Dataset Information

In Vivo Electrocochleography in Hybrid Cochlear Implant Users Implicates TMPRSS3 in Spiral Ganglion Function.

ABSTRACT: Cochlear implantation, a surgical method to bypass cochlear hair cells and directly stimulate the spiral ganglion, is the standard treatment for severe-to-profound hearing loss. Changes in cochlear implant electrode array design and surgical approach now allow for preservation of acoustic hearing in the implanted ear. Electrocochleography (ECochG) was performed in eight hearing preservation subjects to assess hair cell and neural function and elucidate underlying genetic hearing loss. Three subjects had pathogenic variants in TMPRSS3 and five had pathogenic variants in genes known to affect the cochlear sensory partition. The mechanism by which variants in TMPRSS3 cause genetic hearing loss is unknown. We used a 500-Hz tone burst to record ECochG responses from an intracochlear electrode. Responses consist of a cochlear microphonic (hair cell) and an auditory nerve neurophonic. Cochlear microphonics did not differ between groups. Auditory nerve neurophonics were smaller, on average, in subjects with TMPRSS3 deafness. Results of this proof-of-concept study provide evidence that pathogenic variants in TMPRSS3 may impact function of the spiral ganglion. While ECochG as a clinical and research tool has been around for decades, this study illustrates a new application of ECochG in the study of genetic hearing and deafness in vivo.

SUBMITTER: Shearer AE

PROVIDER: S-EPMC6154996 | biostudies-literature | 2018 Sep

REPOSITORIES: biostudies-literature

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In Vivo Electrocochleography in Hybrid Cochlear Implant Users Implicates TMPRSS3 in Spiral Ganglion Function.

Shearer A Eliot AE Tejani Viral D VD Brown Carolyn J CJ Abbas Paul J PJ Hansen Marlan R MR Gantz Bruce J BJ Smith Richard J H RJH

Scientific reports 20180921 1

Cochlear implantation, a surgical method to bypass cochlear hair cells and directly stimulate the spiral ganglion, is the standard treatment for severe-to-profound hearing loss. Changes in cochlear implant electrode array design and surgical approach now allow for preservation of acoustic hearing in the implanted ear. Electrocochleography (ECochG) was performed in eight hearing preservation subjects to assess hair cell and neural function and elucidate underlying genetic hearing loss. Three subj ...[more]

PMID: 30242206

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Project description:ImportanceCochlear implantation produces remarkable results in postlingual deafness, although auditory outcomes vary. Electrocochleography (ECochG) has emerged as a valuable tool for assessing the cochlear-neural substrate and evaluating patient prognosis.ObjectiveTo assess whether ECochG-total response (ECochG-TR) recorded at the best-frequency electrode (BF-ECochG-TR) correlates more strongly with speech perception performance than ECochG-TR measured at the round window (RW-ECochG-TR).Design, setting, and participantsThis single-center cross-sectional study recruited 142 patients from July 1, 2021, to April 30, 2022, with 1-year follow-up. Exclusions included perilymph suctioning, crimped sound delivery tubes, non-native English speakers, inner ear malformations, nonpatent external auditory canals, or cochlear implantation revision surgery.ExposuresCochlear implantation.Main outcomes and measuresSpeech perception testing, including the consonant-nucleus-consonant (CNC) words test, AzBio sentences in quiet, and AzBio sentences in noise plus 10-dB signal to noise ratio (with low scores indicating poor performance and high scores indicating excellent performance on all tests), at 6 months postoperatively; and RW-ECochG-TR and BF-ECochG-TR (measured for 250, 500, 1000, and 2000 Hz).ResultsA total of 109 of the 142 eligible postlingual adults (mean [SD] age, 68.7 [15.8] years; 67 [61.5%] male) were included in the study. Both BF-ECochG-TR and RW-ECochG-TR were correlated with 6-month CNC scores (BF-ECochG-TR: r = 0.74; 95% CI, 0.62-0.82; RW-ECochG-TR: r = 0.67; 95% CI, 0.54-0.76). A multivariate model incorporating age, duration of hearing loss, and angular insertion depth did not outperform BF-ECochG-TR or RW-ECochG-TR alone. The BF-ECochG-TR correlation with CNC scores was significantly stronger than the RW-ECochG-TR correlation (r difference = -0.18; 95% CI, -0.31 to -0.01; z = -2.02). More moderate correlations existed between 6-month AzBio scores in noise, Montreal Cognitive Assessment (MoCA) scores (r = 0.46; 95% CI, 0.29-0.60), and BF-ECochG-TR (r = 0.42; 95% CI, 0.22-0.58). MoCA and the interaction between BF-ECochG-TR and MoCA accounted for a substantial proportion of variability in AzBio scores in noise at 6 months (R2 = 0.50; 95% CI, 0.36-0.61).Conclusions and relevanceIn this case series, BF-ECochG-TR was identified as having a stronger correlation with cochlear implantation performance than RW-ECochG-TR, although both measures highlight the critical role of the cochlear-neural substrate on outcomes. Demographic, audiologic, and surgical factors demonstrated weak correlations with cochlear implantation performance, and performance in noise was found to require a robust cochlear-neural substrate (BF-ECochG-TR) as well as sufficient cognitive capacity (MoCA). Future cochlear implantation studies should consider these variables when assessing performance and related interventions.

Project description:The growing group of cochlear implant (CI) users includes subjects with preserved acoustic hearing on the opposite side to the CI. The use of both listening sides results in improved speech perception in comparison to listening with one side alone. However, large variability in the measured benefit is observed. It is possible that this variability is associated with the integration of speech across electric and acoustic stimulation modalities. However, there is a lack of established methods to assess speech integration between electric and acoustic stimulation and consequently to adequately program the devices. Moreover, existing methods do not provide information about the underlying physiological mechanisms of this integration or are based on simple stimuli that are difficult to relate to speech integration. Electroencephalography (EEG) to continuous speech is promising as an objective measure of speech perception, however, its application in CIs is challenging because it is influenced by the electrical artifact introduced by these devices. For this reason, the main goal of this work is to investigate a possible electrophysiological measure of speech integration between electric and acoustic stimulation in bimodal CI users. For this purpose, a selective attention decoding paradigm has been designed and validated in bimodal CI users. The current study included behavioral and electrophysiological measures. The behavioral measure consisted of a speech understanding test, where subjects repeated words to a target speaker in the presence of a competing voice listening with the CI side (CIS) only, with the acoustic side (AS) only or with both listening sides (CIS+AS). Electrophysiological measures included cortical auditory evoked potentials (CAEPs) and selective attention decoding through EEG. CAEPs were recorded to broadband stimuli to confirm the feasibility to record cortical responses with CIS only, AS only, and CIS+AS listening modes. In the selective attention decoding paradigm a co-located target and a competing speech stream were presented to the subjects using the three listening modes (CIS only, AS only, and CIS+AS). The main hypothesis of the current study is that selective attention can be decoded in CI users despite the presence of CI electrical artifact. If selective attention decoding improves combining electric and acoustic stimulation with respect to electric stimulation alone, the hypothesis can be confirmed. No significant difference in behavioral speech understanding performance when listening with CIS+AS and AS only was found, mainly due to the ceiling effect observed with these two listening modes. The main finding of the current study is the possibility to decode selective attention in CI users even if continuous artifact is present. Moreover, an amplitude reduction of the forward transfer response function (TRF) of selective attention decoding was observed when listening with CIS+AS compared to AS only. Further studies to validate selective attention decoding as an electrophysiological measure of electric acoustic speech integration are required.

Dataset Information

In Vivo Electrocochleography in Hybrid Cochlear Implant Users Implicates TMPRSS3 in Spiral Ganglion Function.

Publications

In Vivo Electrocochleography in Hybrid Cochlear Implant Users Implicates TMPRSS3 in Spiral Ganglion Function.

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