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http://dx.doi.org/10.1001/jamaoto.2018.3073 | DOI Listing |
J Kidney Cancer VHL
December 2024
Department of Urology, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA.
Von Hippel-Lindau (vHL) is a hereditary disease characterized by the development of benign and malignant tumors across multiple organ systems. It is seen in approximately 1 in 36,000 live births. Given that vHL is a rare disease, studies that seek to characterize vHL are often hampered by small sample sizes.
View Article and Find Full Text PDFInt J Pediatr Otorhinolaryngol
November 2024
Department of Paediatric Otolaryngology, Robert Debre Hospital, Assistance Publique Hôpitaux de Paris (APHP), Paris Cité University, Paris, France; Center for Balance Evaluation in Children (EFEE), Department of Paediatric Otolaryngology, Robert Debre Hospital, Assistance Publique Hôpitaux de Paris (APHP), Paris Cité University, Paris, France.
Objectives: To describe the inner ear sectors after an inner ear MRI protocol and search for the presence of endolymphatic pressure anomaly in patients presenting with a congenital CMV infection and audio-vestibular dysfunction.
Methods: A 3D FLAIR MRI sequence, 4 h after gadolinium injection, was performed in patients with sensory-neural hearing loss secondary to a congenital CMV infection in order to analyse the morphology of the endolymphatic space.
Results: Two patients presented with a unilateral SNHL and 4 patients a bilateral SNHL.
Front Oncol
November 2024
Department of Otolaryngology Head and Neck Surgery, Medical University of Lublin, Lublin, Poland.
Introduction: Endolymphatic sac tumors (ELSTs) are rare neuroectodermal neoplasms that originate in the endolymphatic sac and duct. They exhibit no specific age or gender predilection, although they are more prevalent in patients with von Hippel-Lindau syndrome.
Material And Methods: The manuscript preparation adhered to the CARE guidelines for standardizing clinical cases and the PRISMA guidelines for scientific reviews.
Front Neurol
May 2024
Department of Surgical Sciences, Otorhinolaryngology and Head and Neck Surgery, Uppsala University, Uppsala, Sweden.
Background: Despite its location near infection-prone areas, the human inner ear demonstrates remarkable resilience. This suggests that there are inherent instruments deterring the invasion and spread of pathogens into the inner ear. Here, we combined high-resolution light microscopy, super-resolution immunohistochemistry (SR-SIM) and synchrotron phase contrast imaging (SR-PCI) to identify the protection and barrier systems in the various parts of the human inner ear, focusing on the lateral wall, spiral ganglion, and endolymphatic sac.
View Article and Find Full Text PDFBiochim Biophys Acta Mol Basis Dis
June 2024
Department of Neuroscience and Cell Biology, Osaka University Graduate School of Medicine, Osaka 565-0871, Japan; Addiction Research Unit, Osaka Psychiatric Research Center, Osaka Psychiatric Medical Center, Osaka 541-8567, Japan; Global Center for Medical Engineering and Informatics, Osaka University, Suita 565-0871, Japan.
Autoimmune inner ear disease (AIED) is an organ-specific disease characterized by irreversible, prolonged, and progressive hearing and equilibrium dysfunctions. The primary symptoms of AIED include asymmetric sensorineural hearing loss accompanied by vertigo, aural fullness, and tinnitus. AIED is divided into primary and secondary types.
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