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.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7575403PMC
http://dx.doi.org/10.1055/s-0039-3402441DOI Listing

Publication Analysis

Top Keywords

individuals ansd
24
individuals normal
16
p300 response
12
individuals
12
discrimination skill
12
normal hearing
12
neuropathy spectrum
8
spectrum disorder
8
neural discrimination
8
presence noise
8

Similar Publications

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 PDF

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.

View Article and Find Full Text PDF

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 PDF
Article Synopsis
  • - The study aims to map the tonotopy (frequency organization) of the human cochlea in vivo using cochlear implant electrodes, addressing challenges of past research that relied on cadaver and animal models.
  • - Fifty patients with hearing loss underwent cochlear implantation, where their responses to sound stimuli were recorded to analyze how sound intensity and an artificial "third window" influence the tonotopic map.
  • - Results showed notable deviations from the expected Greenwood model in the frequency-position function, especially at higher sound levels, indicating complexities in how the cochlea processes sound intensity.
View Article and Find Full Text PDF

Measurement of thresholds using Chirp-ABR in children with auditory neuropathy spectrum disorder and sensorineural hearing loss.

Int J Pediatr Otorhinolaryngol

September 2024

Department of Otolaryngology-Head and Neck Surgery, Children's Hospital of Fudan University, Shanghai, China. Electronic address:

Article Synopsis
  • The study investigated the effectiveness of Chirp-auditory brainstem response (ABR) in measuring hearing thresholds in children with auditory neuropathy spectrum disorder (ANSD).
  • A total of 20 children with ANSD, 31 with sensorineural hearing loss (SNHL), and 25 normal children participated, undergoing both Chirp-ABR and behavioral audiometry for comparison.
  • Results showed that while Chirp-ABR correlated well with behavioral audiometry in SNHL, it was less reliable for ANSD, particularly when discrepancies exceeded 30 dB, suggesting ANSD should be strongly considered in such cases.
View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!