle, Washington, who specializes in deep brain stimulation, skull base surgery, brain tumors and minimally invasive spine surgery. Dr. Farrokhi commented: “As a regular user of the Viseon HD technology for a variety of minimally invasive spine surgery procedures for more than a year, transition to neurosurgical use will be seamless, especially with the superior image quality and depth perception now enabled by the new 4K-ADV MaxView System. Admittedly, I chose a high hurdle for this first 4K-ADV case in a Vestibular Schwannoma tumor resection. This technology use should be routine for Glioma or Metastatic tumor procedures and I will be using it for these procedures in the future.” Pete Davis, Viseon President & COO, added, “We are honored to have Dr. Farrokhi usher Viseon into the next chapter of our journey, expanding our addressable market from Minimally Invasive Spine Surgery into Cranial Neurosurgery. His feedback from the first case was very helpful as we plan to expand our sales emphasis in 2024. The MaxView 4K-ADV System continues to expand the innovative enabling nature of the Viseon digital technology, disrupting legacy standards of large, expensive capital equipment. We plan to continue to innovate, participate and collaborate in the future of digital health.” About Viseon: Viseon Inc. is privately owned with a founding goal to advance open and minimally invasive surgery with innovative technology that enhances intraoperative visualization with state of the art, single use 4K imaging technology. The Company has remained focused on no capital equipment, no OR footprint, no equipment redundancy, no sterile tray processing, and ease of use with broad procedural diversity, enabling use in-hospital and in the ASC setting. By disrupting antiquated modalities for visualization, the Company is enabling the digital future, accelerating adoption of advanced surgical techniques, improving the surgeon experience, patient outcomes and safety, and introducing economic benefits to hospitals and surgery centers. View source version on businesswire.com: Contacts Viseon, Inc. Customer Service customerservice@viseon-inc.com Source: Viseon Inc. View this news release online at: