scholarly journals Central auditory maturation and behavioral outcomes after cochlear implantation in prelingual auditory neuropathy spectrum disorder related to OTOF variants (DFNB9): Lessons from pilot study

PLoS ONE ◽  
2021 ◽  
Vol 16 (6) ◽  
pp. e0252717
Author(s):  
Sang-Yeon Lee ◽  
Jin Hee Han ◽  
Hoo-Kang Song ◽  
Namju Justin Kim ◽  
Nayoung Yi ◽  
...  
Keyword(s):  
Language Development ◽  
Cochlear Implantation ◽  
Auditory Neuropathy ◽  
Auditory Evoked Potential ◽  
Spectrum Disorder ◽  
Successful Outcome ◽  
Auditory Neuropathy Spectrum Disorder ◽  
Auditory Maturation ◽  
Age Appropriate ◽  
Cortical Maturation

The cortical auditory evoked potential (CAEP)-based P1 component acts as a biomarker for cochlear implantation (CI) outcomes in children with auditory neuropathy spectrum disorder (ANSD). To date, early intervention primarily before the age of two years and six months of CI usage is necessary and sufficient to achieve age-appropriate cortical maturation and good prognosis. However, varying degrees of neural dyssynchrony, resulting from the etiological heterogeneity of ANSD, may preclude uniform application of this hypothesis to ensure auditory cortical maturation. Thus, a focused evaluation of those carrying OTOF variants, which may be the salient molecular etiology of prelingual ANSD, would circumvent the issue of heterogeneity. Here, we sought to provide a much better understanding of the brain perspectives (i.e., P1 maturation) in OTOF-associated ANSD subjects and set the stage for an optimal strategy to enhance language development. We conducted a preliminary study comprising 10 subjects diagnosed with OTOF-related ANSD who underwent CI by a single surgeon and subsequently underwent measurements of the P1 component. We observed that DFNB9 subjects who received CI after 2 years of age exhibited “absent” or “anomalous” P1 components that correspond to delayed language development. However, timely implantation, as early as 12 months of age per se, might be insufficient to achieve age-appropriate cortical maturation of DFNB9 in cases with six to seven months of device use. This suggests the importance of sustained rehabilitation in DFNB9 than in other etiologies. Indeed, an additional follow-up study showed that a reduction in P1 latency was linked to an improvement in auditory performance. Collectively, our results suggest that central auditory maturation and successful outcome of CI in DFNB9 may have more demanding requirements, that is, earlier implantation and more sustained rehabilitation. We believe that the current study opens a new path toward genome-based neuroimaging in the field of hearing research.

Auditory neuropathy spectrum disorder (ANSD) and cochlear implantation

2015 ◽  
Vol 79 (12) ◽  
pp. 1980-1987 ◽  
Author(s):  
Robert V. Harrison ◽  
Karen A. Gordon ◽  
Blake C. Papsin ◽  
Jaina Negandhi ◽  
Adrian L. James
Keyword(s):  
Cochlear Implantation ◽  
Auditory Neuropathy ◽  
Spectrum Disorder ◽  
Auditory Neuropathy Spectrum Disorder

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.

The audiovestibular profile of Brown-Vialetto-Van Laere syndrome

2021 ◽  
Vol 135 (11) ◽  
pp. 1000-1009
Author(s):  
R Omar ◽  
K Rajput ◽  
T Sirimanna ◽  
S Rajput ◽  
W Pagarkar
Keyword(s):  
Hearing Loss ◽  
Characteristic Feature ◽  
Motor Function ◽  
Cochlear Implantation ◽  
Cranial Nerve ◽  
Vestibular Function ◽  
Auditory Neuropathy ◽  
Spectrum Disorder ◽  
Significant Heterogeneity ◽  
Auditory Neuropathy Spectrum Disorder

AbstractBackgroundBrown-Vialetto-Van Laere syndrome, a rare disorder associated with motor, sensory and cranial nerve neuropathy, is caused by mutations in riboflavin transporter genes SLC52A2 and SLC52A3. Hearing loss is a characteristic feature of Brown-Vialetto-Van Laere syndrome and has been shown in recent studies to be characterised by auditory neuropathy spectrum disorder.MethodThis study reports the detailed audiovestibular profiles of four cases of Brown-Vialetto-Van Laere syndrome with SLC52A2 and SLC52A3 mutations. All of these patients had auditory neuropathy spectrum disorder.ResultsThere was significant heterogeneity in vestibular function and in the benefit gained from cochlear implantation. The audiological response to riboflavin therapy was also variable, in contrast to generalised improvement in motor function.ConclusionWe suggest that comprehensive testing of vestibular function should be conducted in Brown-Vialetto-Van Laere syndrome, in addition to serial behavioural audiometry as part of the systematic examination of the effects of riboflavin.

Cochlear Implantation versus Hearing Amplification in Patients with Auditory Neuropathy Spectrum Disorder

2013 ◽  
Vol 148 (5) ◽  
pp. 815-821 ◽  
Author(s):  
Stanley Pelosi ◽  
George Wanna ◽  
Cathrine Hayes ◽  
Linsey Sunderhaus ◽  
David S. Haynes ◽  
...  
Keyword(s):  
Cochlear Implantation ◽  
Auditory Neuropathy ◽  
Spectrum Disorder ◽  
Auditory Neuropathy Spectrum Disorder

scholarly journals Cochlear implantation in a child with auditory neuropathy spectrum disorder

2011 ◽  
Vol 6 (2) ◽  
pp. 29-37 ◽  
Author(s):  
Ji Fei ◽  
Chen Ai–ting ◽  
Hong Meng–di ◽  
Shi Wei ◽  
Li Jia–nan ◽  
...  
Keyword(s):  
Cochlear Implantation ◽  
Auditory Neuropathy ◽  
Spectrum Disorder ◽  
Auditory Neuropathy Spectrum Disorder
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