Gender Differences in Audiological Findings and Hearing Aid Benefit in 255 Individuals with Auditory Neuropathy Spectrum Disorder: A Retrospective Study

2016 ◽  
Vol 27 (10) ◽  
pp. 839-845 ◽  
Author(s):  
Vijaya Kumar Narne ◽  
Prashanth Prabhu ◽  
Hunsur S. Chandan ◽  
Mahadeva Deepthi

Background: There are many studies reported in the literature that have summarized audiological findings and possible rehabilitation in individuals with auditory neuropathy spectrum disorder (ANSD). However, there are very few studies that have attempted to delineate the gender differences in audiological characteristics and hearing aid benefit in individuals with ANSD. Purpose: The study aimed to explore the differences between males and females in terms of demographic details, audiogram, speech identification scores, otoacoustic emissions, acoustic reflexes, long latency responses, and hearing aid benefit. Research Design: A retrospective study. Study Sample: A total of 255 individuals diagnosed with ANSD were selected for the study. The study included 137 females and 88 males. Data Collection and Analysis: The demographic details, results of diagnostic audiological testing, and hearing aid benefit were analyzed retrospectively. The differences in findings across gender were compared. Results: The study shows that females have a relatively higher degree of hearing loss and that the majority of females show a rising type of audiometric configuration. The study shows that females have poorer speech perception abilities and experience limited benefits from hearing aids compared to males. Conclusions: The results of the study show that there are gender differences in audiological findings and hearing aid benefits in individuals with ANSD. However, well-controlled prospective studies are essential to confirm the results obtained and to identify the possible mechanisms underlying the gender differences.

2016 ◽  
Vol 21 (03) ◽  
pp. 243-249 ◽  
Author(s):  
Prashanth Prabhu ◽  
Animesh Barman

Introduction The studies on hearing aid benefit in individuals with auditory neuropathy spectrum disorder (ANSD) shows limited benefit. Low cut modified amplification is found to be effective in few individuals with ANSD. With advancement in technology, receiver in the canal (RIC) hearing aids have proven to be more effective than traditional behind the ear (BTE) hearing aids. Objective Thus, the present study attempts to determine the effectiveness of low cut modified amplification using RIC and BTE. Method Twenty participants with ANSD were fitted with BTE and RIC using traditional and low cut modified amplification. We divided them into good and poor performers based on unaided speech identification scores (SIS). We then compared aided SIS and aided benefit across conditions in good and poor performers with ANSD across both conditions using BTE and RIC. Results The results of the study showed that the aided performance improved with low cut modified amplification in both BTE and RIC hearing aids. The improvement noticed with low-cut modified fitting with RIC was significant in more than BTE, especially in good performers with ANSD. Conclusion The improved clarity and naturalness of sound with RIC may have led to better aided scores and better acceptance of the hearing aid. Thus, low-cut modified amplification, preferably with RIC, needs to be attempted in fitting individuals with ANSD, especially in those with good unaided SIS in quiet.


2020 ◽  
Vol 50 (3) ◽  
pp. 246-248
Author(s):  
Helen Brough

Auditory neuropathy spectrum disorder (ANSD) can cause significant hearing impairment; it occurs when there is intact outer hair cell function in the inner ear, with a dyssynchronous neural response, thought to be due to dysfunction of the inner hair cells (IHCs), the synapse of the IHCs and the auditory nerve, or of the auditory nerve itself. This case report describes the onset of ANSD in a Malawian child after severe malaria treated with quinine. Diagnosis of ANSD was made by confirming the presence of otoacoustic emissions, together with the absence of auditory brainstem response and absent acoustic reflexes.


2021 ◽  
Vol 42 (5) ◽  
pp. 103057
Author(s):  
Firas Sbeih ◽  
Donald M. Goldberg ◽  
Sara Liu ◽  
Maxwell Y. Lee ◽  
Gina Stillitano ◽  
...  

2019 ◽  
Vol 24 (02) ◽  
pp. e140-e148
Author(s):  
Sujeet Kumar Sinha ◽  
Anuj Kumar Neupane ◽  
Krithika Gururaj

Abstract Introduction Auditory neuropathy spectrum disorder (ANSD) features the presence of otoacoustic emissions, poor speech identification score and absent auditory brainstem response. Objective 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. Methods 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). Results 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 (p > 0.05). Conclusion 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.


2011 ◽  
Vol 22 (09) ◽  
pp. 567-577 ◽  
Author(s):  
Christina L. Runge ◽  
Jamie Jensen ◽  
David R. Friedland ◽  
Ruth Y. Litovsky ◽  
Sergey Tarima

Background: The challenges associated with auditory neuropathy spectrum disorder (ANSD) are due primarily to temporal impairment and therefore tend to affect perception of low- to midfrequency sounds. A common treatment option for severe impairment in ANSD is cochlear implantation, and because the degree of impairment is unrelated to degree of hearing loss by audiometric thresholds, this population may have significant acoustic sensitivity in the contralateral ear. Clinically, the question arises as to how we should treat the contralateral ear in this population when there is acoustic hearing—should we plug it, amplify it, implant it, or leave it alone? Purpose: The purpose of this study was to examine the effects of acute amplification and plugging of the contralateral ear compared to no intervention in implanted children with ANSD and aidable contralateral hearing. It was hypothesized that due to impaired temporal processing in ANSD, contralateral acoustic input would interfere with speech perception achieved with the cochlear implant (CI) alone; therefore, speech perception performance will decline with amplification and improve with occlusion. Research Design: Prospective within-subject comparison. Adaptive speech recognition thresholds (SRTs) for monosyllable and spondee word stimuli were measured in quiet and in noise for the intervention configurations. Study Sample: Nine children treated at the Medical College of Wisconsin Koss Cochlear Implant Program participated in the study. Inclusion criteria for this study were children diagnosed with ANSD who were unilaterally implanted, had aidable hearing in the contralateral ear (defined as a three-frequency pure-tone average of ≤80 dB HL), had at least 1 yr of cochlear implant experience, and were able to perform the speech perception task. Intervention: We compared SRT with the CI alone to SRTs with interventions of cochlear implant with a contralateral hearing aid (CI+HA) and cochlear implant with a contralateral earplug (CI+plug). Data Collection and Analysis: SRTs were measured and compared within subjects across listening conditions. Within-subject comparisons were analyzed using paired t-tests, and analyses of predictive variables for effects of contralateral intervention were analyzed using linear regression. Results: Contrary to the hypothesis, the bimodal CI+HA configuration showed a significant improvement in mean performance over the CI-alone configuration in quiet (p = .04). In noise, SRTs were obtained for six subjects, and no significant bimodal benefit was observed (p = .09). There were no consistent effects of occlusion observed across subjects and stimulus conditions. Degree of bimodal benefit showed a significant relationship with performance with the CI alone, with greater bimodal benefit associated with poorer CI-alone performance (p = .01). This finding, however, was limited by floor effects. Conclusions: The results of this study indicate that children with ANSD who are experienced cochlear implant users may benefit from contralateral amplification, particularly for moderate cochlear implant performers. It is unclear from these data whether long-term contralateral hearing aid use in real-world situations would ultimately benefit this population; however, a hearing aid trial is recommended with assessment of bimodal benefit over time. These data may help inform clinical guidelines for determining optimal hearing configurations for unilaterally implanted children with ANSD, particularly when considering candidacy for sequential cochlear implantation.


2015 ◽  
Vol 26 (10) ◽  
pp. 815-823 ◽  
Author(s):  
Jijo Pottackal Mathai ◽  
Sabarish Appu

Background: Auditory neuropathy spectrum disorder (ANSD) is a form of sensorineural hearing loss, causing severe deficits in speech perception. The perceptual problems of individuals with ANSD were attributed to their temporal processing impairment rather than to reduced audibility. This rendered their rehabilitation difficult using hearing aids. Although hearing aids can restore audibility, compression circuits in a hearing aid might distort the temporal modulations of speech, causing poor aided performance. Therefore, hearing aid settings that preserve the temporal modulations of speech might be an effective way to improve speech perception in ANSD. Purpose: The purpose of the study was to investigate the perception of hearing aid–processed speech in individuals with late-onset ANSD. Research Design: A repeated measures design was used to study the effect of various compression time settings on speech perception and perceived quality. Study Sample: Seventeen individuals with late-onset ANSD within the age range of 20–35 yr participated in the study. Data Collection and Analysis: The word recognition scores (WRSs) and quality judgment of phonemically balanced words, processed using four different compression settings of a hearing aid (slow, medium, fast, and linear), were evaluated. The modulation spectra of hearing aid–processed stimuli were estimated to probe the effect of amplification on the temporal envelope of speech. Repeated measures analysis of variance and post hoc Bonferroni’s pairwise comparisons were used to analyze the word recognition performance and quality judgment. Results: The comparison between unprocessed and all four hearing aid–processed stimuli showed significantly higher perception using the former stimuli. Even though perception of words processed using slow compression time settings of the hearing aids were significantly higher than the fast one, their difference was only 4%. In addition, there were no significant differences in perception between any other hearing aid–processed stimuli. Analysis of the temporal envelope of hearing aid–processed stimuli revealed minimal changes in the temporal envelope across the four hearing aid settings. In terms of quality, the highest number of individuals preferred stimuli processed using slow compression time settings. Individuals who preferred medium ones followed this. However, none of the individuals preferred fast compression time settings. Analysis of quality judgment showed that slow, medium, and linear settings presented significantly higher preference scores than the fast compression setting. Conclusions: Individuals with ANSD showed no marked difference in perception of speech that was processed using the four different hearing aid settings. However, significantly higher preference, in terms of quality, was found for stimuli processed using slow, medium, and linear settings over the fast one. Therefore, whenever hearing aids are recommended for ANSD, those having slow compression time settings or linear amplification may be chosen over the fast (syllabic compression) one. In addition, WRSs obtained using hearing aid–processed stimuli were remarkably poorer than unprocessed stimuli. This shows that processing of speech through hearing aids might have caused a large reduction of performance in individuals with ANSD. However, further evaluation is needed using individually programmed hearing aids rather than hearing aid–processed stimuli.


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