Source:Hearing Research
Author(s): David H. Jung, Steven D. Rauch
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Παρασκευή 23 Μαρτίου 2018
Clinical trials for inner ear drugs: Design and execution challenges
Source:Hearing Research
Author(s): David H. Jung, Steven D. Rauch
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Clinical trials for inner ear drugs: Design and execution challenges
Source:Hearing Research
Author(s): David H. Jung, Steven D. Rauch
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Clinical trials for inner ear drugs: Design and execution challenges
Source:Hearing Research
Author(s): David H. Jung, Steven D. Rauch
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Clinical trials for inner ear drugs: Design and execution challenges
Publication date: Available online 22 March 2018
Source:Hearing Research
Author(s): David H. Jung, Steven D. Rauch
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Source:Hearing Research
Author(s): David H. Jung, Steven D. Rauch
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Clinical trials for inner ear drugs: Design and execution challenges
Publication date: Available online 22 March 2018
Source:Hearing Research
Author(s): David H. Jung, Steven D. Rauch
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Source:Hearing Research
Author(s): David H. Jung, Steven D. Rauch
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Cochlear Gifts $10 Million to Build Academic Center on Hearing Loss
Cochlear (http://ift.tt/2oVevGx) has pledged a gift of $10 million to the Johns Hopkins Bloomberg School of Public Health to establish the Cochlear Center for Hearing and Public Health in honor of World Hearing Day this year. The Cochlear Center will focus on addressing the global impact of hearing loss by conducting research to determine the gravity of hearing loss as a public health issue, developing and testing interventions to mitigate the effects of hearing loss, and helping craft policies and strategies to ensure successful implementation of those interventions at the local, national, and global levels. The Center will also recruit and train researchers and public health experts to advance these goals.
The gift of $10 million will be made over a period of 10 years. Cochlear will collaborate with the Center and have representation on the Center’s advisory board to provide feedback and help identify opportunities for continued industry-academic collaborations. Since Cochlear’s headquarters are based on the campus of Macquarie University in Sydney and is next to the Australian Hearing Hub, the company is hoping that this gift will facilitate closer industry, academic, and clinical engagement to spur future training and research partnerships, including shared postdoctoral fellowships, a shared masters of public health program, shared PhD programs, and faculty exchanges. Cochlear and Johns Hopkins Bloomberg School of Public Health celebrated the official opening of the Cochlear Center for Hearing and Public Health on World Hearing Day, March 3.
The gift of $10 million will be made over a period of 10 years. Cochlear will collaborate with the Center and have representation on the Center’s advisory board to provide feedback and help identify opportunities for continued industry-academic collaborations. Since Cochlear’s headquarters are based on the campus of Macquarie University in Sydney and is next to the Australian Hearing Hub, the company is hoping that this gift will facilitate closer industry, academic, and clinical engagement to spur future training and research partnerships, including shared postdoctoral fellowships, a shared masters of public health program, shared PhD programs, and faculty exchanges. Cochlear and Johns Hopkins Bloomberg School of Public Health celebrated the official opening of the Cochlear Center for Hearing and Public Health on World Hearing Day, March 3.
Published: 3/23/2018 9:36:00 AM
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iHear and Atlazo to Develop Audio-Centric Digital Platform for Hearing Devices
iHear Medical (http://ift.tt/1pm5Mpw) is partnering with Atlazo to develop an advanced digital hardware platform, System on Chip (SoC), which will power a new operating system designed to shift devices from relying on visual displays to voice commands and the auditory domain for hearing aids and hearables. With the Hearing of Things (HoT) operating system, users can selectively listen to and send communications to their social media accounts and text messages with hearing devices instead of their cell phone or computer, eliminating the need to be tethered to traditional computing devices for sending and receiving information. The HoT standard will offer bi-directional wireless connectivity to everyday devices, from mobile phones to home appliances. Hearing devices powered by the HoT operating system will streamline the user experience for mobile phones, including phone calling and control of home appliances with a voice user interface. These devices will also offer real-time health tracking and relay personal and health information into the ear privately and discreetly.
Published: 3/23/2018 9:18:00 AM
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Cochlear Gifts $10 Million to Build Academic Center on Hearing Loss
Cochlear (http://ift.tt/2oVevGx) has pledged a gift of $10 million to the Johns Hopkins Bloomberg School of Public Health to establish the Cochlear Center for Hearing and Public Health in honor of World Hearing Day this year. The Cochlear Center will focus on addressing the global impact of hearing loss by conducting research to determine the gravity of hearing loss as a public health issue, developing and testing interventions to mitigate the effects of hearing loss, and helping craft policies and strategies to ensure successful implementation of those interventions at the local, national, and global levels. The Center will also recruit and train researchers and public health experts to advance these goals.
The gift of $10 million will be made over a period of 10 years. Cochlear will collaborate with the Center and have representation on the Center’s advisory board to provide feedback and help identify opportunities for continued industry-academic collaborations. Since Cochlear’s headquarters are based on the campus of Macquarie University in Sydney and is next to the Australian Hearing Hub, the company is hoping that this gift will facilitate closer industry, academic, and clinical engagement to spur future training and research partnerships, including shared postdoctoral fellowships, a shared masters of public health program, shared PhD programs, and faculty exchanges. Cochlear and Johns Hopkins Bloomberg School of Public Health celebrated the official opening of the Cochlear Center for Hearing and Public Health on World Hearing Day, March 3.
The gift of $10 million will be made over a period of 10 years. Cochlear will collaborate with the Center and have representation on the Center’s advisory board to provide feedback and help identify opportunities for continued industry-academic collaborations. Since Cochlear’s headquarters are based on the campus of Macquarie University in Sydney and is next to the Australian Hearing Hub, the company is hoping that this gift will facilitate closer industry, academic, and clinical engagement to spur future training and research partnerships, including shared postdoctoral fellowships, a shared masters of public health program, shared PhD programs, and faculty exchanges. Cochlear and Johns Hopkins Bloomberg School of Public Health celebrated the official opening of the Cochlear Center for Hearing and Public Health on World Hearing Day, March 3.
Published: 3/23/2018 9:36:00 AM
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iHear and Atlazo to Develop Audio-Centric Digital Platform for Hearing Devices
iHear Medical (http://ift.tt/1pm5Mpw) is partnering with Atlazo to develop an advanced digital hardware platform, System on Chip (SoC), which will power a new operating system designed to shift devices from relying on visual displays to voice commands and the auditory domain for hearing aids and hearables. With the Hearing of Things (HoT) operating system, users can selectively listen to and send communications to their social media accounts and text messages with hearing devices instead of their cell phone or computer, eliminating the need to be tethered to traditional computing devices for sending and receiving information. The HoT standard will offer bi-directional wireless connectivity to everyday devices, from mobile phones to home appliances. Hearing devices powered by the HoT operating system will streamline the user experience for mobile phones, including phone calling and control of home appliances with a voice user interface. These devices will also offer real-time health tracking and relay personal and health information into the ear privately and discreetly.
Published: 3/23/2018 9:18:00 AM
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Cochlear Gifts $10 Million to Build Academic Center on Hearing Loss
Cochlear (http://ift.tt/2oVevGx) has pledged a gift of $10 million to the Johns Hopkins Bloomberg School of Public Health to establish the Cochlear Center for Hearing and Public Health in honor of World Hearing Day this year. The Cochlear Center will focus on addressing the global impact of hearing loss by conducting research to determine the gravity of hearing loss as a public health issue, developing and testing interventions to mitigate the effects of hearing loss, and helping craft policies and strategies to ensure successful implementation of those interventions at the local, national, and global levels. The Center will also recruit and train researchers and public health experts to advance these goals.
The gift of $10 million will be made over a period of 10 years. Cochlear will collaborate with the Center and have representation on the Center’s advisory board to provide feedback and help identify opportunities for continued industry-academic collaborations. Since Cochlear’s headquarters are based on the campus of Macquarie University in Sydney and is next to the Australian Hearing Hub, the company is hoping that this gift will facilitate closer industry, academic, and clinical engagement to spur future training and research partnerships, including shared postdoctoral fellowships, a shared masters of public health program, shared PhD programs, and faculty exchanges. Cochlear and Johns Hopkins Bloomberg School of Public Health celebrated the official opening of the Cochlear Center for Hearing and Public Health on World Hearing Day, March 3.
The gift of $10 million will be made over a period of 10 years. Cochlear will collaborate with the Center and have representation on the Center’s advisory board to provide feedback and help identify opportunities for continued industry-academic collaborations. Since Cochlear’s headquarters are based on the campus of Macquarie University in Sydney and is next to the Australian Hearing Hub, the company is hoping that this gift will facilitate closer industry, academic, and clinical engagement to spur future training and research partnerships, including shared postdoctoral fellowships, a shared masters of public health program, shared PhD programs, and faculty exchanges. Cochlear and Johns Hopkins Bloomberg School of Public Health celebrated the official opening of the Cochlear Center for Hearing and Public Health on World Hearing Day, March 3.
Published: 3/23/2018 9:36:00 AM
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iHear and Atlazo to Develop Audio-Centric Digital Platform for Hearing Devices
iHear Medical (http://ift.tt/1pm5Mpw) is partnering with Atlazo to develop an advanced digital hardware platform, System on Chip (SoC), which will power a new operating system designed to shift devices from relying on visual displays to voice commands and the auditory domain for hearing aids and hearables. With the Hearing of Things (HoT) operating system, users can selectively listen to and send communications to their social media accounts and text messages with hearing devices instead of their cell phone or computer, eliminating the need to be tethered to traditional computing devices for sending and receiving information. The HoT standard will offer bi-directional wireless connectivity to everyday devices, from mobile phones to home appliances. Hearing devices powered by the HoT operating system will streamline the user experience for mobile phones, including phone calling and control of home appliances with a voice user interface. These devices will also offer real-time health tracking and relay personal and health information into the ear privately and discreetly.
Published: 3/23/2018 9:18:00 AM
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GeneReviews®
GeneReviews®
Book. 1993
Authors: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A
Abstract
CLINICAL CHARACTERISTICS: KBG syndrome is typically characterized by macrodontia (especially of the upper central incisors), characteristic facial features (triangular face, brachycephaly, synophrys, widely spaced eyes, broad or bushy eyebrows, prominent ears, prominent nasal bridge, bulbous nose, anteverted nares, long philtrum, and thin vermilion of the upper lip), short stature, developmental delay / intellectual disability, and behavioral issues. Affected individuals may have feeding difficulties (particularly in infancy), skeletal anomalies (brachydactyly, large anterior fontanelle with delayed closure, scoliosis), hearing loss (conductive, mixed, and sensorineural), seizure disorder, and brain malformations. There is significant variability in the clinical findings, even between affected members of the same family.
DIAGNOSIS/TESTING: The diagnosis of KBG syndrome is confirmed in a proband by detection of either a heterozygous pathogenic variant in ANKRD11 or deletion of 16q24.3 that includes ANKRD11.
MANAGEMENT: Treatment of manifestations. Surgical corrections and/or speech therapy for palatal anomalies; nasogastric tube feeding in infants; pharmacologic treatment for gastroesophageal reflux disease; pressure-equalizing tubes and/or tonsillectomy/adenoidectomy for chronic otitis media; consideration of amplification for hearing loss; consideration of growth hormone therapy for short stature and medication to arrest puberty for premature pubertal development; standard treatment of seizure disorder, undescended testis in males, congenital heart defects, strabismus / refractive errors, and developmental disabilities. Surveillance. Routine monitoring of hearing, vision, growth, pubertal status (in prepubertal individuals), and cognitive development. Agents/circumstances to avoid. Ototoxic drugs should be avoided because of the risk for hearing loss. Pregnancy management. Pregnancy management should be tailored to the specific features in the affected woman. For example, involvement of a cardiologist and maternal fetal medicine physician for a pregnant woman with a history of a congenital heart defect; control of seizures during pregnancy for those with a seizure disorder.
GENETIC COUNSELING: Recurrence risk for sibs of a proband with KBG syndrome depends on the genetic alteration: Deletion of 16q24.3 (~75% of reported pathogenic variants are de novo and the remainder are inherited in an autosomal dominant manner.) ANKRD11 sequence variants (~66% of reported pathogenic variants are de novo and the remainder are inherited in an autosomal dominant manner.) Prenatal testing and preimplantation genetic diagnosis are possible if the causative genetic alteration has been identified in an affected family member.
PMID: 29565525
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Wolff-Parkinson-White syndrome and noncompaction in Leber's hereditary optic neuropathy due to the variant m.3460G>A.
Wolff-Parkinson-White syndrome and noncompaction in Leber's hereditary optic neuropathy due to the variant m.3460G>A.
J Int Med Res. 2018 Jan 01;:300060518765846
Authors: Finsterer J, Stollberger C, Gatterer E
Abstract
This report describes a 66-year-old Caucasian male who acutely developed severe, bilateral impairment of visual acuity at 24 years of age. Leber's hereditary optic neuropathy (LHON) was suspected but the diagnosis was not genetically confirmed until the age of 49 years when the primary LHON mutation m.3460G>A was detected. Since onset, visual acuity had slightly improved. The family history was positive for LHON (brother, two sisters of mother, female cousin) and genetically confirmed in his brother and one aunt. Since the age of 65 years, he had experienced recurrent vertigo. His cardiological history was positive for arterial hypertension, noncompaction, myocardial thickening, intermittent right bundle-branch-block (RBBB) and Wolff-Parkinson-White (WPW) syndrome. In addition to LHON, he presented with polyneuropathy, hyperCKaemia, carotid artery occlusion, and a history of stroke. Cardiological investigations at 66 years of age revealed mildly reduced systolic function, enlarged atria, and nonsustained ventricular tachycardias. He underwent an electrophysiological investigation, but radiofrequency ablation was ruled out due to a 'bizarre' cardiac conduction system. Instead, an implantable cardioverter defibrillator was proposed but refused by the patient. Since the vertigo did not resolve it was attributed to polyneuropathy. This case demonstrates that LHON may be associated with noncompaction, myocardial thickening, reduced systolic function, enlarged atria, RBBB, WPW syndrome and nonsustained ventricular tachycardias. WPW syndrome in LHON may require invasive antiarrhythmic treatment.
PMID: 29562793 [PubMed - as supplied by publisher]
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