Structural connectivity changes in unilateral hearing loss.

Network connectivity, as mapped by the whole brain connectome, plays a crucial role in regulating auditory function. Auditory deprivation such as unilateral hearing loss might alter structural network connectivity; however, these potential alterations are poorly understood. Thirty-seven acoustic neuroma patients with unilateral hearing loss (19 left-sided and 18 right-sided) and 19 healthy controls underwent diffusion-weighted and T1-weighted imaging to assess edge strength, node strength, and global efficiency of the structural connectome. Edge strength was estimated by pair-wise normalized streamline density from tractography and connectomics. Node strength and global efficiency were calculated through graph theory analysis of the connectome. Pure-tone audiometry and word recognition scores were used to correlate the degree and duration of unilateral hearing loss with node strength and global efficiency. We demonstrate significantly stronger edge strength and node strength through the visual network, weaker edge strength and node strength in the somatomotor network, and stronger global efficiency in the unilateral hearing loss patients. No discernible correlations were observed between the degree and duration of unilateral hearing loss and the measures of node strength or global efficiency. These findings contribute to our understanding of the role of structural connectivity in hearing by facilitating visual network upregulation and somatomotor network downregulation after unilateral hearing loss.

Effects of the BalanCI on Working Memory and Balance in Children and Young Adults With Cochleovestibular Dysfunction.

OBJECTIVES: This study aimed to: (1) determine the interaction between cognitive load and balance in children and young adults with bilateral cochleovestibular dysfunction who use bilateral cochlear implants (CIs) and (2) determine the effect of an auditory balance prosthesis (the BalanCI) on this interaction. Many (20 to 70%) children with sensorineural hearing loss experience some degree of vestibular loss, leading to poorer balance. Poor balance could have effects on cognitive resource allocation which might be alleviated by the BalanCI as it translates head-referenced cues into electrical pulses delivered through the CI. It is hypothesized that children and young adults with cochleovestibular dysfunction will demonstrate greater dual-task costs than typically-developing children during dual balance-cognition tasks, and that BalanCI use will improve performance on these tasks. DESIGN: Study participants were 15 typically-developing children (control group: mean age ± SD = 13.6 ± 2.75 years, 6 females) and 10 children and young adults who use bilateral CIs and have vestibular dysfunction (CI-V group: mean age ± SD=20.6 ± 5.36 years, 7 females). Participants completed two working memory tasks (backward auditory verbal digit span task and backward visuospatial dot matrix task) during three balance conditions: seated, standing in tandem stance with the BalanCI off, and standing in tandem stance with the BalanCI on. Working memory performance was quantified as total number of correct trials achieved. Postural stability was quantified as translational and rotational path length of motion capture markers worn on the head, upper body, pelvis, and feet, normalized by trial time. RESULTS: Relative to the control group, children and young adults in the CI-V group exhibited poorer overall working memory across all balance conditions ( p = 0.03), poorer translational postural stability (larger translational path length) during both verbal and visuospatial working memory tasks ( p < 0.001), and poorer rotational stability (larger rotational path length) during the verbal working memory task ( p = 0.026). The CI-V group also exhibited poorer translational ( p = 0.004) and rotational ( p < 0.001) postural stability during the backward verbal digit span task than backward visuospatial dot matrix task; BalanCI use reduced this stability difference between verbal and visuospatial working memory tasks for translational stability overall ( p > 0.9), as well as for rotational stability during the maximum working memory span (highest load) participants achieved in each task ( p = 0.91). CONCLUSIONS: Balance and working memory were impaired in the CI-V group compared with the control group. The BalanCI offered subtle improvements in stability in the CI-V group during a backward verbal working memory task, without producing a negative effect on working memory outcomes. This study supports the feasibility of the BalanCI as a balance prosthesis for individuals with cochleovestibular impairments.

Importance of early objective auditory testing in the presentation of sudden sensorineural hearing loss in children.

BACKGROUND: Functional hearing loss can be due to an auditory manifestation of functional neurological disorder, previously known as conversion disorder. METHODS: This is a case series of 3 pediatric patients with a diagnosis of idiopathic SSNHL who ultimately were found to have functional neurological disorder. RESULTS: Average age was 12.7 years at presentation (range 10-14 years). All three patients underwent invasive interventions prior to their initial clinic visit. All patients demonstrated profound SNHL on behavioural audiogram, but normal otoacoustic emissions (OAE) and auditory brainstem response testing. With counselling, both patients demonstrated significant hearing improvement. CONCLUSIONS: Early use of OAE's in the workup of SSNHL can avoid unnecessary and potentially harmful therapies and expedite access to counselling services which may help lead to symptom resolution.

Longitudinal Effects of Simultaneous and Sequential Bilateral Cochlear Implantation on Cortical Auditory-Evoked Potentials Recorded at Cz in a Large Cohort of Children.

OBJECTIVES: Auditory development after bilateral cochlear implantation in children has been measured using source localization of multi-channel late latency responses. It is not clear, however, whether this development can be tracked using a more clinically feasible method of recording from one active recording electrode placed at mid-line center of the head (Cz). DESIGN: In this prospective cohort study, cortical auditory-evoked potential responses (CAEPs) were recorded from Cz referenced to each earlobe (Cz-CAEP) from 222 children with bilateral cochlear implant (CI); 128 (mean ± SD age: 2.78 ± 3.30 years) received both CIs in the same surgery (simultaneous group) and 94 (aged 7.72 ± 4.45 years) received a second CI after 4.21 ± 2.98 years of unilateral CI use. We sought to (1) identify cortical development over the first couple of years of bilateral CI use; (2) measure known asymmetries in auditory development between the CIs; and (3) detect the effects of bilateral rather than unilateral CI use. 4556 Cz-CAEPs were recorded across the cohort over 33.50 ± 7.67 months duration of bilateral CI use. Given concerns related to peak picking, amplitude areas were measured across two response time windows (50 to 199 ms and 200 to 400 ms). RESULTS: Results indicated that small response amplitudes occur at initial CI use and amplitudes increase in the negative or positive direction rapidly over the first months of CI use in both time windows. Asymmetries between Cz-CAEPs evoked by each CI were found in the sequential group and reduced with bilateral CI use, particularly in the first time window; these differences increased with longer inter-implant delay. Bilaterally evoked Cz-CAEPs were larger in amplitude than unilateral responses from either CI in the simultaneous group. In the sequential group, bilateral responses were similar to responses from the first implanted side but increased in relative amplitude with bilateral CI use. The Cz-CAEP measures were not able to predict asymmetries or bilateral benefits in speech perception measures. CONCLUSIONS: The Cz-CAEP was able to indicate cortical detection of CI input and showed gross morphological changes with bilateral CI use. Findings indicate Cz-CAEPs can be used to identify gross changes in auditory development in children with bilateral CIs, but they are less sensitive to tracking the remaining abnormalities that are measured by multi-channel CAEPs and speech perception testing.

Cortical imbalance following delayed restoration of bilateral hearing in deaf adolescents.

Unilateral auditory deprivation in early childhood can lead to cortical strengthening of inputs from the stimulated side, yet the impact of this on bilateral processing when inputs are later restored beyond an early sensitive period is unknown. To address this, we conducted a longitudinal study with 13 bilaterally profoundly deaf adolescents who received unilateral access to sound via a cochlear implant (CI) in their right ear in early childhood before receiving bilateral access to sound a decade later via a second CI in their left ear. Auditory-evoked cortical responses to unilateral and bilateral stimulation were measured repeatedly using electroencephalogram from 1 week to 14 months after activation of their second CI. Early cortical responses from the newly implanted ear and bilateral stimulation were atypically lateralized to the left ipsilateral auditory cortex. Duration of unilateral deafness predicted an unexpectedly stronger representation of inputs from the newly implanted, compared to the first implanted ear, in left auditory cortex. Significant initial reductions in responses were observed, yet a left-hemisphere bias and unequal weighting of inputs favoring the long-term deaf ear did not converge to a balanced state observed in the binaurally developed system. Bilateral response enhancement was significantly reduced in left auditory cortex suggesting deficits in ipsilateral response inhibition of new, dominant, inputs during bilateral processing. These findings paradoxically demonstrate the adaptive capacity of the adolescent auditory system beyond an early sensitive period for bilateral input, as well as restrictions on its potential to fully reverse cortical imbalances driven by long-term unilateral deafness.

Vestibular Perceptual Thresholds in Older Adults With and Without Age-related Hearing Loss.

OBJECTIVES: Older adults with age-related hearing loss (ARHL) are at greater risk of falling and have greater mobility problems than older adults with normal hearing (NH). The underlying cause of these associations remains unclear. One possible reason is that age-related declines in the vestibular system could parallel those observed in the auditory system within the same individuals. Here, we compare the sensitivity of vestibular perceptual abilities (psychophysics), vestibular end-organ functioning (vestibular evoked myogenic potentials and video head impulse tests), and standing balance (posturography) in healthy older adults with and without ARHL. DESIGN: A total of 46 community-dwelling older adults, 23 with ARHL and 23 with NH, were passively translated in heave (up and down) and rotated in pitch (tilted forward and backward) in the dark using a motion platform. Using an adaptive staircase psychophysical procedure, participants' heave and pitch detection and discrimination thresholds were determined. In a posturography task, participants' center of pressure (COP) path length was measured as they stood on a forceplate with eyes open and closed, on firm and compliant surfaces, with and without sound suppression. Baseline motor, cognitive, and sensory functioning, including vestibular end-organ function, were measured. RESULTS: Individuals with ARHL were less sensitive at discriminating pitch movements compared to older adults with NH. Poorer self-reported hearing abilities were also associated with poorer pitch discrimination. In addition to pitch discrimination thresholds, lower pitch detection thresholds were significantly associated with hearing loss in the low-frequency range. Less stable standing balance was significantly associated with poorer vestibular perceptual sensitivity. DISCUSSION: These findings provide evidence for an association between ARHL and reduced vestibular perceptual sensitivity.

Interhemispheric auditory connectivity requires normal access to sound in both ears during development.

Despite early bilateral cochlear implantation, children with congenital deafness do not develop accurate spatial hearing; we thus asked whether auditory brain networks are disrupted in these children. EEG responses were evoked unilaterally and bilaterally in 13 children with normal hearing and 16 children receiving bilateral cochlear implants simultaneously. Active cortical areas were estimated by the Time Restricted Artifact and Coherent source Suppression (TRACS) beamformer and connected cortical areas were identified by measuring coherence between source responses. A whole-brain analysis of theta band coherence revealed the strongest connections between the temporal areas in all conditions at early latencies. Stronger imaginary coherence in activity between the two auditory cortices to bilateral than unilateral input was found in children with normal hearing reflecting facilitation in the auditory network during bilateral hearing. The opposite effect, depressed coherence, was found during bilateral stimulation in children using cochlear implants. Children with cochlear implants also showed a unique auditory network in response to bilateral stimulation which was marked by increased connectivity between occipital and frontal areas. These findings suggest that cortical networks for sound processing are normally facilitated by bilateral input but are disrupted in children who hear through two independent cochlear implants. Efforts to improve hearing in children with congenital deafness must thus include corrections to potential mismatches in bilateral input to support brain development.

BalanCI: Head-Referenced Cochlear Implant Stimulation Improves Balance in Children with Bilateral Cochleovestibular Loss.

INTRODUCTION: To determine the impact of a head-referenced cochlear implant (CI) stimulation system, BalanCI, on balance and postural control in children with bilateral cochleovestibular loss (BCVL) who use bilateral CI. METHODS: Prospective, blinded case-control study. Balance and postural control testing occurred in two settings: (1) quiet clinical setting and (2) immersive realistic virtual environment (Challenging Environment Assessment Laboratory [CEAL], Toronto Rehabilitation Institute). Postural control was assessed in 16 and balance in 10 children with BCVL who use bilateral CI, along with 10 typically developing children. Children with neuromotor, cognitive, or visual deficits that would prevent them from performing the tests were excluded. Children wore the BalanCI, which is a head-mounted device that couples with their CIs through the audio port and provides head-referenced spatial information delivered via the intracochlear electrode array. Postural control was measured by center of pressure (COP) and time to fall using the WiiTM (Nintendo, WA, USA) Balance Board for feet and the BalanCI for head, during the administration of the Modified Clinical Test of Sensory Interaction in Balance (CTSIB-M). The COP of the head and feet were assessed for change by deviation, measured as root mean square around the COP (COP-RMS), rate of deviation (COP-RMS/duration), and rate of path length change from center (COP-velocity). Balance was assessed by the Bruininks-Oseretsky Test of Motor Proficiency 2, balance subtest (BOT-2), specifically, BOT-2 score as well as time to fall/fault. RESULTS: In the virtual environment, children demonstrated more stable balance when using BalanCI as measured by an improvement in BOT-2 scores. In a quiet clinical setting, the use of BalanCI led to improved postural control as demonstrated by significant reductions in COP-RMS and COP-velocity. With the use of BalanCI, the number of falls/faults was significantly reduced and time to fall increased. CONCLUSIONS: BalanCI is a simple and effective means of improving postural control and balance in children with BCVL who use bilateral CI. BalanCI could potentially improve the safety of these children, reduce the effort they expend maintaining balance and allow them to take part in more complex balance tasks where sensory information may be limited and/or noisy.

Cochlear Implant Use Remains Consistent Over Time in Children With Single-Sided Deafness.

OBJECTIVES: To measure the acceptance of a cochlear implant by children with single-sided deafness (SSD) using datalogging technology in the cochlear implant processor. DESIGN: Datalogs from follow-up clinical audiology appointments for 23 children with SSD were extracted from their cochlear implant processors ranging from 1 to 8 visits (M = 3.74, SD = 1.79). The number of hours the cochlear implant was in use per day, the number of times the coil disconnected from the internal device, and the percentage of daily cochlear implant use in different auditory environments were collected from the datalogs. Linear mixed-effects regressions were used to analyze the relationship between age, hearing experience, cochlear implant use, and coil-offs per day. Nonlinear regressions were conducted to evaluate cochlear implant use in different environments. RESULTS: Children with SSD wore their cochlear implants for 6.22 (SD = 2.81; range = 0.0004 to 14.74) hours per day on average. No significant change in cochlear implant use was seen as the children grew older or gained more hearing experience. As hearing experience increased, the number of coil-offs per day was reduced. Preschoolers spent more time in "music" and "speech" and less time in "noise" and "quiet" than older and younger children while older children spent more time in "speech-in-noise." CONCLUSIONS: Children with SSD consistently wear their cochlear implants. However, the auditory environments to which they are exposed vary over time. Regular cochlear implant use by this population suggests that it does not detract from a normal-hearing ear and that children with SSD appreciate access to bilateral input.

"Aural Patching" After Bilateral Cochlear Implantation Is Challenging for Children With Prior Long-Term Unilateral Implant Experience.

OBJECTIVES: To assess the use of "aural patching" as a strategy to potentially reduce the known persistence of aural preference in children receiving bilateral cochlear implants (CIs) with long inter-implant delays by removing the first device to increase stimulation to the second implanted side. DESIGN: Children/adolescents who received a second CI at 12.8 ± 3.5 years of age after 9.4 ± 2.9 years of unilateral CI use were asked to remove their first CI for regular periods daily (aural patching). Their compliance was monitored, and asymmetries in speech perception were measured at the end of the study period. RESULTS: Partial adherence to aural patching over the first few months of bilateral hearing use markedly declined with time. As expected, the group demonstrated asymmetries in speech perception that were not significantly affected by the limited aural patching. CONCLUSIONS: The aural patching protocol was a challenge to maintain for most children and families studied, reflecting both the expected aural preference for the first implanted ear and their challenges to reverse it.

Binaural hearing is impaired in children with hearing loss who use bilateral hearing aids.

This paper asked whether children fitted with bilateral hearing aids (BHA) develop normal perception of binaural cues which are the basis of spatial hearing. Data from children with BHA (n = 26, age = 12.6 ± 2.84 years) were compared to data from a control group (n = 12, age = 12.36 ± 2.83 years). Stimuli were 250 Hz click-trains of 36 ms and a 40 ms consonant-vowel /da/ at 1 Hz presented through ER3A insert-earphones unilaterally or bilaterally. Bilateral stimuli were presented at different interaural level difference (ILD) and interaural timing difference (ITD) conditions. Participants indicated whether the sound came from the left or right side (lateralization) or whether one sound or two could be heard (binaural fusion). BHA children lateralized ILDs similarly to the control group but had impaired lateralization of ITDs. Longer response times relative to controls suggest that lateralization of ITDs was challenging for children with BHA. Most, but not all, of the BHA group were able to fuse click and speech sounds similarly to controls. Those unable to fuse showed particularly poor ITD lateralization. Results suggest that ITD perception is abnormal in children using BHAs, suggesting persistent effects of hearing loss that are not remediated by present clinical rehabilitation protocols.

Splenius capitis is a reliable target for measuring cervical vestibular evoked myogenic potentials in adults.

The cervical vestibular evoked myogenic potential (cVEMP) is a common and simple test of vestibulospinal reflex patency. In the clinic, cVEMPs are measured in response to loud sounds from the sternocleidomastoid (SCM) on the ventral neck, as subjects maintain an uncomfortable head posture needed to recruit SCM. Here we characterize the cVEMP in a dorsal neck turner (splenius capitis; SPL), and compare it with the SCM cVEMP. cVEMPs were recorded simultaneously via surface electromyography from SCM and SPL from 17 healthy subjects in a variety of postures, including head-turned postures adopted while either seated or standing, and the clinical posture. Like the SCM cVEMP recorded ipsilateral to the side of sound stimulation, the cVEMP on the contralateral SPL (synergistic with ipsilateral SCM) was characterized by a biphasic wave of muscle activity that began at ~ 13 ms. cVEMP reliability was higher on SPL vs. SCM in standing postures (chi-squared; P < 0.05), and equivalent results were obtained from SPL in a standing or seated posture. In 9 of the 17 subjects, we also obtained bilateral intramuscular (IM) recordings from SPL at the same time as the surface recordings. In these subjects, the initial surface response in SPL was associated with a consistent decrease in multi-unit IM SPL activity. Overall, these results demonstrate that SPL recordings offer a complimentary target for cVEMP assessments. The expression of SPL cVEMPs in simple head-turned postures may also improve the utility of cVEMP testing for vestibular assessment in children, the elderly, or non-compliant.

Benefits of Music Training for Perception of Emotional Speech Prosody in Deaf Children With Cochlear Implants.

OBJECTIVES: Children who use cochlear implants (CIs) have characteristic pitch processing deficits leading to impairments in music perception and in understanding emotional intention in spoken language. Music training for normal-hearing children has previously been shown to benefit perception of emotional prosody. The purpose of the present study was to assess whether deaf children who use CIs obtain similar benefits from music training. We hypothesized that music training would lead to gains in auditory processing and that these gains would transfer to emotional speech prosody perception. DESIGN: Study participants were 18 child CI users (ages 6 to 15). Participants received either 6 months of music training (i.e., individualized piano lessons) or 6 months of visual art training (i.e., individualized painting lessons). Measures of music perception and emotional speech prosody perception were obtained pre-, mid-, and post-training. The Montreal Battery for Evaluation of Musical Abilities was used to measure five different aspects of music perception (scale, contour, interval, rhythm, and incidental memory). The emotional speech prosody task required participants to identify the emotional intention of a semantically neutral sentence under audio-only and audiovisual conditions. RESULTS: Music training led to improved performance on tasks requiring the discrimination of melodic contour and rhythm, as well as incidental memory for melodies. These improvements were predominantly found from mid- to post-training. Critically, music training also improved emotional speech prosody perception. Music training was most advantageous in audio-only conditions. Art training did not lead to the same improvements. CONCLUSIONS: Music training can lead to improvements in perception of music and emotional speech prosody, and thus may be an effective supplementary technique for supporting auditory rehabilitation following cochlear implantation.

Music perception improves in children with bilateral cochlear implants or bimodal devices.

The objectives of this study were to determine if music perception by pediatric cochlear implant users can be improved by (1) providing access to bilateral hearing through two cochlear implants or a cochlear implant and a contralateral hearing aid (bimodal users) and (2) any history of music training. The Montreal Battery of Evaluation of Musical Ability test was presented via soundfield to 26 bilateral cochlear implant users, 8 bimodal users and 16 children with normal hearing. Response accuracy and reaction time were recorded via an iPad application. Bilateral cochlear implant and bimodal users perceived musical characteristics less accurately and more slowly than children with normal hearing. Children who had music training were faster and more accurate, regardless of their hearing status. Reaction time on specific subtests decreased with age, years of musical training and, for implant users, better residual hearing. Despite effects of these factors on reaction time, bimodal and bilateral cochlear implant users' responses were less accurate than those of their normal hearing peers. This means children using bilateral cochlear implants and bimodal devices continue to experience challenges perceiving music that are related to hearing impairment and/or device limitations during development.

Early unilateral cochlear implantation promotes mature cortical asymmetries in adolescents who are deaf.

Unilateral cochlear implant (CI) stimulation establishes hearing to children who are deaf but compromises bilateral auditory development if a second implant is not provided within ∼ 1.5 years. In this study we asked: 1) What are the cortical consequences of missing this early sensitive period once children reach adolescence? 2) What are the effects of unilateral deprivation on the pathways from the opposite ear? Cortical responses were recorded from 64-cephalic electrodes within the first week of bilateral CI activation in 34 adolescents who had over 10 years of unilateral right CI experience and in 16 normal hearing peers. Cortical activation underlying the evoked peaks was localized to areas of the brain using beamformer imaging. The first CI evoked activity which was more strongly lateralized to the contralateral left hemisphere than normal, with abnormal recruitment of the left prefrontal cortex (involved in cognition/attention), left temporo-parietal-occipital junction (multi-modal integration), and right precuneus (visual processing) region. CI stimulation in the opposite deprived ear evoked atypical cortical responses with abnormally large and widespread dipole activity across the cortex. Thus, using a unilateral CI to hear beyond the period of cortical maturation causes lasting asymmetries in the auditory system, requires recruitment of additional cortical areas to support hearing, and does little to protect the unstimulated pathways from effects of auditory deprivation. The persistence of this reorganization into maturity could signal a closing of a sensitive period for promoting auditory development on the deprived side.

The effects of asymmetric hearing on bilateral brainstem function: findings in children with bimodal (electric and acoustic) hearing.

As implantation criteria are broadening to include children with asymmetric hearing loss, it is important to determine the degree of residual hearing needed to protect the bilateral auditory pathways for binaural hearing and whether there is a sensitive period in development for implantation in these children. We have been studying these questions in a growing cohort of children. In the present study, auditory brainstem responses were recorded in 21 children who had 2.2 ± 2.2 years of bimodal hearing. Responses were evoked by 11-Hz acoustic clicks presented to the non-implanted ear and with biphasic electric pulses presented to the implanted ear. Twelve of these children also completed a behavioural task in which they were asked to which side of their heads bilaterally presented clicks/pulses that varied in interaural level or timing lateralized. All children experienced a delay in the non-implanted ear that resulted in 2.0 ± 0.35 ms longer peak latencies. These were further prolonged in 7 children as measured by longer interwave latencies from this ear than from the implanted ear. Despite large asymmetries in timing of brainstem activity between the two ears, all children perceived changes in interaural level differences. They were unable to detect differences in interaural timing cues. Symmetric brainstem function suggests bilateral development was preserved in some children. Future work will explore whether these children have better potential for developing binaural hearing using bimodal input.

Bilateral input protects the cortex from unilaterally-driven reorganization in children who are deaf.

Unilateral hearing in childhood restricts input along the bilateral auditory pathways, possibly causing permanent reorganization. In this study we asked: (i) do the auditory pathways develop abnormally in children who are bilaterally deaf and hear with a unilateral cochlear implant? and (ii) can such differences be reversed by restoring input to the deprived ear? We measured multichannel electroencephalography in 34 children using cochlear implants and seven normal hearing peers. Dipole moments of activity became abnormally high in the auditory cortex contralateral to the first implant as unilateral cochlear implant use exceeded 1.5 years. This resulted in increased lateralization of activity to the auditory cortex contralateral to the stimulated ear and a decline in normal contralateral activity in response to stimulation from the newly implanted ear, corresponding to poorer speech perception. These results reflect an abnormal strengthening of pathways from the stimulated ear in consequence to the loss of contralateral activity including inhibitory processes normally involved in bilateral hearing. Although this reorganization occurred within a fairly short period (∼1.5 years of unilateral hearing), it was not reversed by long-term (3-4 years) bilateral cochlear implant stimulation. In bilateral listeners, effects of side of stimulation were assessed; children with long periods of unilateral cochlear implant use prior to bilateral implantation showed a reduction in normal dominance of contralateral input in the auditory cortex ipsilateral to the stimulated ear, further confirming an abnormal strengthening of pathways from the stimulated ear. By contrast, cortical activity in children using bilateral cochlear implants after limited or no unilateral cochlear implant exposure normally lateralized to the hemisphere contralateral to side of stimulation and retained normal contralateral dominance of auditory input in both hemispheres. Results demonstrate that the immature human auditory cortex reorganizes, potentially permanently, with unilateral stimulation and that bilateral auditory input provided with limited delay can protect the brain from such changes. These results indicate for the first time that there is a sensitive period for bilateral auditory input in human development with implications for functional hearing.

Unilateral cochlear implant use promotes normal-like loudness perception in adolescents with childhood deafness.

OBJECTIVES: Behavioral measures of cochlear implant (CI) device stimulation levels can be difficult to obtain in individuals with limited or no hearing experience. Loudness measures are particularly challenging. It would therefore be useful to have a battery of objective and behavioral measures to determine CI stimulation levels in listeners with childhood deafness. In the present study, the authors characterized loudness growth in 20 adolescents: 8 with normal hearing and 12 CI participants with pre/perilingual bilateral sensorineural hearing loss. They asked (1) do adolescent CI users with childhood deafness experience similar increases in loudness as their peers with normal hearing? and (2) can loudness be predicted by objective measures of auditory activity? The authors hypothesized that loudness perception would be significantly different between CI and normal-hearing groups and that it would correlate with objective measures. DESIGN: CI users were recruited from the Cochlear Implant Program at The Hospital for Sick Children and all had used unilateral Nucleus CIs for at least 2 years. The dynamic range for each participant was defined as the difference between the behavioral threshold and the electrically evoked stapedius reflex (ESR) threshold. Loudness growth was assessed within this range behaviorally on a continuous visual scale and objectively with physiological measures. Auditory brainstem responses (ABRs) and ESRs were recorded in both groups and electrically evoked compound action potentials (ECAPs) of the auditory nerve were recorded in addition in CI listeners. The regression line slopes of ECAP and ABR amplitude growth functions were then calculated and compared with behavioral loudness growth slopes in the upper portion (40-100%) and lower portion (0-40%) of the dynamic range. Electrical pulse stimuli (in CI users) and acoustic clicks (in normal-hearing participants) were presented within each participant's dynamic range. RESULTS: The mean dynamic range in CI listeners was more variable than in normal-hearing individuals. Despite this difference, loudness at the ESR threshold was not significantly different in CI adolescents from their normal-hearing peers, and CI users exhibited normal-like loudness growth. There was a significantly positive correlation between ECAP amplitude growth and loudness growth in CI users in the upper portion of the dynamic range, while ABR wave V amplitude growth was not related to loudness growth in either group. CONCLUSIONS: We confirmed that the ESR threshold is a good measure of comfortably loud levels in adolescents with cochlear implants and their normal-hearing peers. Adolescents using CIs show normal-like rates of loudness growth on average, despite having highly variable dynamic ranges of hearing. Individual rates of loudness growth in the upper dynamic range in CI users can be predicted by the rate of amplitude growth of the ECAP. Thus, the rate of neural recruitment with increasing CI current is important for loudness perception in pre/perilingually deaf listeners and should be considered when programming their CIs.

Head and Eye Movements Reveal Compensatory Strategies for Acute Binaural Deficits During Sound Localization.

The present study aimed to define use of head and eye movements during sound localization in children and adults to: (1) assess effects of stationary versus moving sound and (2) define effects of binaural cues degraded through acute monaural ear plugging. Thirty-three youth (MAge = 12.9 years) and seventeen adults (MAge = 24.6 years) with typical hearing were recruited and asked to localize white noise anywhere within a horizontal arc from -60° (left) to +60° (right) azimuth in two conditions (typical binaural and right ear plugged). In each trial, sound was presented at an initial stationary position (L1) and then while moving at ∼4°/s until reaching a second position (L2). Sound moved in five conditions (±40°, ±20°, or 0°). Participants adjusted a laser pointer to indicate L1 and L2 positions. Unrestricted head and eye movements were collected with gyroscopic sensors on the head and eye-tracking glasses, respectively. Results confirmed that accurate sound localization of both stationary and moving sound is disrupted by acute monaural ear plugging. Eye movements preceded head movements for sound localization in normal binaural listening and head movements were larger than eye movements during monaural plugging. Head movements favored the unplugged left ear when stationary sounds were presented in the right hemifield and during sound motion in both hemifields regardless of the movement direction. Disrupted binaural cues have greater effects on localization of moving than stationary sound. Head movements reveal preferential use of the better-hearing ear and relatively stable eye positions likely reflect normal vestibular-ocular reflexes.

Variants in Genes Associated with Hearing Loss in Children: Prevalence in a Large Canadian Cohort.

OBJECTIVE: The objective of this study was to assess the prevalence of genetic variants associated with hearing loss in a large cohort of children in Canada using high throughput next generation sequencing (NGS). METHODS: A total of 485 children with hearing loss underwent NGS testing with an 80 gene panel of syndromic and non-syndromic variants known to be associated with hearing loss. Genetic variants were classified as pathogenic, likely pathogenic, likely benign, benign, or variants of uncertain significance (VUS), according to the American College of Medical Genetics and Genomics guidelines. RESULTS: Across the 80 genes tested, 923 variants, predominantly in 28 genes, were identified in 324 children. Pathogenic variants occurred in 19/80 (23.8%) of the hearing loss related genes tested and confirmed the etiology of hearing loss in 73/485 (15.1%) of children. GJB2 was the most prevalent gene, affecting 28/73 (38.4%) children with confirmed genetic hearing loss in our cohort. Most identified variants (748/923, 81.0%, in 76/80 genes) were of uncertain significance. CONCLUSION: Genetic testing using NGS identified the etiology in approximately 15% of childhood hearing loss in a Canadian cohort which is lower than what is typically reported. GJB2 was the most common genetic cause of hearing loss. VUS are commonly identified, presenting clinical challenges for counseling. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:3832-3838, 2024.