Auditory neuropathy spectrum disorder (ANSD) is a clinical condition in which individuals have normal cochlear responses and abnormal neural responses. There is a lack of evidence in the literature regarding the neural discrimination skill in individuals with ANSD, especially when the signal is presented in the presence of noise. The present study was performed with the aim to investigate auditory discrimination skill, in quiet and in the presence of noise, in individuals with ANSD and to compare the findings with normal-hearing individuals. A total of 30 individuals with normal hearing sensitivity and 30 individuals with ANSD in the age range of 15 to 55 years old, with the mean age of 27.86 years old, were the participants. P300 response was recorded from both groups using syllable pairs /ba/-/da/ in oddball paradigm and the syllable /da/ in repetitive paradigm in quiet and at +10 dB signal-to-noise ratio (SNR). There was significant prolongation in latency and reaction time, and reduction in amplitude of P300 response and sensitivity in both groups with the addition of noise. The topographic pattern analysis showed activation of the central-parietal-occipital region of the brain in individuals with ANSD, whereas activation of the central-parietal region was observed in individuals with normal hearing. The activation was more diffused in individuals with ANSD compared with that of individuals with normal hearing. The individuals with ANSD showed a significantly more adverse effect of noise on the neural discrimination skill than the normal counterpart.
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http://dx.doi.org/10.1055/s-0039-3402441 | DOI Listing |
Int J Mol Sci
November 2024
The Center for Precision Genome Editing and Genetic Technologies for Biomedicine, Pirogov Russian National Research Medical University, 117513 Moscow, Russia.
Auditory neuropathy spectrum disorder (ANSD) is often missed by standard hearing tests, accounting for up to 10% of hearing impairments (HI) and commonly linked to variants in 23 genes. We assessed 122 children with HI, including 102 with sensorineural hearing loss (SNHL) and 20 with ANSD. SNHL patients were genotyped for common variants using qPCR, while ANSD patients underwent whole exome sequencing, with variants analyzed across 249 genes.
View Article and Find Full Text PDFJ Int Adv Otol
November 2024
Department of Audiology, All India Institute of Speech and Hearing, Mysuru, India.
Background: Despite cochlear microphonic's potential clinical application, especially in ANSD diagnosis, the optimal parameters to record cochlear microphonics and the effect of various stimulus parameters are not well understood yet, which makes its recording a difficult procedure. The present study was undertaken to determine the effect of stimulus polarity, rate, stimulus type, and stimulus frequency on different aspects of cochlear microphonics, which could help to decide an optimal stimulus parameter that can be used to record CM.
Methods: The study involved 32 normal-hearing adults.
Mol Biol Rep
November 2024
Cellular and Molecular Research Center, Gerash University of Medical Sciences, Gerash, Iran.
Background: Chronic Myeloid Leukemia (CML), accounting for 15-20% of adult leukemia cases, is marked by the Philadelphia chromosome, resulting from the t(9;22)(q34;q11) translocation. This leads to uncontrolled cell proliferation and survival. Imatinib therapy lowers BCR-ABL levels, influencing telomere-associated proteins and increasing telomerase accessibility, indirectly boosting its activity.
View Article and Find Full Text PDFEar Hear
December 2024
Department of Otolaryngology-Head and Neck Surgery, Washington University School of Medicine in St. Louis, St Louis, Missouri, USA.
Int J Pediatr Otorhinolaryngol
September 2024
Department of Otolaryngology-Head and Neck Surgery, Children's Hospital of Fudan University, Shanghai, China. Electronic address:
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