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Revision cochlear implant (RCI) is a growing burden on cochlear implant programs. While reports on RCI rate are frequent, outcome measures are limited. The objectives of the current study were to: (1) evaluate RCI rate, (2) classify indications, (3) delineate the pre-RCI clinical course, and (4) measure surgical and speech perception outcomes, in a large cohort of patients implanted in a tertiary referral center between 1989–2018. Retrospective data review was performed and included patient demographics, medical records, and audiologic outcomes. Results indicated that RCI rate was 11.7% (172/1465), with a trend of increased RCI load over the years. The main indications for RCI were device-related failures (soft-45.4%, hard-23.8%), medical failure (14%), trauma (8.1%), and surgical failure (6.4%). Success rate was 98.8%. Children comprised 78% (134) of the cohort and were more likely than adults to undergo RCI. Most (70%) of the RCIs were performed within 10 years from primary implantation. Speech perception outcome analysis revealed unchanged or improved performance in 85% of the cases and declined performance in 15%. Current findings confirm that RCI is a safe with high clinical efficacy, however, the non-negligible percentage of patients that exhibited declined performance post-RCI should be considered in decision-making processes regarding RCI. Routine follow-up during their first years post-implantation is warranted.

Numerous studies have attempted to identify early physiological abnormalities in infants and toddlers who later develop autism spectrum disorder (ASD). One potential measure of early neurophysiology is the auditory brainstem response (ABR), which has been reported to exhibit prolonged latencies in children with ASD. We examined whether prolonged ABR latencies appear in infancy, before the onset of ASD symptoms, and irrespective of hearing thresholds. To determine how early in development these differences appear, we retrospectively examined clinical ABR recordings of infants who were later diagnosed with ASD. Of the 118 children in the participant pool, 48 were excluded due to elevated ABR thresholds, genetic aberrations, or old testing age, leaving a sample of 70 children: 30 of which were tested at 0-3 months, and 40 were tested at toddlerhood (1.5-3.5 years). In the infant group, the ABR wave-V was significantly prolonged in those who later developed ASD as compared with case-matched controls (n = 30). Classification of infants who later developed ASD and case-matched controls using this measure enabled accurate identification of ASD infants with 80% specificity and 70% sensitivity. In the group of toddlers with ASD, absolute and interpeak latencies were prolonged compared to clinical norms. Findings indicate that ABR latencies are significantly prolonged in infants who are later diagnosed with ASD irrespective of their hearing thresholds; suggesting that abnormal responses might be detected soon after birth. Further research is needed to determine if ABR might be a valid marker for ASD risk. Autism Res 2016, 9: 689-695. © 2015 The Authors Autism Research published by Wiley Periodicals, Inc. on behalf of International Society for Autism Research.

Highlights • Selective mutism (SM) is characterized by inability to speak in specific situations. • Auditory efferent function anomalies are evident in most children with SM. • Aberrant efferent activity during vocalization is implicated in SM maintenance. • Potential intervention and prevention strategies are discussed., Selective mutism (SM) is a relatively rare psychiatric disorder of childhood characterized by consistent inability to speak in specific social situations despite the ability to speak normally in others. SM typically involves severe impairments in social and academic functioning. Common complications include school failure, social difficulties in the peer group, and aggravated intra-familial relationships. Although SM has been described in the medical and psychological literatures for many years, the potential underlying neural basis of the disorder has only recently been explored. Here we explore the potential role of specific auditory neural mechanisms in the psychopathology of SM and discuss possible implications for treatment.

Background: While hearing aids for a contralateral routing of signals (CROS-HA) and bone conduction devices have been the traditional treatment for single-sided deafness (SSD) and asymmetric hearing loss (AHL), in recent years, cochlear implants (CIs) have increasingly become a viable treatment choice, particularly in countries where regulatory approval and reimbursement schemes are in place. Part of the reason for this shift is that the CI is the only device capable of restoring bilateral input to the auditory system and hence of possibly reinstating binaural hearing. Although several studies have independently shown that the CI is a safe and effective treatment for SSD and AHL, clinical outcome measures in those studies and across CI centers vary greatly. Only with a consistent use of defined and agreed-upon outcome measures across centers can high-level evidence be generated to assess the safety and efficacy of CIs and alternative treatments in recipients with SSD and AHL. Methods: This paper presents a comparative study design and minimum outcome measures for the assessment of current treatment options in patients with SSD/AHL. The protocol was developed, discussed, and eventually agreed upon by expert panels that convened at the 2015 APSCI conference in Beijing, China, and at the CI 2016 conference in Toronto, Canada. Results: A longitudinal study design comparing CROS-HA, BCD, and CI treatments is proposed. The recommended outcome measures include (1) speech in noise testing, using the same set of 3 spatial configurations to compare binaural benefits such as summation, squelch, and head shadow across devices; (2) localization testing, using stimuli that rove in both level and spectral content; (3) questionnaires to collect quality of life measures and the frequency of device use; and (4) questionnaires for assessing the impact of tinnitus before and after treatment, if applicable. Conclusion: A protocol for the assessment of treatment options and outcomes in recipients with SSD and AHL is presented. The proposed set of minimum outcome measures aims at harmonizing assessment methods across centers and thus at generating a growing body of high-level evidence for those treatment options.

With the growing number of older adults receiving cochlear implants (CI), there is general agreement that substantial benefits can be gained. Nonetheless, variability in speech perception performance is high, and the relative contribution and interactions among peripheral, central-auditory, and cognitive factors are not fully understood. The goal of the present study was to compare auditory-cognitive processing in older-adult CI recipients with that of older normal-hearing (NH) listeners by means of behavioral and electrophysiologic manifestations of a high-load cognitive task. Auditory event-related potentials (AERPs) were recorded from 9 older postlingually deafened adults with CI (age at CI >60) and 10 age-matched listeners with NH, while performing an auditory Stroop task. Participants were required to classify the speaker's gender (male/female) that produced the words ‘mother' or ‘father' while ignoring the irrelevant congruent or incongruent word meaning. Older CI and NH listeners exhibited comparable reaction time, performance accuracy, and initial sensory-perceptual processing (i.e. N1 potential). Nonetheless, older CI recipients showed substantially prolonged and less efficient perceptual processing (i.e. P3 potential). Congruency effects manifested in longer reaction time (i.e. Stroop effect), execution time, and P3 latency to incongruent versus congruent stimuli in both groups in a similar fashion; however, markedly prolonged P3 and shortened execution time were evident in older CI recipients. Collectively, older adults (CI and NH) employed a combined perceptual and postperceptual conflict processing strategy; nonetheless, the relative allotment of perceptual resources was substantially enhanced to maintain adequate performance in CI recipients. In sum, the recording of AERPs together with the simultaneously obtained behavioral measures during a Stroop task exposed a differential time course of auditory-cognitive processing in older CI recipients that was not manifested in the behavioral end products of processing. These data may have implications regarding clinical evaluation and rehabilitation procedures that should be tailored specifically for this unique group of patients.

This study evaluated the cognitive profiles of children with idiopathic generalized epilepsy (IGE), uniformly treated with valproic acid with well-controlled seizures. Twenty-four were neuropsychologically evaluated. They comprised: 14 females, 10 males: 12 with generalized tonic-clonic seizures (GTCS), mean age 14y 4mo, SD ly 7mo, range 12y to 16y 4 mo; 12 with absence seizures (AS]) mean age 14y 5mo, SD ly 10mo, range 11y to 16y 4mo, with intellectual abilities within the normal range and age-appropriate scholastic skills, and 20 healthy controls (12 females, 8 males; mean age 14y 5mo, SD 1y 10mo, range 10y 7mo to 16y 7mo). As a group, children with IGE performed significantly poorer in all tests (non-verbal and verbal attention, verbal learning and memory, word fluency, and controlled sequential fine motor responses) excluding non-verbal memory. Analysis according to type of seizure revealed that both patient groups (AS and GTCS) had an attention deficit, whereas only children with AS showed deficits in verbal learning and memory, word fluency, and controlled fine motor responses. These results suggest a long-term risk of learning impairment for children with IGE, even if they have normal intelligence and their seizures are well controlled.

This study was designed to characterize the effect of background noise on the identification of syllables using behavioral and electrophysiological measures. Twenty normal-hearing adults (18-30 years) performed an identification task in a two-alternative forced-choice paradigm. Stimuli consisted of naturally produced syllables [da] and [ga] embedded in white noise. The noise was initiated 1000 ms before the onset of the speech stimuli in order to separate the auditory event related potentials (AERP) response to noise onset from that to the speech. Syllables were presented in quiet and in five SNRs: +15, +3, 0, -3, and -6 dB. Results show that (1) performance accuracy, d', and reaction time were affected by the noise, more so for reaction time; (2) both N1 and P3 latency were prolonged as noise levels increased, more so for P3; (3) [ga] was better identified than [da], in all noise conditions; and (4) P3 latency was longer for [da] than for [ga] for SNR 0 through -6 dB, while N1 latency was longer for [ga] than for [da] in most listening conditions. In conclusion, the unique stimuli structure utilized in this study demonstrated the effects of noise on speech recognition at both the physical and the perceptual processing levels.

Performing actions with auditory consequences modulates the response in auditory cortex to otherwise identical stimuli passively heard. Such modulation has been suggested to occur through a corollary discharge sent from the motor cortex during voluntary actions. However, the relationship between the effector used to generate the sound, type of modulation and changes in perceptual sensitivity are unclear. Here we use functional magnetic resonance imaging on healthy subjects and demonstrate bilateral enhancement in the auditory cortex to self-generated versus externally generated sounds. Furthermore, we find that this enhancement is stronger when the sound-producing hand is contralateral to the auditory cortex. At the behavioural level, binaural hearing thresholds are lower for self-generated sounds and monaural thresholds are lower for sounds triggered by the hand ipsilateral to the stimulated ear. Together with functional connectivity analysis, our results suggest that a corollary discharge sent from active motor cortex enhances activity in the auditory cortex and increases perceptual sensitivity in a lateralized manner.

The aim of the present study was to investigate the effect of ear asymmetry, order of testing, and gender on transient-evoked otoacoustic emission (TEOAE) pass rates and response levels in newborn hearing screening. The screening results of 879 newborns, of whom 387 (study group) passed screening successfully in only one ear in the first TEOAE screening, but passed screening successfully in both ears thereafter, and 492 (control group) who passed screening successfully in both ears in the first TEOAE, were retrospectively examined for pass rates and TEOAE characteristics. Results indicated a right-ear advantage, as manifested by significantly higher pass rates in the right ear (61% and 39% for right and left ears, respectively) in the study group, and in 1.75 dB greater TEOAE response amplitudes in the control group. The right-ear advantage was enhanced when the first tested ear was the right ear (76%). When the left ear was tested first, pass rates were comparable in both ears. The right-ear advantage in pass rates was similar in females versus males, but manifested in 1.5 dB higher response amplitudes in females compared with males, regardless of the tested ear and order of testing in both study and control groups. The study provides further evidence for the functional lateralization of the auditory system at the cochlear level already apparent soon after birth in both males and females. While order of testing plays a significant role in the asymmetry in pass rates, the innate right-ear advantage seems to be a more dominant contributor.

The aim of this study was to characterize the auditory brainstem responses (ABRs) of young children with suspected autism spectrum disorders (ASDs) and compare them with the ABRs of children with language delay and with clinical norms. The ABRs of 26 children with suspected ASDs (21 males, five females; mean age 32.5 mo) and an age- and sex-matched group of 26 children with language delay (22 males, four females) were analysed. All children had normal hearing. The absolute latencies of waves I, III, and V, and interpeak latencies (IPLs) I to III, I to V, and III to V of the group with ASDs and the group with language delay were compared. Data from both groups were further compared with clinical norms. All absolute latencies and IPLs were significantly prolonged in the group with suspected ASDs compared with the group with language delay, excluding IPL III-V (all p-values0.05) and with clinical norms (all p-values0.001; IPL III-V, p0.05). Significant prolongation of absolute and IPLs was also evident in the group with language delay compared with clinical norms, excluding IPL III to V (all p-values0.001). The prevalence of abnormal findings in two or more absolute latencies was found to be significantly higher in the group with ASDs (50%) than in the group with language delay (8%; p=0.002). The results provide first-time evidence for a neurodevelopmental brainstem abnormality that is already apparent in young children with suspected ASD and language delay. The overlap in ABR findings supports the assertion that an auditory processing deficit may be at the core of these two disorders.

Performing actions with sensory consequences modifies physiological and behavioral responses relative to otherwise identical sensory input perceived in a passive manner. It is assumed that such modifications occur through an efference copy sent from motor cortex to sensory regions during performance of voluntary actions. In the auditory domain most behavioral studies report attenuated perceived loudness of self-generated auditory action-consequences. However, several recent behavioral and physiological studies report enhanced responses to such consequences. Here we manipulated the intensity of self-generated and externally-generated sounds and examined the type of perceptual modification (enhancement vs. attenuation) reported by healthy human subjects. We found that when the intensity of self-generated sounds was low, perceived loudness is enhanced. Conversely, when the intensity of self-generated sounds was high, perceived loudness is attenuated. These results might reconcile some of the apparent discrepancies in the reported literature and suggest that efference copies can adapt perception according to the differential sensory context of voluntary actions.