Audiology and Neuro-Otology最新文献

Introduction: Age-related hearing loss (ARHL) is a multifactorial disorder due to impairment in the peripheral or central auditory pathway (CAP). CAP impairment refers to difficulties in speech understanding, particularly in the presence of background noise, problems in discrimination, and integrating and separating binaural auditory information. Changes in CAP mechanisms, such as neurotransmitters or ion movement, may correlate with this impairment. The regulation of calcium (Ca2+) concentrations by Ca2+-binding proteins (CaBPs) is critical for synaptic function and plasticity, and changes are directly related to ARHL. Thus, changes in the neuronal expression of CaBPs in aging could be related to CAP disorders. Considering that supplementation with antioxidant and/or neuroprotective substances can delay neuronal loss in aging, antioxidant-based therapies can prevent and/or reduce the progression of ARHL in animal models and humans. The hormone melatonin has important antioxidant and neuroprotective actions, which can sometimes depend on melatonin receptors. This work aimed to investigate changes in the expression of CaBPs and melatonin receptors in the CAP stations and whether this expression could vary with aging.

Methods: Brain sections of Wistar rats young and elderly were processed using immunohistochemistry technique, and for analysis and quantification, the stereology and optical density technique were used.

Results: The analysis revealed a lower density of CaBP-immunoreactive cells in most CAP nuclei. In contrast, elderly rats showed increased expression of MT1 and MT2 in CAP stations.

Conclusion: These findings suggest that changes in neuronal number and transmission in these nuclei in aging are concomitant with increased melatonin's potential sites of action in the CAP.

Introduction: This study aimed to investigate differences between two precurved cochlear implant electrode arrays - Contour Advance (CA) and Slim Modiolar Electrode (SME) - in adult users, with a focus on electrically evoked compound action potential (ECAP) thresholds, 1-year postoperative C-levels, and battery consumption.

Methods: Data from 70 adult cochlear implant recipients (35 CA, 35 SME) were analyzed. Intra- and postoperative ECAP thresholds were obtained using Cochlear Custom Sound software. One-year C-levels were measured through psychophysical loudness scaling. Estimated battery runtimes were examined in relation to median C-levels. A linear mixed-effects model was used to assess differences across electrode positions and between arrays. Model validation was performed through examination of standardized residuals versus fitted values, QQ-plots analysis, and comparison of observed versus expected serial correlations.

Results: The CA array showed a more linear and more directly proportional relationship between ECAPs and C-levels. The SME array demonstrated significantly higher ECAPs and C-levels for the basal electrodes, larger pre- to postoperative changes in thresholds, and larger differences between ECAPs and C-levels, compared to the CA array. Battery runtime was significantly correlated with C-levels in the CA group, whereas impedance appeared more closely related to battery consumption in the SME group.

Conclusion: Electrode array design affects electrophysiological and behavioral responses as well as battery consumption. We found significant differences between two precurved electrode arrays from the same manufacturer. These findings highlight the importance of array-specific programming strategies and suggest that electrode characteristics should be considered in clinical decision-making and device optimization.

Introduction: The ADHEAR is a non-surgical bone conduction device that makes use of an adhesive adapter. While clinical trials have demonstrated its efficacy with regard to audiological performance, safety, and compliance, data on real-world paediatric cohorts are scarce.

Methods: This retrospective cohort study analysed data from paediatric patients fitted with ADHEAR at a tertiary centre between January 2017 and September 2024. Baseline characteristics such as hearing loss aetiology were recorded. Long-term free-field aided and unaided warble-tone audiometry was performed within a subsample of 19 patients. Compliance and reasons for discontinuation were recorded over a follow-up period of up to 78 months and based on the last recorded status.

Results: A total of 111 children (mean age: 10.1 years) were included. Mean functional gain was 22.7 (95% CI: 18.3-27.1) decibels hearing loss (dB HL) and the mean effective gain was 20.3 dB HL (95% CI: 20.3 [16.4, 24.2]). Skin complications were low and reported in 16 children (14.4%), of whom 7 needed to temporarily refrain from device use. Long-term compliance was 40.9% (median follow-up: 24 months). Key reasons for discontinuation included lack of perceived benefit (23.5%), appearance or social stigmatization issues (20.6%), and practical issues with adhesives (16.2%).

Conclusion: For congenital malformation and chronic suppurative otitis media patients, the ADHEAR provides effective amplification, although air-bone gap closure is suboptimal. Complications associated with device use were relatively rare and mild in nature. Compliance rates were notably lower than observed in other studies. Appearance and social stigmatization issues should be addressed during consultation.

Introduction: This study aimed to evaluate long-term auditory outcomes in patients with inner ear malformations (IEMs) treated with cochlear or auditory brainstem implants (CI/ABI) and assess the influence of anatomical subtype, electrode design, insertion depth, and genetic/syndromic background on hearing performance over a 10-year follow-up.

Methods: We conducted a prospective cohort study including patients with radiologically confirmed IEMs and bilateral severe-to-profound hearing loss, all of whom underwent implantation and completed at least 10 years of follow-up. Outcomes were assessed using pure-tone average (PTA) and speech recognition scores (SRSs) at defined intervals. Additional analyses explored the influence of electrode array design, insertion depth, genetic and syndromic diagnosis, and intraoperative complications.

Results: Eighty-two patients were included in the study, of whom 71 (86.58%) received a CI, while the remaining 11 patients (13.41%) were treated with an ABI. After a 10-year follow-up, the mean PTA gain with CI was 64.56 ± 14.52 dB, and mean SRS improved by 56.88 ± 25.32%. On the other hand, patients treated with ABI presented a gain of 45.00 ± 18.22 and 32.62 ± 10.67% dB, respectively. Enlarged vestibular aqueduct had the highest 10-year SRS (80.04%), whereas cochlear nerve agenesis, Michel deformity, and cochlear hypoplasia type I had the lowest (23.72%, 24.00%, and 26.50%, respectively). The mean insertion depth was 19.66 ± 3.74 mm and showed a non-significant correlation with final SRS (r = 0.243, p = 0.084). The presence of residual auditory brainstem responses was significantly associated with better pre-implant PTA (p = 0.041) and higher SRS at 10 years (p = 0.034). Finally, having a syndromic condition was not significantly associated with the risk of a worse SRS at 10 years post-implantation (p = 0.091), nor with poorer auditory outcomes measured in PTA prior to surgery (p = 0.315).

Conclusion: Auditory outcomes in IEMs are modulated by malformation subtype, neural status, and electrode design. A stratified, anatomy-guided approach is essential, especially when considering perimodiolar arrays. Delayed gains in some subtypes underscore the value of long-term follow-up, and integrating imaging and genetic data may enhance personalized implant strategies.

Background: Accurate intracochlear positioning of electrode arrays is critical for optimizing auditory outcomes during cochlear implantation (CI). While radiographic imaging remains the standard for postoperative evaluation, transimpedance matrix (TIM) measurements have emerged as a promising intraoperative technique for real-time assessment of electrode placement, particularly with a slim modiolar electrode array.

Methods: This retrospective observational study included 15 patients (24 ears) who underwent CI using CI532® and CI632® electrode arrays. Intraoperative TIM data were obtained and compared with intraoperative and immediate postoperative radiographic imaging data to determine the accuracy and reliability of TIM in detecting electrode placement anomalies. The electrode positioning patterns were analyzed using TIM heatmaps, with a focus on the voltage gradient distribution and identification of the electrode tip fold-over (TFO).

Results: Of the 24 CIs, 23 (96%) exhibited a regular TIM voltage distribution consistent with proper modiolar placement. A single instance of TFO (4%) was identified intraoperatively using TIM and confirmed via radiographic imaging. The electrode was reinserted following intraoperative correction with TIM and imaging, subsequently confirming the optimal placement. In all remaining cases, the TIM findings were in concordance with radiological assessments.

Conclusion: TIM may be a reliable intraoperative monitoring tool for CI using slim modiolar CI532® and CI632® electrode arrays position during CI. Their ability to accurately detect deviations in electrode configuration, such as the TFO, affirms their potential to augment surgical precision, eliminate the need of intraoperative radiation exposure by providing real-time feedback, minimize postoperative imaging requirements, and enhance patient outcomes. Further prospective studies with larger cohorts are warranted to validate these preliminary findings and establish standardized protocols for clinical implementation.

Introduction: Cochlear implantation (CI) is the standard treatment for severe-to-profound sensorineural hearing loss, but CI users often struggle with speech understanding in noisy environments. The Dutch/Flemish Matrix test is frequently used to evaluate speech-in-noise performance due to its assumed immunity to learning effects. However, studies challenge this assumption, revealing significant learning effects that can confound research outcomes. In this study, we modeled the learning curves of the Dutch/Flemish Matrix test to assess the influence of both between-session and between-test effects. We hypothesized that a exponential model would describe the learning effects more accurately than a linear model.

Methods: The perceptual learning effects associated with the Dutch/Flemish Matrix test were assessed in 17 bimodal CI users. All participants performed the Matrix speech-in-noise tests across four sessions, with 13 randomized tests per session. The tests were conducted in a soundproof booth with an eight-speaker babble noise. The outcome parameter was the speech recognition threshold and was analyzed with a linear mixed model to account for confounders.

Results: The results showed a statistically significant learning effect between sessions that added up to a speech intelligibility increase of 1.3 dB signal-to-noise ratio (SNR) (equivalent to ∼10% word score) between the first and second sessions, 0.86 dB SNR (∼7%) between the second and third sessions and 0.67 dB SNR (∼5%) between the third and fourth sessions. In addition, a statistically significant within-session learning effect (i.e., between tests) was observed with a linear slope of -0.11 dB SNR/test (∼0.9% word score/test), which accumulates to a total of 1.7 dB SNR (13%) between session start and end. The between-session learning curve was described more accurately with an exponential fit than with a linear fit. The between-test learning curve can be described equally well with a linear and an exponential fit.

Conclusion: A robust between-test learning effect was observed, which could be accurately modeled using either a linear or exponential learning curve. Additionally, a between-session learning effect was evident and was best described by an exponential learning curve. This study provides an important handle for correcting these learning effects in future studies.

Introduction: The reason why chronic tympanic membrane (TM) perforations fail to heal is an enigma. A better insight into the premises and cellular events in this process is fundamental. Previous large studies on human TMs are lacking. The main objective of the present study was to map potential stem cells in the human TM and to address the regenerative capacity in the TM keratinocytes. Further, the study aimed to assess the regenerative premises for three regions of the TM: the malleus, the annulus, and the unattached portion of the pars tensa (UPT), including different anatomical levels (inferior, intermediate, and superior) within each region.

Methods: Ten healthy human TMs were harvested during lateral skull base surgery. Using immunohistochemistry, the TMs were stained for α6 and β1 integrin, CK19, p63, and Ki-67. Semi-quantification was made with two single-blinded observers scoring the staining of the slides. Inter-rater reliability was evaluated with weighted Cohen's kappa, and group differences between the mean grading were further calculated.

Results: Antibodies for α6 and β1 integrin, CK19, p63, and Ki-67 could be detected along the malleus, annulus, and UPT of human TMs displaying diversity in the regenerative capacity of the human TM. The staining of Ki-67 and p63 was more prevalent in the annulus and malleus compared to in the UPT. α6 staining was more prevalent in the annulus, while CK19 staining was more prevalent in the malleus. Finally, several antibodies displayed a higher staining intensity in the inferior anatomical levels of the TM.

Conclusion: The present study shows the presence of putative stem cells in all regions of the human TM, with a stronger regenerative capacity close to the annulus and malleus compared to the UPT. These findings may set a baseline for the localization and diversity of putative stem cells in the human TM.

Introduction: Early pediatric conductive hearing loss intervention is crucial for ensuring optimal development outcomes. This study evaluated the impact of early access to bone conduction devices (BCDs) on children's quality of life (QoL) and academic performance, comparing results between those who received BCDs and those who did not.

Methods: We conducted a prospective cohort study involving children aged <12 years with congenital conductive hearing loss caused by external auditory canal atresia. The participants were categorized into three groups: those without BCDs, those using nonsurgical BCDs, and those implanted with active transcutaneous BCDs. QoL was assessed using the Arabic version of the Hearing Environments and Reflection on Quality of Life Measurement for Children Survey (HEAR-QL-AR) questionnaire at enrollment and during the last month of the study. School performance was evaluated based on annual academic results, grade repetition rates, and involvement in extracurricular activities. For children who were implanted with a piezoelectrive active transcutaneous bone conduction implant (OSI), data collection included age of implantation, duration of surgery, perioperative and long-term complications, and duration of device use.

Results: A total of 57 children participated in the study and were categorized into three groups: 14 in the non-BCDs group, 17 in the nonsurgical BCDs group, and 27 in the active transcutaneous BCDs group. At enrollment, the median HEAR-QL-AR scores were significantly higher in both BCD groups (80 and 81 in the nonsurgical and implanted groups, respectively) than in the non-BCD group (66, p = 0.013). After 6 months, the implanted group achieved the highest median HEAR-QL-AR score (90, IQR = 8), followed by the nonsurgical group (76, IQR = 14) and the non-BCD group (64, IQR = 16; p < 0.001). Academic performance was better in the BCD groups, with a median grade of "A" compared to "B" in the non-BCD group (p = 0.004). Participation in extracurricular activities was also higher in the implant group (median = 1.5) than in the nonsurgical (median = 1) and non-BCD groups (median = 0; p < 0.001). No surgical complications occurred, and the median surgical duration was 38 min (IQR = 7.5).

Conclusion: Early access to BCDs significantly improves QoL, academic performance, and social participation in children with conductive hearing loss. The implantation of active transcutaneous BCDs has been demonstrated to be both safe and effective, delivering superior auditory and developmental outcomes. These findings underscore the importance of advocating for timely intervention and expanding access to active transcutaneous BCDs for young children.

Introduction: Cochlear implantation has demonstrated benefit for restoring hearing in patients with Ménière's disease. We sought to examine disease and management factors that may influence postoperative speech performance and vertigo control.

Methods: A single-center retrospective chart review between 2010 and 2023 of patients with Ménière's disease receiving a cochlear implant (CI). The primary outcome was 1-year postoperative word recognition score on Consonant-Nucleus-Consonant (CNC) testing. Postoperative vertigo control was assessed as a secondary outcome. Variables including concurrent labyrinthectomy, pre- and postoperative Ménière's disease symptoms and treatments, duration of deafness, and hearing loss laterality were analyzed.

Results: Twenty-five patients were identified over the study period. Of those, 9 (36%) also underwent labyrinthectomy; 6 (67%) were performed simultaneously with CI and 3 (33%) received a CI following surgical ablation. There was a statistically significant (p = 0.03) higher rate of bilateral Ménière's disease in the CI-only cohort (n = 9, 56%), compared to the cochlear implant and labyrinthectomy (CI + L) cohort (n = 1, 11%). There was also a higher rate of preoperative uncontrolled vertigo (n = 5, 56%) in the CI + L cohort compared to the CI-only cohort (n = 3, 20%), although this did not reach statistical significance (p = 0.08). The average pre- and postoperative CNC score for the CI + L cohort was 3.6 (SD 5.9) and 36.7 (SD 17.5), respectively, and 7.1 (SD 10.1) and 62.1 (SD 14.3) for the CI-only group. There was a statistically significant difference noted at most recent CNC follow-up testing (p = 0.01) between the CI + L and CI-only group.

Conclusion: Patients with Ménière's disease meeting CI candidacy criteria may undergo CI safely and achieve speech performance benefit. A trend toward worse performance in patients who undergo concurrent labyrinthectomy with CI compared to CI alone was seen which warrants further study.

Introduction: Tinnitus and smell dysfunction have been reported to be associated. The long-term course of tinnitus and sensory dysfunctions, including olfactory and gustatory dysfunctions, following COVID-19 infection requires further study.

Materials and methods: A cohort study of tinnitus and sensory dysfunctions before and during the COVID-19 pandemic was conducted through health checkups in a rural area in Japan. Five hundred ten participants attended health checkups in August 2019, 434 in 2023, and 437 in 2024. Two hundred twenty-five participants attended the 2019 and 2023 health checkups, and one hundred seventy-one participants attended all three. The subjects included people 40 years of age or older. The questionnaire included information on tinnitus, hearing, vertigo, headache, smell, taste, and lifestyle habits. In 2023, the questionnaire included questions about COVID-19.

Results: Tinnitus, hearing impairment, and smell dysfunction were associated. Fear and anxiety about COVID-19 infection were significantly associated with tinnitus after adjusting for age and sex. Irregular sleep time and fewer sports and exercise habits were significantly associated with tinnitus, not smell or taste dysfunction. In an investigation of identical persons, deterioration of tinnitus was conspicuous in 2023 compared to 2019 in persons with strong fear and anxiety about COVID-19.

Conclusions: Tinnitus is related to lifestyle habits compared to other sensory disorders. Fear and anxiety about COVID-19, rather than COVID-19 infection itself, significantly influenced tinnitus during the COVID-19 pandemic in rural areas.