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Background: -related schwannomatosis ( -SWN) is a debilitating condition that calls for robust treatment options. The defining feature of -SWN is the presence of bilateral vestibular schwannomas (VSs), which grow over time and can result in irreversible sensorineural hearing loss, significantly affecting the quality of life for those affected. At present, there are no FDA-approved medications specifically for treating VS or related hearing loss.
View Article and Find Full Text PDFEffect of cochlear implants on cognitive function in children and adolescents with hearing loss: a systematic review protocol.
JBI Evid Synth
January 2025
Office of Institutional Effectiveness, Touro University Nevada, Henderson, NV, USA.
Objective: The objective of this review is to evaluate the effect of cochlear implants on cognitive function in pediatric patients with hearing loss.
Introduction: Cognitive impairments have been reported in children with hearing loss. This is supported by the auditory scaffolding hypothesis, which describes sound as the basis for processing sequential information.
Phosphoribosyl pyrophosphate synthetase 1 () associated retinal degeneration: an international study.
Ophthalmic Genet
January 2025
Department of Ophthalmology, Casey Eye Institute, Oregon Health & Science University, Portland, Oregon, USA.
Introduction: Phosphoribosyl pyrophosphate synthetase 1 () is an X-linked gene critical for nucleotide metabolism. Pathogenic variants cause three overlapping phenotypes: Arts syndrome (severe neurological disease), Charcot-Marie-Tooth type 5 [CMTX5] (peripheral neuropathy), and non-syndromic sensorineural hearing loss (SNHL). Each may be associated with retinal dystrophy.
View Article and Find Full Text PDFDavid Ferrier's second monkey ('monkey F'): The inaugural experimental studies of the auditory cortex.
J Hist Neurosci
January 2025
Biosciences Institute, Newcastle University, Newcastle upon Tyne, UK.
The story of David Ferrier's demonstration at the International Medical Congress in London in August 1881 of a monkey experimentally rendered hemiplegic by a focal surgical brain lesion-prompting Charcot's observation, "C'est un malade!"-is well known as a seminal event in the history of the localization of functions in the cerebral cortex. Less well known is the fact that, on the same occasion, Ferrier demonstrated a second monkey, known as monkey F, apparently deaf as a consequence of bilateral temporo-sphenoidal brain lesions. The purpose of this article is, first, to give a chronological account of this demonstration and subsequent related events, including Ferrier's trial under the Vivisection Act, the publication of the pathological findings in the animal's brain, the dispute about the localization of the "auditory centre" with Edward Schäfer, and the first glimmerings of human homologues of cortical deafness.
View Article and Find Full Text PDFNeural correlates of age-related hearing loss: An MRI and FDG-PET study.
Geriatr Gerontol Int
January 2025
Research Team for Neuroimaging, Tokyo Metropolitan Institute for Geriatrics and Gerontology, Tokyo, Japan.
Aim: Age-related hearing loss (ARHL) is a common problem among older adults and contributes to adverse health outcomes such as cognitive impairment. However, the neural mechanisms underlying ARHL remain unclear. We aimed to reveal the structural and metabolic (i.
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