Hearing instability and abnormal auditory pathways in infants with congenital cytomegalovirus infection: An audiological and radiological single-centre prospective cohort analysis.

INTRODUCTION: This prospective cohort study aims to investigate the hearing dynamics and the changes in the central auditory pathways in infants with congenital cytomegalovirus (cCMV) infection. MATERIALS AND METHODS: cCMV-infected neonates aged ≤3 weeks old were recruited and underwent clinical and laboratory tests to detect viremia and symptomatic infection, hearing examinations at three and six months of age, and radiological imaging of brain auditory pathways using diffusion tensor imaging. RESULTS: From 26 eligible infants (52 ears), we detected symptomatic infection in nine (34.6%), viremia in 14 (14/25; 56.0%) and sensorineural hearing loss (SNHL) in 14 infants (53.8%). We observed 40 ears (76.9%) with unstable hearing thresholds, 17 (42.5%) of which fluctuated. Hearing fluctuation and progressivity were more common in symptomatic infection (66.7% vs. 14.7%, p<0.001; and 38.9% vs. 2.9%, p=0.002; respectively). A substantial proportion of ears had reduced fractional anisotropy (FA) in the medial geniculate body (59.1%), superior olivary nucleus (45.5%), trapezoid body (40.9%), auditory radiation (36.4%) and inferior colliculus (31.8%). Symptomatic infection was associated with an increased FA in the medial geniculate body (mean difference, MD: 0.12; 95% Confidence Intervals, 95%CI: 0.03, 0.22) and viremia in the inferior colliculus (MD: 0.09; 95%CI: 0.02, 0.16). An FA in the inferior colliculus of ≥0.404 had a sensitivity and specificity of 68.8% and 83.3% in predicting viremia (area under the curve 0.823; 95%CI: 0.633, 1.000, p=0.022). CONCLUSION: SNHL along with its fluctuation and progression are common in cCMV-infected infants. cCMV infection may induce structural changes in the central auditory pathway.

Peripheral and brainstem auditory evaluation in post-COVID-19 individuals.

PURPOSE: The purpose of this study was to investigate the peripheral and central auditory pathways in adult individuals after COVID-19 infection. METHOD: A total of 44 individuals aged between 19 and 58 years, of both genders, post-COVID-19 infection, confirmed by serological tests, with no previous hearing complaints and no risk factors for hearing loss, were assessed. All the participants underwent the following procedures: pure tone audiometry, logoaudiometry, immitanciometry, and Brainstem Auditory Evoked Potentials (BAEP), in addition to answering a questionnaire about auditory symptoms. RESULTS: Thirteen individuals (29.5 %) had some hearing threshold impairment, mainly sensorineural hearing loss. In the BAEP, 18 individuals (40.9 %) presented longer latencies, mainly in waves III and V. According to the questionnaire answers, 3 individuals (9.1 %) reported worsened hearing and 7 (15.9 %) tinnitus that emerged after the infection. As for the use of ototoxic drugs during treatment, 7 individuals (15.9 %) reported their use, of which 5 showed abnormalities in peripheral and/or central auditory assessments. CONCLUSION: Considering the self-reported hearing complaints after COVID-19 infection and the high rate of abnormalities found in both peripheral and central audiological assessments, it is suggested that the new COVID-19 may compromise the auditory system. Due to the many variables involved in this study, the results should be considered with caution. However, it is essential that audiological evaluations are carried out on post-COVID-19 patients in order to assess the effects of the infection in the short, medium, and long term. Future longitudinal investigations are important for a better understanding of the auditory consequences of COVID-19.

The Audiological Profile and Rehabilitation of Patients with Incomplete Partition Type II and Large Vestibular Aqueducts.

BACKGROUND:  Incomplete partition type II (IP-II) malformation is often accompanied by a large vestibular aqueduct (LVA). In IP anomalies, the patient's auditory rehabilitation requirements are decided according to the presence of inner ear structures and the degree of hearing loss (HL). There has been limited research on auditory rehabilitation (AR) requirement selection in patients diagnosed with IP-II and LVA. This study investigated the typical characteristics of HL and AR choices in patients diagnosed with IP-II and LVA. METHODS:  Patients with IP-II and LVA (n=55; 25 women and 30 men) were identified, and audiological evaluations were performed. The patient's demographic characteristics, the type and degree of HL, the AR method, age at diagnosis, and educational status were retrospectively compared. RESULTS:  The distribution of our 55 patients according to cochlear implants, hearing aids (HA), and bimodal applications was 29.1% (n=16), 43.6% (n=24), and 27.3% (n=15), respectively. Statistical analyses using chi-square tests found no significant differences in the incidence of dizziness/imbalance, tinnitus, HL progression, or the degree and onset of HL among the patients. CONCLUSION:  The data revealed different audiological characteristics among patients with IP-II and LVA, as well as different AR solutions. The most widely used AR modality was found to be HA. Prediction of sudden versus progressive HL development among patients is challenging, and the characteristics of IP-II vary. Therefore, they should be interpreted with caution.

Toward parametric Bayesian adaptive procedures for multi-frequency categorical loudness scaling.

A series of Bayesian adaptive procedures to estimate loudness growth across a wide frequency range from individual listeners was developed, and these procedures were compared. Simulation experiments were conducted based on multinomial psychometric functions for categorical loudness scaling across ten test frequencies estimated from 61 listeners with normal hearing and 87 listeners with sensorineural hearing loss. Adaptive procedures that optimized the stimulus selection based on the interim estimates of two types of category-boundary models were tested. The first type of model was a phenomenological model of category boundaries adopted from previous research studies, while the other type was a data-driven model derived from a previously collected set of categorical loudness scaling data. An adaptive procedure without Bayesian active learning was also implemented. Results showed that all adaptive procedures provided convergent estimates of the loudness category boundaries and equal-loudness contours between 250 and 8000 Hz. Performing post hoc model fitting, using the data-driven model, on the collected data led to satisfactory accuracies, such that all adaptive procedures tested in the current study, independent of modeling approach and stimulus-selection rules, were able to provide estimates of the equal-loudness-level contours between 20 and 100 phons with root-mean-square errors typically under 6 dB after 100 trials.

[Advances in vestibular function and rehabilitation of large vestibular aqueduct syndrome].

Large vestibular aqueduct syndrome（LVAS） is a common recessive hereditary hearing loss disease, and some patients may also experience vestibular dysfunction. With the wide application of cochlear implant（CI） and the development of vestibular medicine, the pathophysiological mechanism of LVAS and the influence mechanism of CI on vestibular function are gradually elucidated. Consequently, the evaluation and rehabilitation of vestibular dysfunction function have also become research hotspots. This article reviews studies on vestibular function and related rehabilitation in patients with large vestibular aqueduct syndrome.

Amplitude Parameters Are Predictive of Hearing Preservation in a Randomized Controlled Trial of Intracochlear Electrocochleography During Cochlear Implant Surgery.

OBJECTIVE: To prospectively evaluate the association between hearing preservation after cochlear implantation (CI) and intracochlear electrocochleography (ECochG) amplitude parameters. STUDY DESIGN: Multi-institutional, prospective randomized clinical trial. SETTING: Ten high-volume, tertiary care CI centers. PATIENTS: Adults (n = 87) with sensorineural hearing loss meeting CI criteria (2018-2021) with audiometric thresholds of ≤80 dB HL at 500 Hz. METHODS: Participants were randomized to CI surgery with or without audible ECochG monitoring. Electrode arrays were inserted to the full-depth marker. Hearing preservation was determined by comparing pre-CI, unaided low-frequency (125-, 250-, and 500-Hz) pure-tone average (LF-PTA) to LF-PTA at CI activation. Three ECochG amplitude parameters were analyzed: 1) insertion track patterns, 2) magnitude of ECochG amplitude change, and 3) total number of ECochG amplitude drops. RESULTS: The Type CC insertion track pattern, representing corrected drops in ECochG amplitude, was seen in 76% of cases with ECochG "on," compared with 24% of cases with ECochG "off" ( p = 0.003). The magnitude of ECochG signal drop was significantly correlated with the amount of LF-PTA change pre-CI and post-CI ( p < 0.05). The mean number of amplitude drops during electrode insertion was significantly correlated with change in LF-PTA at activation and 3 months post-CI ( p ≤ 0.01). CONCLUSIONS: ECochG amplitude parameters during CI surgery have important prognostic utility. Higher incidence of Type CC in ECochG "on" suggests that monitoring may be useful for surgeons in order to recover the ECochG signal and preventing potentially traumatic electrode-cochlear interactions.

The Role of Asymmetry Values, Gain, and Pathological Saccades of the Video Head Impulse Test (vHIT) in Sudden Sensorineural Hearing Loss.

OBJECTIVE: The purpose of this study was to evaluate the value of asymmetry values, gain, and pathological saccades of the video head impulse test (vHIT) in sudden sensorineural hearing loss (SSNHL). STUDY DESIGN: Retrospective study. SETTING: Tertiary referral center. PATIENTS: A total of 226 individuals diagnosed with unilateral definite SSNHL were hospitalized. The assessment included a comprehensive evaluation of medical history, pure-tone test, acoustic impedance, positional test, video nystagmography (VNG), vHIT, vestibular evoked myogenic potentials (VEMPs) and magnetic resonance. INTERVENTIONS: vHIT, VNG, cVEMP, oVEMP. Statistical analysis was performed with SPSS version 22.0 for Windows. MAIN OUTCOME MEASURES: The asymmetry values, gain, and pathological saccades of the vHIT. RESULTS: The abnormal gain of vHIT in anterior, horizontal, and posterior canal in SSNHL patients with vertigo were revealed in 20 of 112 (17.9%), 24 of 112 (21.4%), and 60 of 112 (53.6%), respectively. The vHIT pathological saccades (overt + covert) of anterior, horizontal, and posterior canal in SSNHL patients with vertigo were observed in 5 of 112 (4.6%), 52 of 112 (46.4%), and 58 of 112 (51.8%), respectively. Multivariate analysis indicated that the prognosis of patients with vertigo was correlated with vHIT gain of posterior canal, pathological saccade in horizontal canal, asymmetric ratio of horizontal canal gain, asymmetric ratio of posterior canal gain, Canal paresis (%) on caloric test and spontaneous nystagmus. CONCLUSION: In the vHIT of patients with SSNHL with vertigo, the posterior canal is most easily affected. Reduced gain of posterior canal, pathological saccade of horizontal canal, and larger asymmetric gain of posterior canal and horizontal canal may be negative prognostic factors.

Postural Sway Velocity of Deaf Children with and without Vestibular Dysfunction.

BACKGROUND: Sensory information obtained from the visual, somatosensory, and vestibular systems is responsible for regulating postural control, and if damage occurs in one or more of these sensory systems, postural control may be altered. OBJECTIVE: To evaluate and compare the postural sway velocity between children with normal hearing and with sensorineural hearing loss (SNHL), matched by sex and age group, and to compare the postural sway velocity between children with normal hearing and with SNHL, with and without vestibular dysfunction. METHODS: Cross-sectional study that evaluated 130 children (65 with normal hearing and 65 with SNHL), of both sexes and aged between 7 and 11 years, from public schools of the city of Caruaru, Pernambuco state, Brazil. The postural sway velocity of the center of pressure (COP) was assessed by a force platform, in two directions, anteroposterior (AP) and mediolateral (ML)), in three positions, namely bipedal support with feet together and parallel (parallel feet (PF)), bipedal support with one foot in front of the other (tandem foot (TF)), and single-leg support (one foot (OF)), evaluated with the eyes open and closed. RESULTS: Children with SNHL demonstrated greater postural sway velocity compared to children with normal hearing in all the positions evaluated, with significant differences in the AP direction, with the eyes open (PF: p = 0.001; TF: p = 0.000; OF: p = 0.003) and closed (PF: p = 0.050; TF: p = 0.005). The same occurred in the ML direction, with the eyes open (PF: p = 0.001; TF: p = 0.000; OF: p = 0.001) and closed (PF: p = 0.002; TF: p = 0.000). The same occurred in relation to vestibular function, where the children with SNHL with an associated vestibular dysfunction demonstrated greater postural sway velocity compared to children with normal hearing in all the positions evaluated, demonstrating significant differences in the AP direction, with the eyes open (TF: p = 0.001; OF: p = 0.029) and eyes closed (PF: p = 0.036; TF: p = 0.033). The same occurred in the ML direction, with the eyes open (TF: p = 0.000) and with the eyes closed (PF: p = 0.008; TF: p = 0.009). CONCLUSIONS: Children with SNHL demonstrated greater instability of postural control than children with normal hearing in all the directions assessed. Children with SNHL and an associated vestibular dysfunction demonstrated the greatest instability of postural control in this study.

Automated hearing loss type classification based on pure tone audiometry data.

Hearing problems are commonly diagnosed with the use of tonal audiometry, which measures a patient's hearing threshold in both air and bone conduction at various frequencies. Results of audiometry tests, usually represented graphically in the form of an audiogram, need to be interpreted by a professional audiologist in order to determine the exact type of hearing loss and administer proper treatment. However, the small number of professionals in the field can severely delay proper diagnosis. The presented work proposes a neural network solution for classification of tonal audiometry data. The solution, based on the Bidirectional Long Short-Term Memory architecture, has been devised and evaluated for classifying audiometry results into four classes, representing normal hearing, conductive hearing loss, mixed hearing loss, and sensorineural hearing loss. The network was trained using 15,046 test results analysed and categorised by professional audiologists. The proposed model achieves 99.33% classification accuracy on datasets outside of training. In clinical application, the model allows general practitioners to independently classify tonal audiometry results for patient referral. In addition, the proposed solution provides audiologists and otolaryngologists with access to an AI decision support system that has the potential to reduce their burden, improve diagnostic accuracy, and minimise human error.

Vestibular evaluation with video head impulse test in pediatric cochlear implant patients.

BACKGROUND: The cochlear implant (CI) is effective for rehabilitating patients with severe to profound sensorineural hearing loss. However, its placement and use have been associated with various complications, such as those affecting the vestibular system. The objective of this study was to compare vestibular function using the video head impulse test (vHIT) in pediatric patients before and after CI placement. METHODS: A descriptive and retrospective study was conducted. The outcomes of 11 pediatric patients of both sexes with a history of profound hearing loss were evaluated. The results of vestibular-ocular reflex (VOR) gain, saccades, asymmetry, Pérez Rey (PR) index, and VOR/saccade ratio for both ears obtained by the vHIT test before and after CI placement were compared. RESULTS: Of the 11 patients evaluated, the VOR gain showed that 81.8% had normal function, 18.2% had hypofunction, and no patients had hyperfunction before implantation. No statistically significant differences were found when compared with post-implant off and post-implant on conditions (p > 0.05). The extracted variables, asymmetry, PR index, and the VOR/saccades ratio also showed no statistically significant differences between the pre- and post-implant conditions, whether off or on. CONCLUSIONS: The vestibular function of pediatric patients did not show significant changes before and after CI placement. The vHIT test is a valuable tool for assessing vestibular function and could be considered a criterion for surgical and rehabilitation decisions in patients undergoing CI placement.

INTRODUCCIÓN: El implante coclear es un dispositivo eficaz para la rehabilitación de pacientes con hipoacusia neurosensorial severa a profunda. Sin embargo, su colocación y uso se ha asociado a diversas complicaciones, entre ellas a nivel del sistema vestibular. El objetivo del presente estudio fue comparar la función vestibular mediante la prueba de videoimpulso cefálico (vHIT) de pacientes pediátricos antes y después de la colocación del implante coclear. MÉTODOS: Se llevó a cabo un estudio descriptivo y retrospectivo. Se evaluaron los resultados de 11 pacientes pediátricos de ambos sexos con antecedente de hipoacusia profunda. Se compararon los resultados de ganancia del VOR, sacadas, asimetría, índice PR así como la relación VOR/sacadas para ambos oídos obtenidos mediante la prueba vHIT antes y después de la colocación del implante coclear. RESULTADOS: De los 11 pacientes evaluados, la ganancia del VOR mostró que el 81.8% tenía normofunción, 18.2% hipofunción y ningún paciente hiperfunción antes del implante. Al compararlo con la ganancia post implante apagado y post implante encendido no se encontraron diferencias estadísticamente significativas (p > 0.05). Las variables sacadas, asimetría, índice PR así como la relación VOR/sacadas tampoco mostraron diferencias estadísticamente significativas entre las condiciones pre y pos implante ya sea apagado o encendido. CONCLUSIONES: La función vestibular de pacientes pediátricos no mostró cambios significativos previo y posterior a la colocación del implante coclear. La prueba vHIT es una herramienta útil que permite evaluar la función vestibular y que podría considerarse como criterio para tomar decisiones quirúrgicas en pacientes que se encuentran en protocolo para implante coclear.

Associations between diabetes mellitus and sensorineural hearing loss from humans and animal studies.

There is controversy regarding the association and etiopathogenesis of diabetes mellitus (DM) and sensorineural hearing loss (SNHL). Some studies support that SNHL develops because of angiopathy and/or neuropathy caused by DM, but many of the findings have been inconsistent. This review aims to highlight a select number of studies that effectively describe the relationship between DM and SNHL, thus bringing more attention and awareness to this area of research. This review also describes animal models to understand better the mechanisms of DM contributing to SNHL in the inner ear. The goal of this narrative review is for researchers and healthcare professionals to further their understanding and investigation of the etiopathogenesis of both DM and SNHL, therefore leading to the development of effective treatments for diabetic patients displaying symptoms of SNHL.

Elderly people with hearing loss and cognitive decline: speech perception performance in noise. / Idosos com perda auditiva e declínio cognitivo: desempenho da percepção de fala no ruído.

PURPOSE: To verify the influence of verbal intellectual-cognitive skills on speech perception in noise, in elderly with sensorineural hearing loss, considering education, age, and degree of hearing loss. METHODS: 36 elderly between 60 and 89 years old with bilateral sensorineural hearing loss participated in the study. After psychological assessment using the Wechsler Intelligence Scale for Adults (WAIS-III), they were grouped into (GI) 24 elderly without cognitive alteration and (GII) 12 elderly with risk of cognitive alteration. They underwent otorhinolaryngological assessment, audiological interview, pure tone audiometry, and assessment of speech perception in noise using the Hearing in Noise Test (HINT-Brazil). The Mann-Whitney U statistical test compared the results between the groups, and the Spearman correlation verified the variable's age, degree of hearing loss, and level of education. RESULTS: There was no difference between the groups in the ability to perceive speech in noise, except in the noise on the left condition, in which GII showed better performance in HINT-Brazil. The degree of hearing loss and level of education influenced the perception of speech in noise. The level of education was correlated with the WAIS-III results. CONCLUSION: The decline in verbal intellectual-cognitive skills did not affect speech perception of noise in the elderly with hearing loss. The degree of hearing loss and level of education influenced the performance of the elderly in the speech perception test in noise. Performance in verbal cognitive skills varied according to the level of education.

OBJETIVO: Verificar a influência das habilidades intelectuais-cognitivas verbais na percepção de fala no ruído, em idosos com perda auditiva sensorioneural, considerando a escolaridade, a idade e o grau da perda auditiva. MÉTODO: Participaram 36 idosos entre 60 e 89 anos com perda auditiva sensorioneural bilateral, que após avaliação psicológica por meio do Wechsler Intelligence Scale for Adults (WAIS III), foram divididos em (GI) 24 idosos sem alteração cognitiva e (GII) 12 idosos com risco de alteração cognitiva. Foram submetidos à avaliação otorrinolaringológica, entrevista audiológica, audiometria tonal liminar e a avaliação da percepção de fala no ruído por meio do Hearing in Noise Test (HINT-Brasil). O teste estatístico U de Mann-Whitney comparou os resultados entre os grupos, e a correlação de Spearman verificou as variáveis idade, grau da perda auditiva e nível de escolaridade. RESULTADOS: Não houve diferença entre os grupos na habilidade de percepção de fala no ruído, exceto na condição ruído à esquerda, no qual o GII apresentou melhor desempenho no HINT-Brasil. O grau da perda auditiva e o nível de escolaridade influenciaram na percepção de fala no ruído. O nível de escolaridade teve correlação com os resultados do WAIS III. CONCLUSÃO: O declínio das habilidades intelectuais-cognitivas verbais não interferiu na percepção de fala no ruído nos idosos com perda auditiva. O grau da perda auditiva e o nível de escolaridade influenciaram no desempenho dos idosos no teste de percepção de fala no ruído. O desempenho nas habilidades cognitivas verbais variou com o nível de escolaridade.

A Randomized Crossover Study in Single-Sided Deafness Comparing a Cartilage Conduction CROS System and an Air-Conduction CROS System.

OBJECTIVE: To investigate if cartilage conduction (CC) rerouting devices are noninferior to air-conduction (AC) rerouting devices for single-sided deafness (SSD) patients by measuring objective and subjective performance using speech-in-noise tests that resemble a realistic hearing environment, sound localization tests, and standardized questionnaires. STUDY DESIGN: Prospective, single-subject randomized, crossover study. SETTING: Anechoic room inside a university. PATIENTS: Nine adults between 21 and 58 years of age with severe or profound unilateral sensorineural hearing loss. INTERVENTIONS: Patients' baseline hearing was assessed; they then used both the cartilage conduction contralateral routing of signals device (CC-CROS) and an air-conduction CROS hearing aid (AC-CROS). Patients wore each device for 2 weeks in a randomly assigned order. MAIN OUTCOME MEASURES: Three main outcome measures were 1) speech-in-noise tests, measuring speech reception thresholds; 2) proportion of correct sound localization responses; and 3) scores on the questionnaires, "Abbreviated Profile of Hearing Aid Benefit" (APHAB) and "Speech, Spatial, and Qualities of Hearing Scale" with 12 questions (SSQ-12). RESULTS: Speech reception threshold improved significantly when noise was ambient, and speech was presented from the front or the poor-ear side with both CC-CROS and AC-CROS. When speech was delivered from the better-ear side, AC-CROS significantly improved performance, whereas CC-CROS had no significant effect. Both devices mainly worsened sound localization, whereas the APHAB and SSQ-12 scores showed benefits. CONCLUSION: CC-CROS has noninferior hearing-in-noise performance except when the speech was presented to the better ear under ambient noise. Subjective measures showed that the patients realized the effectiveness of both devices.

Predictors of cochlear implant outcomes in pediatric auditory neuropathy: A matched case-control study.

OBJECTIVES: Current evidence supports the benefits of cochlear implants (CIs) in children with hearing loss, including those with auditory neuropathy spectrum disorder (ANSD). However, there is limited evidence regarding factors that hold predictive value for intervention outcomes. DESIGN: This retrospective case-control study consisted of 66 children with CIs, including 22 with ANSD and 44 with sensorineural hearing loss (SNHL) matched on sex, age, age at CI activation, and the length of follow-up with CIs (1:2 ratio). The case and control groups were compared in the results of five open-set speech perception tests, and a Forward Linear Regression Model was used to identify factors that can predict the post-CI outcomes. RESULTS: There was no significant difference in average scores between the two groups across five outcome measures, ranging from 88.40% to 95.65%. The correlation matrix revealed that younger ages at hearing aid fitting and CI activation positively influenced improvements in speech perception test scores. Furthermore, among the variables incorporated in the regression model, the duration of follow-up with CIs, age at CI activation, and the utilization of two CIs demonstrated prognostic significance for improved post-CI speech perception outcomes. CONCLUSIONS: Children with ANSD can achieve similar open-set speech perception outcomes as children with SNHL. A longer CI follow-up, a lower age at CI activation, and the use of two CIs are predictive for optimal CI outcome.

Comparison of vestibular function in hereditary hearing loss patients with GJB2, CDH23, and SLC26A4 variants.

To investigate the association between hereditary hearing loss and vestibular function, we compared vestibular function and symptoms among patients with GJB2, SLC26A4, and CDH23 variants. Thirty-nine patients with sensory neural hearing loss (11 males and 28 females) with biallelic pathogenic variants in either GJB2, SLC26A4, or CDH23 were included in this study (13 GJB2, 15 SLC26A4, and 11 CDH23). The patients were examined using caloric testing and cervical and ocular vestibular-evoked myogenic potentials (cVEMP and oVEMP). We also compared vestibular function and symptoms between patients with these gene variants and 78 normal-hearing ears without vestibular symptoms as controls. The frequency of semicircular canal hypofunction in caloric testing was higher in patients with SLC26A4 variants (47%) than in those with GJB2 (0%) and CDH23 variants (27%). According to the cVEMP results, 69% of patients with GJB2 variants had saccular hypofunction, a significantly higher proportion than in those carrying other variants (SLC26A4, 20%; CDH23, 18%). In oVEMP, which reflects utricular function, no difference was observed in the frequency of hypofunction among the three genes (GJB2, 15%; SLC26A4, 40%; and CDH23, 36%). Hence, discernable trends indicate vestibular dysfunction associated with each gene.

Neural Envelope Processing at Low Frequencies Predicts Speech Understanding of Children With Hearing Loss in Noise and Reverberation.

OBJECTIVE: Children with hearing loss experience greater difficulty understanding speech in the presence of noise and reverberation relative to their normal hearing peers despite provision of appropriate amplification. The fidelity of fundamental frequency of voice (f0) encoding-a salient temporal cue for understanding speech in noise-could play a significant role in explaining the variance in abilities among children. However, the nature of deficits in f0 encoding and its relationship with speech understanding are poorly understood. To this end, we evaluated the influence of frequency-specific f0 encoding on speech perception abilities of children with and without hearing loss in the presence of noise and/or reverberation. METHODS: In 14 school-aged children with sensorineural hearing loss fitted with hearing aids and 29 normal hearing peers, envelope following responses (EFRs) were elicited by the vowel /i/, modified to estimate f0 encoding in low (<1.1 kHz) and higher frequencies simultaneously. EFRs to /i/ were elicited in quiet, in the presence of speech-shaped noise at +5 dB signal to noise ratio, with simulated reverberation time of 0.62 sec, as well as both noise and reverberation. EFRs were recorded using single-channel electroencephalogram between the vertex and the nape while children watched a silent movie with captions. Speech discrimination accuracy was measured using the University of Western Ontario Distinctive Features Differences test in each of the four acoustic conditions. Stimuli for EFR recordings and speech discrimination were presented monaurally. RESULTS: Both groups of children demonstrated a frequency-dependent dichotomy in the disruption of f0 encoding, as reflected in EFR amplitude and phase coherence. Greater disruption (i.e., lower EFR amplitudes and phase coherence) was evident in EFRs elicited by low frequencies due to noise and greater disruption was evident in EFRs elicited by higher frequencies due to reverberation. Relative to normal hearing peers, children with hearing loss demonstrated: (a) greater disruption of f0 encoding at low frequencies, particularly in the presence of reverberation, and (b) a positive relationship between f0 encoding at low frequencies and speech discrimination in the hardest listening condition (i.e., when both noise and reverberation were present). CONCLUSIONS: Together, these results provide new evidence for the persistence of suprathreshold temporal processing deficits related to f0 encoding in children despite the provision of appropriate amplification to compensate for hearing loss. These objectively measurable deficits may underlie the greater difficulty experienced by children with hearing loss.

Machine learning-based longitudinal prediction for GJB2-related sensorineural hearing loss.

BACKGROUND: Recessive GJB2 variants, the most common genetic cause of hearing loss, may contribute to progressive sensorineural hearing loss (SNHL). The aim of this study is to build a realistic predictive model for GJB2-related SNHL using machine learning to enable personalized medical planning for timely intervention. METHOD: Patients with SNHL with confirmed biallelic GJB2 variants in a nationwide cohort between 2005 and 2022 were included. Different data preprocessing protocols and computational algorithms were combined to construct a prediction model. We randomly divided the dataset into training, validation, and test sets at a ratio of 72:8:20, and repeated this process ten times to obtain an average result. The performance of the models was evaluated using the mean absolute error (MAE), which refers to the discrepancy between the predicted and actual hearing thresholds. RESULTS: We enrolled 449 patients with 2184 audiograms available for deep learning analysis. SNHL progression was identified in all models and was independent of age, sex, and genotype. The average hearing progression rate was 0.61 dB HL per year. The best MAE for linear regression, multilayer perceptron, long short-term memory, and attention model were 4.42, 4.38, 4.34, and 4.76 dB HL, respectively. The long short-term memory model performed best with an average MAE of 4.34 dB HL and acceptable accuracy for up to 4 years. CONCLUSIONS: We have developed a prognostic model that uses machine learning to approximate realistic hearing progression in GJB2-related SNHL, allowing for the design of individualized medical plans, such as recommending the optimal follow-up interval for this population.

[Bilateral vestibulopathy and sensorineural hearing loss in necrobiotic xanthogranuloma, multiple myeloma and amiloidosis]. / Dvustoronnyaya vestibulopatiya i sensonevral'naya tugoukhost' u patsienta s nekroticheskoi ksantogranulemoi, mnozhestvennoi mielomoi i amiloidozom.

This article describes a rare case of necrotic xanthogranuloma in a 46-year-old patient who presented with the development of periorbital xanthelasms, progressive bilateral sensorineural hearing loss and bilateral vestibulopathy, followed by multiple myeloma and amyloidosis. For several years, the patient underwent standard rehabilitation for chronic sensorineural hearing loss and was fitted with a hearing aid. During hospitalisation for exacerbation of chronic bronchitis, monoclonal gammopathy was identified, and later, after careful examination and repeated biopsies, necrotic xanthogranuloma, multiple myeloma and AL-amyloidosis were confirmed. Targeted immunochemotherapy resulted in improvement of hearing and significant recovery of the vestibuloocular reflex bilaterally.

Speech perception outcomes in hearing-impaired individuals with microphone & receiver in the ear (M&RIE) hearing aids.

PURPOSE: The Microphone and Receiver in the ear (M&RIE) hearing aid is designed to consider the pinna cues by placing an additional microphone along with a standard receiver at the entrance of the ear canal. There is a need to clinically validate the technology using speech identification measures, as a literature shortage exists. The objective of the study was to evaluate speech identification and quality rating with hearing aids fitted with standard and M&RIE receivers. METHOD: Twenty individuals (mean ± SD age being 34.10 ± 11.9 years) with mild-to-moderately severe sensorineural hearing loss were divided into two groups: naïve and experienced. Their speech identification obtained using the receiver in the ear hearing aid compatible with standard and M&RIE receivers were compared. The study assessed speech identification in quiet using ten sentences, calculated SNR-50 using the Spearman-Karber equation, and used an 11-point scale for self-perceived quality rating. RESULTS: The study analyzed data using SPSS and found no significant difference between naive and experienced hearing aid users in quiet and SNR-50 conditions (p > 0.05). However, naive users rated higher in clarity, naturalness, pleasantness, and overall impression of standard receiver settings. Across different receiver settings, both naïve and experienced groups revealed better speech identification with the M&RIE receiver for both SIS (p < 0.05) and SNR-50 (p < 0.05) measures. CONCLUSION: The study found that placing a third microphone in the ear canal improves speech identification and quality rating in both naïve and experienced users, with pinna and ear canal resonance playing a crucial role in prescribing hearing aid gain.

Spatial Hearing in Children With and Without Hearing Loss: Where and What the Speech Is Matters for Local Speech Intelligibility.

PURPOSE: This study examined children's ability to perceive speech from multiple locations on the horizontal plane. Children with hearing loss were compared to normal-hearing peers while using amplification with and without advanced noise management. METHOD: Participants were 21 children with normal hearing (9-15 years) and 12 children with moderate symmetrical hearing loss (11-15 years). Word recognition, nonword detection, and word recall were assessed. Stimuli were presented randomly from multiple discrete locations in multitalker noise. Children with hearing loss were fit with devices having separate omnidirectional and noise management programs. The noise management feature is designed to preserve audibility in noise by rapidly analyzing input from all locations and reducing the noise management when speech is detected from locations around the hearing aid user. RESULTS: Significant effects of left/right and front/back lateralization occurred as well as effects of hearing loss and hearing aid noise management. Children with normal hearing experienced a left-side advantage for word recognition and a right-side advantage for nonword detection. Children with hearing loss demonstrated poorer performance overall on all tasks with better word recognition from the back, and word recall from the right, in the omnidirectional condition. With noise management, performance improved from the front compared to the back for all three tasks and from the right for word recognition and word recall. CONCLUSIONS: The shape of children's local speech intelligibility on the horizontal plane is not omnidirectional. It is task dependent and shaped further by hearing loss and hearing aid signal processing. Front/back shifts in children with hearing loss are consistent with the behavior of hearing aid noise management, while the right-side biases observed in both groups are consistent with the effects of specialized speech processing in the left hemisphere of the brain.