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Radiological Perspectives in Congenital Sensorineural Hearing Loss: Insights from Cochlear Implant Candidates.
J Clin Med
December 2024
Department of Radiology, Cerrahpaşa Medical Faculty, İstanbul University-Cerrahpaşa, 34147 Istanbul, Türkiye.
Congenital hearing loss is a significant health concern, with diverse etiologies encompassing cochlear and cochleovestibular pathologies. Preoperative radiological evaluation in cochlear implant candidates is pivotal for treatment planning. We aim to elucidate the spectrum of radiological findings in patients with congenital hearing loss undergoing cochlear implant assessment.
View Article and Find Full Text PDF[Evaluation of inner ear malformation based on high-resolution CT and MRI].
Lin Chuang Er Bi Yan Hou Tou Jing Wai Ke Za Zhi
January 2025
To explore the value of high resolution computed tomography（HRCT） combined with Magnetic Resonance Imaging（MRI） in the diagnosis of inner ear malformation. HRCT and MRI data of 82 patients with inner ear malformations were analyzed retrospectively. HRCT MPR and CPR reconstruction of the inner ear structure, facial nerve canal and oblique sagittal MRI reconstruction of the internal auditory canal were performed.
View Article and Find Full Text PDFMesencephalosynapsis and aqueductal stenosis.
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Fetal Medicine Unit, Ontario Fetal Center, Department of Obstetrics and Gynaecology, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada.
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View Article and Find Full Text PDFClinical Phenotypic Characterization of the SLC26A4 Mutation in Pendred Syndrome/Nonsyndromic Enlarged Vestibular Aqueduct.
Laryngoscope
February 2025
Senior Department of Otolaryngology-Head & Neck Surgery, The Sixth Medical Center of PLA General Hospital, PLA Medical School, Beijing, China.
Article Synopsis
- The study focuses on the mutations in the SLC26A4 gene and the clinical features of Pendred syndrome and nonsyndromic enlarged vestibular aqueduct (PS/NSEVA) in a Chinese patient population.
- Researchers analyzed hearing test results from 406 patients, finding significant differences in hearing loss among those with various types of SLC26A4 mutations, but not related to sex or the exact mutation type.
- Conclusions highlight that SLC26A4 mutations, age, and the presence of specific ear malformations are important for understanding hearing loss, while mutation type and patient demographics had little impact on auditory outcomes.
Short- and Long-Term Outcomes of Prenatally Identified Congenital Aqueductal Stenosis by Fetal MRI.
Prenat Diagn
December 2024
Division of Neurology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
Objective: Providing accurate prenatal prognostication for expectant parents is challenging due to limited literature on factors impacting outcomes in children with congenital aqueductal stenosis (CAS). This study stratified CAS patients into isolated or complex categories (presence of additional intra- or extra-cranial anomalies or genetic syndromes) and evaluated both short- and long-term outcomes. Additionally, the role of ventricular rupture was assessed.
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