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Charcot-Marie-Tooth (CMT) disease is a heterogeneous hereditary motor and sensory neuropathy of the peripheral nervous system, with CMT1A in particular being the most common form. We encountered a 76-year-old woman with CMT1A who had a history of pain attacks and hearing loss from a young age, with motor symptoms manifesting late in life. Her pain and hearing loss may have been related to CMT. Our case also raises the possibility that neuropathic pain and hearing loss may precede the classic motor symptoms of CMT1A.
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http://dx.doi.org/10.2169/internalmedicine.1883-23 | DOI Listing |
Introduction: Some medical conditions may be associated with increased risks of collision and poor performance while driving. Traffic crashes could result in fatalities and injuries. The Australian national medical guidelines do not provide specific instructions for all medical conditions.
View Article and Find Full Text PDFCereb Cortex
August 2025
Department of Psychology, University of Lübeck, Ratzeburger Allee 160, Lübeck 23562, Germany.
The human auditory system must distinguish relevant sounds from noise. Severe hearing loss can be treated with cochlear implants (CIs), but how the brain adapts to electrical hearing remains unclear. This study examined adaptation to unilateral CI use in the first and seventh months after CI activation using speech comprehension measures and electroencephalography recordings, both during passive listening and an active spatial listening task.
View Article and Find Full Text PDFMol Biol Rep
September 2025
Cytogenetics and Molecular Genetics Lab, Pathology Unit, Medical Division (BARC Hospital), Bhabha Atomic Research Centre, Anushakti Nagar, Mumbai, India.
Background: Hearing loss (HL) is one of the most common congenital anomalies and is a complex etiologically diverse condition. Molecular genetic characterization of HL remains challenging owing to the high genetic heterogeneity. This study aimed to screen for potential disease-causing genetic variations in a cohort of Indian patients with congenital bilateral severe-to-profound sensorineural HL.
View Article and Find Full Text PDFBrain Behav
September 2025
Radiology Department, Yantaishan Hospital, Yantai, Shandong, China.
Objective: To investigate the characteristics of brain structures in patients with noise-induced hearing loss (NIHL) using source-based morphometry (SBM) and to evaluate the correlation between abnormal brain regions and clinical data.
Methods: High-resolution 3D T1 structural images were acquired from 81 patients with NIHL and 74 age- and education level-matched healthy controls (HCs). The clinical data of all subjects were collected, including noise exposure time, monaural hearing threshold weighted values (MTWVs), Mini-Mental State Examination (MMSE), and Hamilton Anxiety Scale (HAMA) scores.