Auditory neuropathy spectrum disorder (ANSD) features the presence of otoacoustic emissions, poor speech identification score and absent auditory brainstem response. The present study was designed to evaluate the functioning of all six semicircular canals in individuals with ANSD and to compare it with those of normal-hearing individuals. A total of 50 individuals participated in the present study, in which Group I comprised 25 normal-hearing individuals, and Group II comprised 25 individuals with ANSD. All of the participants underwent case history, pure tone audiometry, immittance, otoacoustic emissions, auditory evoked response and video head impulse test (vHIT). The independent sample t-test revealed significantly lower vestibulo-ocular reflex gain values in individuals with ANSD. A presence of 100% corrective refixation saccades was observed in the same group. The Pearson correlation test revealed no significant correlation between vestibulo-ocular reflex (VOR) gain with duration of hearing loss and pure tone thresholds for any of the three orthogonal planes. The chi-squared test revealed no association between the VOR gain values and the presence or absence of saccades in any of the semicircular canals ( > 0.05). Huge percentages of individuals with ANSD have been found to have associated vestibular dysfunction as well. Therefore, the vHIT can be used as one of the important tests of the vestibular test battery to evaluate all six semicircular canals in individuals with ANSD.
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http://dx.doi.org/10.1055/s-0039-1697004 | 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:
Aim: This study explored the value of Chirp-auditory brainstem response (ABR) thresholds in assessing the hearing threshold of children diagnosed with auditory neuropathy spectrum disorder (ANSD).
Methods: A total of 20 children with ANSD (40 ears, aged 1.5-7.
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