scholarly journals Measurement of Thresholds Using Auditory Steady-State Response and Cochlear Microphonics in Children with Auditory Neuropathy

2019 ◽  
Vol 30 (08) ◽  
pp. 672-676 ◽  
Author(s):  
Ping Lu ◽  
Yue Huang ◽  
Wen-Xia Chen ◽  
Wen Jiang ◽  
Ni-Yi Hua ◽  
...  

AbstractThe detection of precise hearing thresholds in infants and children with auditory neuropathy (AN) is challenging with current objective methods, especially in those younger than six months of age.The aim of this study was to compare the thresholds using auditory steady-state response (ASSR) and cochlear microphonics (CM) in children with AN and children with normal hearing.The thresholds of CM, ASSR, and visual reinforcement audiometry (VRA) tests were recorded; the ASSR and VRA frequencies used were 250, 500, 1000, 2000, and 4000 Hz.The participants in this study were 15 children with AN (27 ears) (1–7.6 years, median age 4.1 years) and ten children with normal hearing (20 ears) (1–8 years, median age four years).The thresholds of the three methods were compared, and histograms were used to represent frequency distributions of threshold differences obtained from the three methods.In children with normal hearing, the average CM thresholds (84.5 dB) were significantly higher than the VRA thresholds (10.0–10.8 dB); in children with AN, both CM and VRA responses were seen at high signal levels (88.9 dB and 70.6–103.4 dB, respectively). In normal children, the difference between mean VRA and ASSR thresholds ranged from 17.5 to 30.3 dB, which was significantly smaller than the difference seen between the mean CM and VRA thresholds (71.5–72.3 dB). The correlation between VRA and ASSR in children with normal hearing ranged from 0.38 to 0.48, whereas no such correlation was seen in children with AN at any frequency (0.03–0.19).Our results indicated that ASSR and CM were poor predictors of the conventional behavioral threshold in children with AN.

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Pei-Hsuan Lin ◽  
Chuan-Jen Hsu ◽  
Yin-Hung Lin ◽  
Yi-Hsin Lin ◽  
Shu-Yu Yang ◽  
...  

Abstract Auditory neuropathy is an important entity in childhood sensorineural hearing loss. Due to diverse etiologies and clinical features, the management is often challenging. This study used an integrative patient-history, audiologic, genetic, and imaging-based approach to investigate the etiologies and audiologic features of 101 children with auditory neuropathy. Etiologically, 48 (47.5%), 16 (15.8%), 11 (10.9%), and 26 (25.7%) children were categorized as having acquired, genetic, cochlear nerve deficiency-related, and indefinite auditory neuropathy, respectively. The most common causes of acquired and genetic auditory neuropathy were prematurity and OTOF mutations, respectively. Patients with acquired auditory neuropathy presented hearing loss earlier (odds ratio, 10.2; 95% confidence interval, 2.2–47.4), whereas patients with genetic auditory neuropathy had higher presence rate of distortion product otoacoustic emissions (odds ratio, 10.7; 95% confidence interval, 1.3–85.4). In patients with different etiologies or pathological sites, moderate to strong correlations (Pearson’s r = 0.51–0.83) were observed between behavioral thresholds and auditory steady-state response thresholds. In conclusion, comprehensive assessments can provide etiological clues in ~75% of the children with auditory neuropathy. Different etiologies are associated with different audiologic features, and auditory steady-state responses might serve as an objective measure for estimating behavioral thresholds.


2010 ◽  
Vol 124 (9) ◽  
pp. 950-956 ◽  
Author(s):  
A A Emara ◽  
T A Gabr

AbstractReview:Auditory neuropathy is a disorder characterised by preservation of outer hair cell function, with normal otoacoustic emissions and/or cochlear microphonics, but an absent or distorted auditory brainstem response.Purpose:This study aimed to objectively assess hearing thresholds in patients with auditory neuropathy, using the auditory steady state response.Materials and methods:Thirteen patients with auditory neuropathy and 15 normal hearing subjects were examined. Audiological evaluation included basic audiological tests, otoacoustic emissions, auditory brainstem response and auditory steady state response.Results:In the auditory neuropathy patients, the auditory brainstem response was absent in 11 patients, while the auditory steady state response was absent in only three.Conclusion:The auditory steady state response may serve as a valuable objective measure for assessing the hearing threshold across different frequencies in patients with auditory neuropathy. We recommend that auditory steady state response be used to complete the evaluation of patients with auditory neuropathy.


2008 ◽  
Vol 235 (1-2) ◽  
pp. 105-113 ◽  
Author(s):  
Mikaël Ménard ◽  
Stéphane Gallégo ◽  
Christian Berger-Vachon ◽  
Lionel Collet ◽  
Hung Thai-Van

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