Down syndrome (DS) is the most common chromosomal disorder and a significant cause of intellectual disability, with children at increased risk for hearing loss. This study investigates the prevalence and characteristics of conductive hearing loss in Indian children with DS. Fifty children with karyotypically confirmed DS, aged 2 to 18 years, were examined at a tertiary care hospital. Otolaryngological assessments included evaluations of the external ear and tympanic membrane. Audiometric evaluations involved pure tone audiometry (PTA), impedance audiometry, and brainstem evoked response audiometry (BERA). Interventions like wax removal and tympanostomy tube placement were performed as needed, with follow-ups after three months. Conductive hearing loss was found in 72% of participants. Common issues included impacted cerumen (66%) and tympanic membrane retraction (34%). Sixteen patients received wax removal, and 7 of 17 with serous otitis media underwent tympanostomy tube placement. Significant improvements in hearing thresholds were observed post-intervention. The high prevalence of conductive hearing loss and ear abnormalities in children with DS highlights the need for regular audiological evaluations and timely interventions to enhance educational outcomes and quality of life.
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http://dx.doi.org/10.1007/s12070-024-05233-2 | DOI Listing |
Elife
March 2025
Department of Neuroscience, Georgetown University Medical Center, Washington DC, United States.
Research on brain plasticity, particularly in the context of deafness, consistently emphasizes the reorganization of the auditory cortex. But to what extent do all individuals with deafness show the same level of reorganization? To address this question, we examined the individual differences in functional connectivity (FC) from the deprived auditory cortex. Our findings demonstrate remarkable differentiation between individuals deriving from the absence of shared auditory experiences, resulting in heightened FC variability among deaf individuals, compared to more consistent FC in the hearing group.
View Article and Find Full Text PDFEndokrynol Pol
March 2025
Department of Clinical Laboratory, the First Affiliated Hospital of Guangxi Medical University, Key Laboratory of Clinical Laboratory Medicine of Guangxi Department of Education, Nanning, Guangxi, China.
Introduction: Thiamine-responsive megaloblastic anaemia syndrome (TRMA) is a rare genetic disease caused by mutations in the SLC19A2 gene that encodes thiamine transporter 1 (THTR-1). The common manifestations are diabetes, anaemia, and deafness. The pathogenic mechanism has not yet been clarified.
View Article and Find Full Text PDFAnn Otol Rhinol Laryngol
March 2025
Department of Mechanical Engineering, National Chung Hsing University, Taichung, Taiwan (R.O.C.).
Objectives: This study compared a simplified in situ self-administered hearing screening test, conducted with a neckband-type self-fitting device, with conventional pure-tone audiometry. It evaluated the maximum speech-shaped noise level for screening (MSNLS), crucial for evaluating the feasibility of this in situ screening test in quiet environments.
Methods: This study included 30 adults with normal hearing and 30 adults with mild to moderately severe hearing impairment.
Indian J Otolaryngol Head Neck Surg
January 2025
Department of Neuro-Otology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh India.
Unlabelled: In India, 7-8% of the population is affected by post lingual deafness, which can lead to job dissatisfaction, depression, social withdrawal, and educational challenges. Despite the benefits, cochlear implantation in Northern India remains underutilized. This study examines the impact of cochlear implants on post lingual deafness, along with their etiology and indications.
View Article and Find Full Text PDFIndian J Otolaryngol Head Neck Surg
January 2025
Department of Otorhinolaryngology, All India Institute of Medical Sciences, Jodhpur Mobile No: + 91, Jodhpur, 8547956262 India.
Unlabelled: Background: Petrous bone cholesteatoma (PBC) is a challenging condition involving the development of an epidermoid cyst within the petrous portion of the temporal bone. Advances in radiological imaging and skull base surgery have refined the management of PBC. Methods: An ambispective descriptive study was conducted on patients diagnosed with PBC between 2021 and 2024.
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