Corrigendum: Brain morphological modifications in congenital and acquired auditory deprivation: a systematic review and coordinate-based meta-analysis.

[This corrects the article DOI: 10.3389/fnins.2022.850245.].

Influence of the Electrode Montage to Detect Ocular Vestibular Evoked Myogenic Potentials.

OBJECTIVE: To compare ocular vestibular evoked myogenic potentials (oVEMPs) obtained with three different electrode montages (infra-orbital vs belly-tendon vs chin) in a group of healthy subjects. To assess the electrical activity recorded at the level of the reference electrode in the belly-tendon and chin montages. STUDY DESIGN: Prospective study. SETTING: Tertiary referral center. PATIENTS: Twenty-five healthy adult volunteers. INTERVENTIONS: Each ear was tested separately via air-conducted sound (500 Hz Narrow Band CE-Chirps at 100 dB nHL) for recording contralateral myogenic responses. Recording conditions were randomized. MAIN OUTCOME MEASURES: n1-p1 amplitudes values, interaural amplitude asymmetry ratios (ARs) and response rates. RESULTS: The belly-tendon electrode montage (BTEM) produced larger amplitudes than the chin ( p = 0.008) and the IOEM (infra-orbital electrode montage; p < 0.001). The chin montage displayed larger amplitudes than the IOEM ( p < 0.001). The interaural amplitude asymmetry ratios (ARs) were not affected by the different electrode montages ( p = 0.549). In 100% of cases, oVEMPs were detected bilaterally with the BTEM which is higher than with the chin and the IOEM ( p < 0.001; p = 0.020, respectively). We did not record any VEMP when placing the active electrode on the contralateral internal canthus or the chin and the reference electrode on the dorsum of the hand. CONCLUSIONS: The BTEM increased the amplitudes recorded and response rate in healthy subjects. No positive or negative reference contamination was observed with the belly-tendon or chin montages.

Genetic Evaluation of Prelingual Hearing Impairment: Recommendations of an European Network for Genetic Hearing Impairment.

The cause of childhood hearing impairment (excluding infectious pathology of the middle ear) can be extrinsic (embryofoetopathy, meningitis, trauma, drug ototoxicity, noise trauma, etc [...].

Prospective Comparison Between Manual and Computer-Assisted (FOX) Cochlear Implant Fitting in Newly Implanted Patients.

OBJECTIVE: A prospective, longitudinal, randomized controlled trial with an original crossover design for 1 year was conducted to compare manual fitting to artificial intelligence-based fitting in newly implanted patients. DESIGN: Twenty-four patients who received their first cochlear implant (CI) were randomly assigned to the manual or Fitting to Outcome eXpert (FOX) arm; they followed the corresponding fitting procedures for 1 year. After 1 year, each patient was switched to another arm. The number of fittings, auditory outcomes (pure-tone thresholds, loudness scaling curves, spectral discrimination scores, bisyllabic word recognition in quiet and noise, and speech tracking), fitting session questionnaire, and CI parameters (T level, C level, Threshold Sound Pressure Level (T-SPL), Comfortable Sound Pressure Level (C-SPL), and loudness growth value) were compared between the two groups. Differences between the two groups were analyzed using the Mann-Whitney test, and Holm corrections were applied for multiple statistical tests. At the end of the crossover session, patients were offered the choice to continue with their old or new map. RESULTS: As early as 3 mo postactivation, the FOX group showed less variability and significantly better speech intelligibility in quiet conditions at 40 and 55 dB SPL and noise ( p < 0.05) with median phoneme scores of 50%, 70%, and 50% at 55, 70, and 85 dB SPL compared with 45%, 50%, and 40%, respectively. This group showed better results at 12 mo postactivation ( p < 0.05). In the manual group, 100% of the patients decided to keep the new FOX map, and 82% performed better with the FOX map. In the FOX group, 63% of the patients decided to keep the manual map, although the measurable outcome had not improved. In this group, participants reported to prefer the manual map because it felt more comfortable, even if the FOX map gave better measured outcome. CONCLUSION: Although the study size remains relatively small, the AI-FOX approach was equivalent to or even outperformed the manual approach in hearing performance, comfort, and resources. Furthermore, FOX is a tool capable of continuous improvement by comparing its predictions with observed results and is continuously learning from clinicians' practice, which is why this technology promises major advances in the future.

Three Years of Vestibular Infant Screening in Infants With Sensorineural Hearing Loss.

OBJECTIVES: Although vestibular deficits are more prevalent in hearing-impaired children and can affect their development on many levels, a pediatric vestibular assessment is still uncommon in clinical practice. Since early detection may allow for timely intervention, this pioneer project has implemented a basic vestibular screening test for each six-month-old hearing-impaired infant in Flanders, Belgium. This study aims to report the vestibular screening results over a period of three years and to define the most important risk factors for abnormal vestibular screening results. METHODS: Cervical Vestibular Evoked Myogenic Potentials with bone-conduction were used as a vestibular screening tool in all reference centers affiliated to the Universal Newborn Hearing Screening Program in Flanders. From June 2018 until June 2021, 254 infants (mean age: 7.4 months, standard deviation: 2.4 months) with sensorineural hearing loss were included. RESULTS: Overall, abnormal vestibular screening results were found in 13.8% (35 of 254) of the infants. The most important group at risk for abnormal vestibular screening results were infants with unilateral or bilateral severe to profound sensorineural hearing loss (20.8%, 32 of 154) (P < .001, odds ratio = 9.16). Moreover, abnormal vestibular screening results were more prevalent in infants with hearing loss caused by meningitis (66.7%, 2 of 3), syndromes (28.6%, 8 of 28), congenital cytomegalovirus infection (20.0%, 8 of 40), and cochleovestibular anomalies (19.2%, 5 of 26). CONCLUSIONS: The vestibular screening results in infants with sensorineural hearing loss indicate the highest risk for vestibular deficits in severe to profound hearing loss, and certain underlying etiologies of hearing loss, such as meningitis, syndromes, congenital cytomegalovirus, and cochleovestibular anomalies.

Brain Morphological Modifications in Congenital and Acquired Auditory Deprivation: A Systematic Review and Coordinate-Based Meta-Analysis.

Neuroplasticity following deafness has been widely demonstrated in both humans and animals, but the anatomical substrate of these changes is not yet clear in human brain. However, it is of high importance since hearing loss is a growing problem due to aging population. Moreover, knowing these brain changes could help to understand some disappointing results with cochlear implant, and therefore could improve hearing rehabilitation. A systematic review and a coordinate-based meta-analysis were realized about the morphological brain changes highlighted by MRI in severe to profound hearing loss, congenital and acquired before or after language onset. 25 papers were included in our review, concerning more than 400 deaf subjects, most of them presenting prelingual deafness. The most consistent finding is a volumetric decrease in gray matter around bilateral auditory cortex. This change was confirmed by the coordinate-based meta-analysis which shows three converging clusters in this region. The visual areas of deaf children is also significantly impacted, with a decrease of the volume of both gray and white matters. Finally, deafness is responsible of a gray matter increase within the cerebellum, especially at the right side. These results are largely discussed and compared with those from deaf animal models and blind humans, which demonstrate for example a much more consistent gray matter decrease along their respective primary sensory pathway. In human deafness, a lot of other factors than deafness could interact on the brain plasticity. One of the most important is the use of sign language and its age of acquisition, which induce among others changes within the hand motor region and the visual cortex. But other confounding factors exist which have been too little considered in the current literature, such as the etiology of the hearing impairment, the speech-reading ability, the hearing aid use, the frequent associated vestibular dysfunction or neurocognitive impairment. Another important weakness highlighted by this review concern the lack of papers about postlingual deafness, whereas it represents most of the deaf population. Further studies are needed to better understand these issues, and finally try to improve deafness rehabilitation.

Evaluation of the transmastoid plugging approach for superior semicircular canal dehiscences: a retrospective series of 30 ears.

PURPOSE: The classical surgical approach for superior semicircular canal dehiscences (SSCD) is via the extradural middle cranial fossa. This pathway is used to resurface or to plug the SSC. In this paper, we present long-term data on an alternative route: the transmastoid pathway. The predictive factors for a successful surgery are equally presented in this paper. METHODS: Thirty reports of patients operated between September 2007 to January 2020 were analysed. SSCD was confirmed by the association of concordant complaints, audiometric data, cervical vestibular evoked myogenic potentials (cVEMP) responses and computerized tomography findings. Before and after surgery, the following factors were analysed: auditory and vestibular subjective symptoms, Tullio phenomenon, pure-tone audiometry thresholds for air and bone conduction, air-bone gap, cVEMP threshold, and computerized tomography data, for instance the size of the dehiscence. RESULTS: The follow-up is 21 months on average. The transmastoid approach significantly improves all symptoms (although there were less probing results for the vestibular symptoms). Objectively, we can observe, a closure of the audiometric air-bone gap on the low frequencies and an improvement in the cVEMP. The only correlation that was identified was between the preoperative cVEMP results and the postoperative air conduction. CONCLUSIONS: The originality of this study is the long postoperative follow-up. It allowed us to evaluate the symptoms in the long term and to determine a predictive factor of postoperative complication, which has not yet been described until today.The transmastoid plugging technique is safe and effective. Additional long-term data with a larger cohort are needed to confirm our results and correlation studies.

Using Narrow Band CE-Chirps to Elicit Cervical Vestibular Evoked Myogenic Potentials.

OBJECTIVES: To compare the effects of Narrow band CE-Chirps (NB CE-Chirps) and tone bursts (TBs) at 500 Hz and 1000 Hz on the amplitudes and latencies in cervical vestibular evoked myogenic potentials (cVEMPs). DESIGN: Thirty-one healthy adult volunteers of varying ages were tested by air conduction at 95 dB nHL. Recording conditions were randomized for each participant and each modality was tested twice. RESULTS: NB CE-Chirps showed larger corrected amplitudes than TBs at 500 Hz (p < 0.001) which were themselves larger than NB CE-Chirps and TBs at 1000 Hz (p < 0.001). In older volunteers, NB CE-Chirps 500 and 1000 Hz had significantly higher response rates than TBs 500 Hz (p = 0.039). A negative correlation was observed between the corrected amplitudes and the age of the participants regardless of the stimulus and the frequency studied. The p13 and n23 latencies were not correlated with the age of the subjects. CONCLUSIONS: NB CE-Chirps at 500 Hz improved the corrected amplitudes of waveforms in cVEMPs as a result of a better frequency specificity compared with TBs. In the elderly, eliciting cVEMPs at a frequency of 1000 Hz might not be necessary to improve response rates with NB CE-Chirps. Additional studies including a higher number of healthy participants and patients with vestibular disorders are required to confirm these observations.

The Cognitive-Vestibular Compensation Hypothesis: How Cognitive Impairments Might Be the Cost of Coping With Compensation.

Previous research in vestibular cognition has clearly demonstrated a link between the vestibular system and several cognitive and emotional functions. However, the most coherent results supporting this link come from rodent models and healthy human participants artificial stimulation models. Human research with vestibular-damaged patients shows much more variability in the observed results, mostly because of the heterogeneity of vestibular loss (VL), and the interindividual differences in the natural vestibular compensation process. The link between the physiological consequences of VL (such as postural difficulties), and specific cognitive or emotional dysfunction is not clear yet. We suggest that a neuropsychological model, based on Kahneman's Capacity Model of Attention, could contribute to the understanding of the vestibular compensation process, and partially explain the variability of results observed in vestibular-damaged patients. Several findings in the literature support the idea of a limited quantity of cognitive resources that can be allocated to cognitive tasks during the compensation stages. This basic mechanism of attentional limitations may lead to different compensation profiles in patients, with or without cognitive dysfunction, depending on the compensation stage. We suggest several objective and subjective measures to evaluate this cognitive-vestibular compensation hypothesis.

The Visuo-Spatial Abilities Diagnosis (VSAD) test: Evaluating the potential cognitive difficulties of children with vestibular impairment through a new tablet-based computerized test battery.

Recent data collected on adult patients with vestibular loss (VL) tend to demonstrate possible cognitive impairments in visuospatial working memory, mental rotation, selective attention, and space orientation. However, the neuropsychological profile of children with VL remains largely under-investigated in the scientific literature. Although previous research has shown that children with VL may experience some degree of delayed motor development, it is not yet clear if VL could also lead to specific delayed cognitive development. In this study, we will present the development and validation of a new tablet-based computerized test battery (VSAD) that evaluates visuospatial working memory, mental rotation, selective attention, and space orientation abilities. Thirteen children with VL and 54 average-age matched healthy children performed the VSAD and classical paper-and-pencil neuropsychological tasks twice within a 1-month interval. Our results demonstrated a good concurrent validity with strong correlations between the visuospatial working memory, mental rotation, and space orientation tests of the VSAD and classical tasks. Test-retest reliability was also supported through good intra-class coefficients. However, the test of selective attention showed no concurrent validity with the matched classical task. The discriminant validity of the VSAD was partially supported for visuospatial working memory and mental rotation performance accuracy. The VSAD shows good concurrent validity and reliability for measuring visuospatial working memory, mental rotation, and space orientation in children with VL. Future studies are needed to extend discriminant validity with other populations.

Variability of fitting parameters across cochlear implant centres.

OBJECTIVE: As a follow-up to the studies by Vaerenberg et al. (Sci World J 501738:1-12, 2014) and Browning et al. (Cochlear Implant Int 21(3):1-13, 2020), who used questionnaires, we determined whether there are between-centre variations in the fitting of cochlear implants by analysing the methodology, fitting parameters and hearing results of patients from four centres with real data. The purpose of this study is to highlight the lack of streamlined mapping guides and outcome measures with respect to cochlear implant (CI) fittings. METHODS: A retrospective study with ninety-seven post-lingual adults with a nucleus cochlear implant placed between 2003 and 2013 was included to ensure at least 5 years of follow-up. The studied data were as follows: the methodology, including the fitter's professional background, the method of activation, the sequence of fitting sessions, the objectives measures and hearing outcomes; and the fitting parameters, including the speech processors, programming strategy, stimulation mode, T and C levels, T-SPL and C-SPL, maxima, pulse width, loudness growth and hearing results. RESULTS: This investigation highlights some common practices across professionals and CI centres: the activation of a CI is behavioural; impedances are systematically measured at each fitting; and some parameters are rarely modified. However, there are also differences, either between centres, such as the sequences of fitting sessions (p < 0.05) or their approach to spectral bands (p < 0.05), or even within centres, such as the policy regarding T and C levels at high frequencies compared to those at low and mid-frequencies. CONCLUSION: There are important variations between and within centres that reflect a lack of CI-related policies and outcome measures in the fitting of CI. CLINICAL TRIALS REGISTRY: NCT03700268.

Chudley-McCullough Syndrome: A Recognizable Clinical Entity Characterized by Deafness and Typical Brain Malformations.

Chudley-McCullough syndrome, a rare autosomal recessive disorder due to pathogenic variants in the GPSM2 (G-protein signaling modulator 2) gene, is characterized by early-onset sensorineural deafness and a typical combination of brain malformations, including ventriculomegaly, (partial) agenesis of the corpus callosum, cerebellar dysplasia, arachnoid cysts, frontal subcortical heterotopia, and midline polymicrogyria. When hearing loss is managed early, most patients have minor or no impairment of motor and cognitive development, despite the presence of brain malformations. We report 2 cases of Chudley-McCullough syndrome, one presenting with congenital deafness and normal development except for speech delay and one presenting prenatally with ventriculomegaly and an atypical postnatal course characterized by epileptic spasms, deafness, and moderate intellectual disability. These highlight the challenges faced by clinicians when predicting prognosis based on pre- or postnatal imaging of brain malformations. We have also reviewed the phenotype and genotype of previous published cases to better understand Chudley-McCullough syndrome.

Vestibular Infant Screening (VIS)-Flanders: results after 1.5 years of vestibular screening in hearing-impaired children.

Due to the close anatomical relationship between the auditory and vestibular end organs, hearing-impaired children have a higher risk for vestibular dysfunction, which can affect their (motor) development. Unfortunately, vestibular dysfunction often goes unnoticed, as vestibular assessment in these children is not standard of care nowadays. To timely detect vestibular dysfunction, the Vestibular Infant Screening-Flanders (VIS-Flanders) project has implemented a basic vestibular screening test for hearing-impaired infants in Flanders (Belgium) with a participation rate of 86.7% during the first year and a half. The cervical Vestibular Evoked Myogenic Potentials (cVEMP) test was applied as vestibular screening tool to map the occurrence of vestibular (mainly saccular) dysfunction in this population. At the age of 6 months, 184 infants were screened. No refers on vestibular screening were observed in infants with permanent conductive hearing loss. In infants with permanent sensorineural hearing loss, a cVEMP refer rate of 9.5% was observed. Failure was significantly more common in infants with severe-profound compared to those with mild-moderate sensorineural hearing loss (risk ratio = 9.8). Since this is the first regional study with a large sample size and successful participation rate, the VIS-Flanders project aims to set an example for other regions worldwide.

Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports.

Auditory neuropathy spectrum disorder (ANSD) refers to a range of hearing impairments characterized by deteriorated speech perception, despite relatively preserved pure-tone detection thresholds. Affected individuals usually present with abnormal auditory brainstem responses (ABRs), but normal otoacoustic emissions (OAEs). These electrophysiological characteristics have led to the hypothesis that ANSD may be caused by various dysfunctions at the cochlear inner hair cell (IHC) and spiral ganglion neuron (SGN) levels, while the activity of outer hair cells (OHCs) is preserved, resulting in discrepancies between pure-tone and speech comprehension thresholds. The exact prevalence of ANSD remains unknown; clinical findings show a large variability among subjects with hearing impairment ranging from mild to profound hearing loss. A wide range of prenatal and postnatal etiologies have been proposed. The study of genetics and of the implicated sites of lesion correlated with clinical findings have also led to a better understanding of the molecular mechanisms underlying the various forms of ANSD, and may guide clinicians in better screening, assessment and treatment of ANSD patients. Besides OAEs and ABRs, audiological assessment includes stapedial reflex measurements, supraliminal psychoacoustic tests, electrocochleography (ECochG), auditory steady-state responses (ASSRs) and cortical auditory evoked potentials (CAEPs). Hearing aids are indicated in the treatment of ANSD with mild to moderate hearing loss, whereas cochlear implantation is the first choice of treatment in case of profound hearing loss, especially in case of IHC presynaptic disorders, or in case of poor auditory outcomes with conventional hearing aids.

Neuropsychological profiles of children with vestibular loss.

BACKGROUND: The impact of vestibular loss (VL) on cognition has been previously studied in experimental animal, human and adult patient studies showing links between VL, and cognitive impairments in space orientation, working memory, mental rotation and selective attention. However, few studies have been conducted on children with VL. OBJECTIVE: We investigated for the first time, the impact of a VL on children's cognition. METHODS: 13 children with VL (10 years, 5 months) and 60 average-age matched controls performed a neuropsychological assessment consisting of visuospatial working memory, selective visual attention, mental rotation and space orientation tasks. RESULTS: Children with VL recalled smaller sequences for both forward and backward memory subtasks (mean±SD = 6.3±1.9 and 5.3±2.6) than controls (8.2±2.3 and 7.3±2.0), have less accurate mental rotation scores (25.4±6 versus 30.8±5.1) and greater additional distance travelled in the maze task (96.4±66.6 versus 60.4±66.3); all corrected p-values <0.05. Selective visual attention measures do not show significant differences. CONCLUSIONS: Children with VL show similar cognitive difficulties that adults with VL, in tasks involving dynamic cognitive processes (higher attentional load) that in tasks requiring static cognitive processes such as visual attention task.

From manual to artificial intelligence fitting: Two cochlear implant case studies.

Objective: To assess whether CI programming by means of a software application using artificial intelligence (AI), FOX®, may improve cochlear implant (CI) performance. Patients: Two adult CI recipients who had mixed auditory results with their manual fitting were selected for an AI-assisted fitting. Even after 17 months CI experience and 19 manual fitting sessions, the first subject hadn't developed open set word recognition. The second subject, after 9 months of manual fitting, had developed good open set word recognition, but his scores remained poor at soft and loud presentation levels. Main outcome measure(s): Cochlear implant fitting parameters, pure tone thresholds, bisyllabic word recognition, phonemic discrimination scores and loudness scaling curves. Results: For subject 1, a first approach trying to optimize the home maps by means of AI-proposed adaptations was not successful whereas a second approach based on the use of Automaps (an AI approach based on universal, i.e. population based group statistics) during 3 months allowed the development of open set word recognition. For subject 2, the word recognition scores improved at soft and loud intensities with the AI suggestions. The AI-suggested modifications seem to be atypical. Conclusions: The two case studies illustrate that adults implanted with manual CI fitting may experience an improvement in their auditory results with AI-assisted fitting.

Changes of spatial and temporal characteristics of dynamic postural control in children with typical neurodevelopment with age: Results of a multicenter pediatric study.

BACKGROUND: The aim of this multicenter study is to investigate the effect of chronological age and gender in postural control. METHODS: To approach an ecological model, we used a multicenter posturography assessment. We analyzed postural control with surface, mean velocity of center of pressure [CoP] and temporal analysis, with Postural Instability Index [PII] being a more sensitive parameter in postural evaluation. A large sample of 156 age- and gender-matched healthy children recruited in several pediatrics hospitals, participated. RESULTS: Our current results showed a significant decrease of all postural parameters (surface, mean velocity of CoP and PII) with age, and only on stable support condition. Our study additionally described a gender effect in conditions where all sensory inputs are most challenged with a mean velocity of CoP being significantly smaller in girls with respect to boys. CONCLUSION: We concluded that postural control improves with age linked with maturation process. Moreover, this maturation process seems not yet achieved at 16.08 years and still ongoing beyond. Interestingly, our result reported specificities linked with gender effect. Indeed, girls and boys do not proceed in the same way to maintain their postural control. We could make hypothesis that more children maintain their postural control efficiently; with a low energy cost, the more they could allocate attention to learning during childhood.

A key role of the prefrontal cortex in the maintenance of chronic tinnitus: An fMRI study using a Stroop task.

INTRODUCTION: Since we recently showed in behavioural tasks that the top-down cognitive control was specifically altered in tinnitus sufferers, here we wanted to establish the link between this impaired executive function and brain alterations in the frontal cortex in tinnitus patients. METHOD: Using functional magnetic resonance imaging (fMRI), we monitored the brain activity changes in sixteen tinnitus patients (TP) and their control subjects (CS) while they were performing a spatial Stroop task, both in audition and vision. RESULTS: We observed that TP differed from CS in their functional recruitment of the dorsolateral prefrontal cortex (dlPFC, BA46), the cingulate gyrus and the ventromedial prefrontal cortex (vmPFC, BA10). This recruitment was higher during interference conditions in tinnitus participants than in controls, whatever the sensory modality. Furthermore, the brain activity level in the right dlPFC and vmPFC correlated with the performance in the Stroop task in TP. CONCLUSION: Due to the direct link between poor executive functions and prefrontal cortex alterations in TP, we postulate that a lack of inhibitory modulation following an impaired top-down cognitive control may maintain tinnitus by hampering habituation mechanisms. This deficit in executive functions caused by prefrontal cortex alterations would be a key-factor in the generation and persistence of tinnitus.

Childhood hearing loss is a key feature of CAPOS syndrome: A case report.

CAPOS syndrome (cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss) is a rare neurological disorder, recently associated with the c.2452G > A hotspot mutation in the ATP1A3 gene, with sensorineural hearing loss as a prominent feature. We herein report on a girl who has experienced hearing loss for three years following an initial encephalitic episode when aged 15 months old. CAPOS was diagnosed only when she was six years old by targeted testing whilst she displayed optic atrophy, cerebellar signs and areflexia. CAPOS syndrome should be considered in the differential diagnosis of acquired childhood deafness, prompting clinicians to search for associated neurological features.