scholarly journals Children with Auditory Neuropathy Spectrum Disorder Fitted with Hearing Aids Applying the American Academy of Audiology Pediatric Amplification Guideline: Current Practice and Outcomes

2016 ◽  
Vol 27 (03) ◽  
pp. 204-218 ◽  
Author(s):  
Elizabeth Walker ◽  
Ryan McCreery ◽  
Meredith Spratford ◽  
Patricia Roush
Keyword(s):  
Hearing Loss ◽  
Speech Perception ◽  
American Academy ◽  
Hearing Aids ◽  
Maternal Education ◽  
Longitudinal Design ◽  
Auditory Neuropathy ◽  
Spectrum Disorder ◽  
Auditory Neuropathy Spectrum Disorder ◽  
The University

Background: Up to 15% of children with permanent hearing loss (HL) have auditory neuropathy spectrum disorder (ANSD), which involves normal outer hair cell function and disordered afferent neural activity in the auditory nerve or brainstem. Given the varying presentations of ANSD in children, there is a need for more evidence-based research on appropriate clinical interventions for this population. Purpose: This study compared the speech production, speech perception, and language outcomes of children with ANSD, who are hard of hearing, to children with similar degrees of mild-to-moderately severe sensorineural hearing loss (SNHL), all of whom were fitted with bilateral hearing aids (HAs) based on the American Academy of Audiology pediatric amplification guidelines. Research Design: Speech perception and communication outcomes data were gathered in a prospective accelerated longitudinal design, with entry into the study between six mo and seven yr of age. Three sites were involved in participant recruitment: Boys Town National Research Hospital, the University of North Carolina at Chapel Hill, and the University of Iowa. Study Sample: The sample consisted of 12 children with ANSD and 22 children with SNHL. The groups were matched based on better-ear pure-tone average, better-ear aided speech intelligibility index, gender, maternal education level, and newborn hearing screening result (i.e., pass or refer). Data Collection and Analysis: Children and their families participated in an initial baseline visit, followed by visits twice a year for children <2 yr of age and once a yr for children >2 yr of age. Paired-sample t-tests were used to compare children with ANSD to children with SNHL. Results: Paired t-tests indicated no significant differences between the ANSD and SNHL groups on language and articulation measures. Children with ANSD displayed functional speech perception skills in quiet. Although the number of participants was too small to conduct statistical analyses for speech perception testing, there appeared to be a trend in which the ANSD group performed more poorly in background noise with HAs, compared to the SNHL group. Conclusions: The American Academy of Audiology Pediatric Amplification Guidelines recommend that children with ANSD receive an HA trial if their behavioral thresholds are sufficiently high enough to impede speech perception at conversational levels. For children with ANSD in the mild-to-severe HL range, the current results support this recommendation, as children with ANSD can achieve functional outcomes similar to peers with SNHL.

scholarly journals Plasticity in the Developing Auditory Cortex: Evidence from Children with Sensorineural Hearing Loss and Auditory Neuropathy Spectrum Disorder

2012 ◽  
Vol 23 (06) ◽  
pp. 396-411 ◽  
Author(s):  
Garrett Cardon ◽  
Julia Campbell ◽  
Anu Sharma
Keyword(s):  
Hearing Loss ◽  
Auditory Cortex ◽  
Sensorineural Hearing Loss ◽  
Hearing Aids ◽  
Auditory Neuropathy ◽  
Sensorineural Hearing ◽  
Spectrum Disorder ◽  
Early Deprivation ◽  
Auditory Neuropathy Spectrum Disorder ◽  
Cortical Input

The developing auditory cortex is highly plastic. As such, the cortex is both primed to mature normally and at risk for reorganizing abnormally, depending upon numerous factors that determine central maturation. From a clinical perspective, at least two major components of development can be manipulated: (1) input to the cortex and (2) the timing of cortical input. Children with sensorineural hearing loss (SNHL) and auditory neuropathy spectrum disorder (ANSD) have provided a model of early deprivation of sensory input to the cortex and demonstrated the resulting plasticity and development that can occur upon introduction of stimulation. In this article, we review several fundamental principles of cortical development and plasticity and discuss the clinical applications in children with SNHL and ANSD who receive intervention with hearing aids and/or cochlear implants.

Auditory testing outcomes with hearing aids in patients with auditory neuropathy spectrum disorder

2021 ◽  
Vol 42 (5) ◽  
pp. 103057
Author(s):  
Firas Sbeih ◽  
Donald M. Goldberg ◽  
Sara Liu ◽  
Maxwell Y. Lee ◽  
Gina Stillitano ◽  
...  
Keyword(s):  
Hearing Aids ◽  
Auditory Neuropathy ◽  
Spectrum Disorder ◽  
Auditory Neuropathy Spectrum Disorder

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
Keyword(s):  
Gender Differences ◽  
Retrospective Study ◽  
Hearing Aids ◽  
Otoacoustic Emissions ◽  
Hearing Aid ◽  
Auditory Neuropathy ◽  
Spectrum Disorder ◽  
Auditory Neuropathy Spectrum Disorder ◽  
Acoustic Reflexes ◽  
Hearing Aid Benefit

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.

scholarly journals Aiding and Occluding the Contralateral Ear in Implanted Children with Auditory Neuropathy Spectrum Disorder

2011 ◽  
Vol 22 (09) ◽  
pp. 567-577 ◽  
Author(s):  
Christina L. Runge ◽  
Jamie Jensen ◽  
David R. Friedland ◽  
Ruth Y. Litovsky ◽  
Sergey Tarima
Keyword(s):  
Speech Perception ◽  
Cochlear Implant ◽  
Cochlear Implantation ◽  
Hearing Aid ◽  
Auditory Neuropathy ◽  
Spectrum Disorder ◽  
Auditory Neuropathy Spectrum Disorder ◽  
Predictive Variables ◽  
Hearing Aid Use ◽  
Contralateral Ear

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.

Perception of Hearing Aid–Processed Speech in Individuals with Late-Onset Auditory Neuropathy Spectrum Disorder

2015 ◽  
Vol 26 (10) ◽  
pp. 815-823 ◽  
Author(s):  
Jijo Pottackal Mathai ◽  
Sabarish Appu
Keyword(s):  
Speech Perception ◽  
Word Recognition ◽  
Hearing Aids ◽  
Repeated Measures ◽  
Late Onset ◽  
Hearing Aid ◽  
Auditory Neuropathy ◽  
Spectrum Disorder ◽  
Temporal Envelope ◽  
Compression Time

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.

scholarly journals Cortical Neurophysiologic Correlates of Auditory Threshold in Adults and Children With Normal Hearing and Auditory Neuropathy Spectrum Disorder

2020 ◽  
pp. 1-15
Author(s):  
Garrett Cardon ◽  
Anu Sharma
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Normal Hearing ◽  
Auditory Neuropathy ◽  
Auditory Threshold ◽  
Sensorineural Hearing ◽  
Spectrum Disorder ◽  
Threshold Estimation ◽  
Auditory Thresholds ◽  
Auditory Neuropathy Spectrum Disorder

Purpose Auditory threshold estimation using the auditory brainstem response or auditory steady state response is limited in some populations (e.g., individuals with auditory neuropathy spectrum disorder [ANSD] or those who have difficulty remaining still during testing and cannot tolerate general anesthetic). However, cortical auditory evoked potentials (CAEPs) can be recorded in many such patients and have been employed in threshold approximation. Thus, we studied CAEP estimates of auditory thresholds in participants with normal hearing, sensorineural hearing loss, and ANSD. Method We recorded CAEPs at varying intensity levels to speech (i.e., /ba/) and tones (i.e., 1 kHz) to estimate auditory thresholds in normal-hearing adults ( n = 10) and children ( n = 10) and case studies of children with sensorineural hearing loss and ANSD. Results Results showed a pattern of CAEP amplitude decrease and latency increase as stimulus intensities declined until waveform components disappeared near auditory threshold levels. Overall, CAEP thresholds were within 10 dB HL of behavioral thresholds for both stimuli. Conclusions The above findings suggest that CAEPs may be clinically useful in estimating auditory threshold in populations for whom such a method does not currently exist. Physiologic threshold estimation in difficult-to-test clinical populations could lead to earlier intervention and improved outcomes.

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