A detailed description of the physiology of the auditory system (including new essential knowledge) is provided, followed by the description of five morphological levels of acoustic signal analysis, of which four play a significant part in establishing the selection capacity of human hearing. As an essential phenomenon connected with loss of the analyzing capacity of the ear, ascending degeneration of the afferent nerve fibers of the inner hair cells and of the hearing nerve (after destruction of the inner hair cells) develops. A distorted-speech test is submitted that offers a simple and easily performed calculation of a ratio, which value renders a reliable estimation of the pathological analyzing capacity of the ear caused by ascending degeneration. The test is recommended as a reliable basic component of an audiometric battery of tests.
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Acta Otolaryngol
January 2025
Department of Otomicrosurgery, Sixth Medical Center of The PLA General Hospital, Beijing, China.
Background: In some rare cases of congenital aplasia of the oval window (OW), malformed facial nerve (FN) locations covering the most or entire OW present a challenge to hearing reconstruction, there is no a highly effective surgical hearing reconstruction methods.
Aims/objectives: To update a Scala tympani drill-out technique (SDT) for abnormal FN course covering the OW.
Material And Methods: All patients of congenital atresia of the OW was recruited between August 2014 and July 2023 in a tertiary-care center.
J Comp Eff Res
January 2025
Head of the 3rd Neuropsychiatric Department of the Research Clinical Institute of Childhood of the Moscow Region, Moscow, Russia.
What Is This Summary About?: Neurofibromatosis type 1 (also called NF1) is a rare genetic condition. It causes a range of symptoms that develop from childhood onwards and worsen over time. Some children with NF1 develop non-cancerous nerve tumors called plexiform neurofibromas.
View Article and Find Full Text PDFFront Neurol
January 2025
Oregon Hearing Research Center, Oregon Health & Science University, Portland, OR, United States.
Introduction: The brainstem vestibular nuclei neurons receive synaptic inputs from inner ear acceleration-sensing hair cells, cerebellar output neurons, and ascending signals from spinal proprioceptive-related neurons. The lateral (LVST) and medial (MVST) vestibulospinal (VS) tracts convey their coded signals to the spinal circuits to rapidly counter externally imposed perturbations to facilitate stability and provide a framework for self-generated head movements.
Methods: The present study describes the morphological characteristics of intraaxonally recorded and labeled VS neurons monosynaptically connected to the 8th nerve.
JBMR Plus
February 2025
Radiology and Imaging Sciences, National Institutes of Health Clinical Center, National Institutes of Health, Bethesda, MD 20892, United States.
Jansen metaphyseal chondrodysplasia (JMC) is an ultra-rare disorder caused by constitutive activation of parathyroid hormone type 1 receptor (PTH1R). We sought to characterize the craniofacial phenotype of patients with the disease. Six patients with genetically confirmed JMC underwent comprehensive craniofacial phenotyping revealing a distinct facial appearance that prompted a cephalometric analysis demonstrating a pattern of mandibular retrognathia.
View Article and Find Full Text PDFEur Arch Otorhinolaryngol
January 2025
Vrije Universiteit Brussel, Brussels Health Centre, Brussels, Belgium.
Purpose: Cochlear implants (CI) are the most successful bioprosthesis in medicine probably due to the tonotopic anatomy of the auditory pathway and of course the brain plasticity. Correct placement of the CI arrays, respecting the inner ear anatomy are therefore important. The ideal trajectory to insert a cochlear implant array is defined by an entrance through the round window membrane and continues as long as possible parallel to the basal turn of the cochlea.
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