scholarly journals Speech Perception in Quiet and in Noise Condition in Individuals with Auditory Neuropathy Spectrum Disorder

2017 ◽  
Vol 13 (1) ◽  
pp. 83-87 ◽  
Author(s):  
Kumari Apeksha ◽  
Ajith U Kumar
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.


2016 ◽  
Vol 27 (03) ◽  
pp. 204-218 ◽  
Author(s):  
Elizabeth Walker ◽  
Ryan McCreery ◽  
Meredith Spratford ◽  
Patricia Roush

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.


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