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The vestibular and oculomotor dysfunction in Fabry disease: a cohort study in China.
Ann Med
December 2025
Department of Neurology, Peking University First Hospital, China.
Objective: Whereas a few studies have evaluated vestibular involvement in Fabry disease (FD), the relationship between vestibular/oculomotor abnormalities and disease-specific biomarkers remain unclear. Therefore, we seek to evaluate these quantitatively and analyze their relationship with disease phenotype and biomarkers in FD.
Methods: This cohort study enrolled 37 Chinese FD patients registered in our center.
Cochlear Mechanics Are Preserved After Inner Ear Delivery of Gold Nanoparticles.
Int J Mol Sci
December 2024
Caruso Department of Otolaryngology-Head and Neck Surgery, University of Southern California, Los Angeles, CA 90033, USA.
Novel therapeutic delivery systems and delivery methods to the inner ear are necessary to treat hearing loss and inner ear disorders. However, numerous barriers exist to therapeutic delivery into the bone-encased and immune-privileged environment of the inner ear and cochlea, which makes treating inner ear disorders challenging. Nanoparticles (NPs) are a type of therapeutic delivery system that can be engineered for multiple purposes, and posterior semicircular canal (PSCC) infusion is a method to directly deposit them into the cochlea.
View Article and Find Full Text PDF[Experiences of poor recovery after total endoscopic middle ear surgery].
Lin Chuang Er Bi Yan Hou Tou Jing Wai Ke Za Zhi
January 2025
To investigate the occurrence and managements of poor recovery after total endoscopic middle ear surgery. A total of 302 cases（315 ears） who underwent endoscopic middle ear surgery in our hospital from June 2020 to June 2021 were collected. Follow up by means of endoscopy, pure tone hearing threshold, tympanogram was conducted at 1 month, 3 months, 6 months and 1 year after surgery to analyze the incidence, possible causes, treatment strategies and effects of poor results tympanic membrane healing and hearing recovery.
View Article and Find Full Text PDFEvaluation of auditory pathways and comorbid inner ear malformations in pediatric patients with Duane retraction syndrome.
Int J Pediatr Otorhinolaryngol
January 2025
Hacettepe University, Faculty of Medicine, Department of Opthalmology, Ankara, Turkey.
Aims And Objectives: This study aimed to investigate the presence, type, and severity of hearing losses in individuals with Duane Retraction Syndrome (DRS), and to ascertain if there are anomalies in the auditory pathways at the brainstem level in DRS, believed to arise from aberrant interaction between cranial nerves and brainstem nuclei.
Study Design: Cross-sectional observational study.
Setting: Tertiary referral centre.
Dorsolateral pontine lesions produce distinct ocular motor abnormalities with anatomical correlations.
Eur J Neurol
January 2025
Department of Neurology, Pusan National University Hospital, Pusan National University School of Medicine and Biomedical Research Institute, Busan, South Korea.
Background And Purpose: The dorsolateral portion of the caudal pons contains the vestibular nucleus (VN) and inferior cerebellar peduncle (ICP) that play important roles in conveying and processing vestibular and ocular motor signals. This study aimed to characterize ocular motor abnormalities along with their anatomical correlations in dorsolateral pons (DLP) lesions.
Methods: We analyzed clinical features, and results of neuro-otological evaluations and neuroimaging of 18 patients with unilateral DLP lesions (17 with DLP infarction and 1 with cavernous malformation) from among 506 patients with pontine infarction in a stroke registry.
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