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Genetic variants in the peripheral auditory system significantly affect adult cochlear implant performance.

ABSTRACT: Cochlear implantation is an effective habilitation modality for adults with significant hearing loss. However, post-implant performance is variable. A portion of this variance in outcome can be attributed to clinical factors. Recent physiological studies suggest that the health of the spiral ganglion also impacts post-operative cochlear implant outcomes. The goal of this study was to determine whether genetic factors affecting spiral ganglion neurons may be associated with cochlear implant performance.Adults with post-lingual deafness who underwent cochlear implantation at the University of Iowa were studied. Pre-implantation evaluation included comprehensive genetic testing for genetic diagnosis. A novel score of genetic variants affecting genes with functional effects in the spiral ganglion was calculated. A Z-scored average of up to three post-operative speech perception tests (CNC, HINT, and AzBio) was used to assess outcome.Genetically determined spiral ganglion health affects cochlear implant outcomes, and when considered in conjunction with clinically determined etiology of deafness, accounts for 18.3% of the variance in postoperative speech recognition outcomes. Cochlear implant recipients with deleterious genetic variants that affect the cochlear sensory organ perform significantly better on tests of speech perception than recipients with deleterious genetic variants that affect the spiral ganglion.Etiological diagnosis of deafness including genetic testing is the single largest predictor of postoperative speech outcomes in adult cochlear implant recipients. A detailed understanding of the genetic underpinning of hearing loss will better inform pre-implant counseling. The method presented here should serve as a guide for further research into the molecular physiology of the peripheral auditory system and cochlear implants.

SUBMITTER: Shearer AE

PROVIDER: S-EPMC5527292 | biostudies-literature | 2017 May

REPOSITORIES: biostudies-literature

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Genetic variants in the peripheral auditory system significantly affect adult cochlear implant performance.

Shearer A Eliot AE Eppsteiner Robert W RW Frees Kathy K Tejani Viral V Sloan-Heggen Christina M CM Brown Carolyn C Abbas Paul P Dunn Camille C Hansen Marlan R MR Gantz Bruce J BJ Smith Richard J H RJH

Hearing research 20170215

<h4>Background</h4>Cochlear implantation is an effective habilitation modality for adults with significant hearing loss. However, post-implant performance is variable. A portion of this variance in outcome can be attributed to clinical factors. Recent physiological studies suggest that the health of the spiral ganglion also impacts post-operative cochlear implant outcomes. The goal of this study was to determine whether genetic factors affecting spiral ganglion neurons may be associated with coc ...[more]

PMID: 28213135

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Project description:ObjectiveThis paper reports a noninvasive method for quantifying neural synchrony in the cochlear nerve (i.e., peripheral neural synchrony) in cochlear implant (CI) users, which allows for evaluating this physiological phenomenon in human CI users for the first time in the literature. In addition, this study assessed how peripheral neural synchrony was correlated with temporal resolution acuity and speech perception outcomes measured in quiet and in noise in post-lingually deafened adult CI users. It tested the hypothesis that peripheral neural synchrony was an important factor for temporal resolution acuity and speech perception outcomes in noise in post-lingually deafened adult CI users.DesignStudy participants included 24 post-lingually deafened adult CI users with a Cochlear™ Nucleus® device. Three study participants were implanted bilaterally, and each ear was tested separately. For each of the 27 implanted ears tested in this study, 400 sweeps of the electrically evoked compound action potential (eCAP) were measured at four electrode locations across the electrode array. Peripheral neural synchrony was quantified at each electrode location using the phase locking value (PLV), which is a measure of trial-by-trial phase coherence among eCAP sweeps/trials. Temporal resolution acuity was evaluated by measuring the within-channel gap detection threshold (GDT) using a three-alternative, forced-choice procedure in a subgroup of 20 participants (23 implanted ears). For each ear tested in these participants, GDTs were measured at two electrode locations with a large difference in PLVs. For 26 implanted ears tested in 23 participants, speech perception performance was evaluated using Consonant-Nucleus-Consonant (CNC) word lists presented in quiet and in noise at signal-to-noise ratios (SNRs) of +10 and +5 dB. Linear Mixed effect Models were used to evaluate the effect of electrode location on the PLV and the effect of the PLV on GDT after controlling for the stimulation level effects. Pearson product-moment correlation tests were used to assess the correlations between PLVs, CNC word scores measured in different conditions, and the degree of noise effect on CNC word scores.ResultsThere was a significant effect of electrode location on the PLV after controlling for the effect of stimulation level. There was a significant effect of the PLV on GDT after controlling for the effects of stimulation level, where higher PLVs (greater synchrony) led to lower GDTs (better temporal resolution acuity). PLVs were not significantly correlated with CNC word scores measured in any listening condition or the effect of competing background noise presented at a SNR of +10 dB on CNC word scores. In contrast, there was a significant negative correlation between the PLV and the degree of noise effect on CNC word scores for a competing background noise presented at a SNR of +5 dB, where higher PLVs (greater synchrony) correlated with smaller noise effects on CNC word scores.ConclusionsThis newly developed method can be used to assess peripheral neural synchrony in CI users, a physiological phenomenon that has not been systematically evaluated in electrical hearing. Poorer peripheral neural synchrony leads to lower temporal resolution acuity and is correlated with a larger detrimental effect of competing background noise presented at a SNR of 5 dB on speech perception performance in post-lingually deafened adult CI users.

Project description:BACKGROUND:Cochlear implants (CIs) are neural prostheses that have been used routinely in the clinic over the past 25 years. They allow children who were born profoundly deaf, as well as adults affected by hearing loss for whom conventional hearing aids are insufficient, to attain a functional level of hearing. The "modern" CI (i.e., a multi-electrode implant using sequential coding strategies) has yielded good speech comprehension outcomes (recognition level for monosyllabic words about 50% to 60%, and sentence comprehension close to 90%). These good average results however hide a very important interindividual variability as scores in a given patients' population often vary from 5 to 95% in comparable testing conditions. Our aim was to develop a prognostic model for patients with unilateral CI. A novel method of objectively measuring electrical and neuronal interactions using electrical auditory brainstem responses (eABRs) is proposed. METHODS AND FINDINGS:The method consists of two measurements: 1) eABR measurements with stimulation by a single electrode at 70% of the dynamic range (four electrodes distributed within the cochlea were tested), followed by a summation of these four eABRs; 2) Measurement of a single eABR with stimulation from all four electrodes at 70% of the dynamic range. A comparison of the eABRs obtained by these two measurements, defined as the monaural interaction component (MIC), indicated electrical and neural interactions between the stimulation channels. Speech recognition performance without lip reading was measured for each patient using a logatome test (64 "vowel-consonant-vowel"; VCV; by forced choice of 1 out of 16). eABRs were measured in 16 CI patients (CIs with 20 electrodes, Digisonic SP; Oticon Medical ®, Vallauris, France). Significant correlations were found between speech recognition performance and the ratio of the amplitude of the V wave of the eABRs obtained with the two measurements (Pearson's linear regression model, parametric correlation: r2 = 0.26, p<0.05). CONCLUSIONS:This prognostic model allowed a substantial amount of the interindividual variance in speech recognition scores to be explained. The present study used measurements of electrical and neuronal interactions by eABR to assess patients' bio-electric capacity to use multiple information channels supplied by the implant. This type of prognostic information may be valuable in several ways. On the patient level, it allows customizing of individual treatments. ClinicalTrials.gov Identifier: NCT01805167.

Project description:In naturalistic situations, sounds are often perceived in conjunction with matching visual impressions. For example, we see and hear the neighbor's dog barking in the garden. Still, there is a good chance that we recognize the neighbor's dog even when we only hear it barking, but do not see it behind the fence. Previous studies with normal-hearing (NH) listeners have shown that the audio-visual presentation of a perceptual object (like an animal) increases the probability to recognize this object later on, even if the repeated presentation of this object occurs in a purely auditory condition. In patients with a cochlear implant (CI), however, the electrical hearing of sounds is impoverished, and the ability to recognize perceptual objects in auditory conditions is significantly limited. It is currently not well understood whether CI users - as NH listeners - show a multisensory facilitation for auditory recognition. The present study used event-related potentials (ERPs) and a continuous recognition paradigm with auditory and audio-visual stimuli to test the prediction that CI users show a benefit from audio-visual perception. Indeed, the congruent audio-visual context resulted in an improved recognition ability of objects in an auditory-only condition, both in the NH listeners and the CI users. The ERPs revealed a group-specific pattern of voltage topographies and correlations between these ERP maps and the auditory recognition ability, indicating a different processing of congruent audio-visual stimuli in CI users when compared to NH listeners. Taken together, our results point to distinct cortical processing of naturalistic audio-visual objects in CI users and NH listeners, which however allows both groups to improve the recognition ability of these objects in a purely auditory context. Our findings are of relevance for future clinical research since audio-visual perception might also improve the auditory rehabilitation after cochlear implantation.

Dataset Information

Genetic variants in the peripheral auditory system significantly affect adult cochlear implant performance.

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Genetic variants in the peripheral auditory system significantly affect adult cochlear implant performance.

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