Noise-induced hearing loss (NIHL) is one of the more common sources of environmentally induced hearing loss in adults. In a mouse model, Castaneous (CAST/Ei) is an inbred strain that is resistant to NIHL, while the C57BL/6J strain is susceptible. We have used the genome-tagged mice (GTM) library of congenic strains, carrying defined segments of the CAST/Ei genome introgressed onto the C57BL/6J background, to search for loci modifying the noise-induced damage seen in the C57BL/6J strain. NIHL was induced by exposing 6-8-week old mice to 108 dB SPL intensity noise. We tested the hearing of each mouse strain up to 23 days after noise exposure using auditory brainstem response (ABR). This study identifies a number of genetic loci that modify the initial response to damaging noise, as well as long-term recovery. The data suggest that multiple alleles within the CAST/Ei genome modify the pathogenesis of NIHL and that screening congenic libraries for loci that underlie traits of interest can be easily carried out in a high-throughput fashion.
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http://dx.doi.org/10.1007/s00335-009-9178-5 | DOI Listing |
Clin Case Rep
January 2025
Department of Otolaryngology-Head and Neck Surgery University of California, San Francisco (UCSF) San Francisco California USA.
In this report, we describe the first reported case of an oropharyngeal accessory tragus in a 20-year-old female presenting with moderate to severe left-sided conductive hearing loss. This case shares rare developmental anomalies to consider when diagnosing oropharyngeal masses that highlight the intricate embryological development of the head and neck region.
View Article and Find Full Text PDFNat Sci Sleep
December 2024
Graduate Institute of Life Sciences, National Defense Medical Center, Taipei, Taiwan.
Purpose: Benzodiazepine receptor agonists (BZRAs), including benzodiazepines (BZDs) and Z drugs, are widely prescribed for anxiety and sleep. Therefore, issues of tolerance, dependence and adverse effects are of concern. Recent studies suggested a potential link between BZRAs and hearing problems.
View Article and Find Full Text PDFJ Otol
July 2024
Department of Otolaryngology-Head & Neck Surgery, The Sixth Medical Center of PLA General Hospital, Beijing, China.
Cochlear implantation (CI) is currently recognized as the most effective treatment for severe to profound sensorineural deafness and is considered one of the most successful neural prostheses. Since its inception in 1961, cochlear implantation has expanded its range of applications to encompass younger newborns, older people, and individuals with unilateral hearing loss. In addition, it has improved its surgical methods to minimize the occurrence of complications.
View Article and Find Full Text PDFObjectives: The movement towards personalization of cochlear implantation has continued to generate interest about variabilities in cochlear size. In a recent meta-analysis, Atalay et al. (2022) examined organ of corti length, cochlear lateral wall, and "A" value and found that most covariates, other than congenital sensorineural hearing loss, did not impact cochlear size via these measurements.
View Article and Find Full Text PDFJ Otol
July 2024
Department of ENT and Head-Neck Surgery, Seth G.S. Medical College and KEM Hospital, Acharya Donde Marg, Parel, Mumbai, Maharashtra, 400012, India.
Thiamine responsive megaloblastic anemia (TRMA), also known as Roger's syndrome, is an exceptionally rare autosomal recessive disorder stemming from mutations in the SLC19A2 gene responsible for encoding a thiamine carrier protein. This syndrome manifests as the classic triad of megaloblastic anemia, sensorineural hearing loss, and diabetes mellitus. Here, we present the case of a one-and-a-half-year-old male infant born to non-consanguineous parents in India, a region where TRMA cases are seldom reported.
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