Unilateral Hearing Loss Research Articles

Intra Operative Middle Ear Surprise Presenting with Unilateral Conductive Hearing Loss in an Intact Ear Drum - An Interesting Case Report

Intra Operative Middle Ear Surprise Presenting with Unilateral Conductive Hearing Loss in an Intact Ear Drum - An Interesting Case Report

Localization and Speech-in-Noise Performance in a Virtual Reality Test Environment: A Pilot Study of Adults With Single-Sided Deafness Using a Cochlear Implant.

The purpose of this exploratory study was to (a) construct a virtual reality (VR) test environment to measure speech recognition in noise (SIN) and localization, and (b) use the VR test environment to establish degree of binaural hearing benefit among a small number of adults with single-sided deafness (SSD) using a cochlear implant (CI). This pilot study included five adults implanted for SSD. The test environment was composed of an eight-speaker array that delivered restaurant noise and Institute of Electrical and Electronics Engineers sentences. VR head-mounted display goggles delivered a video recording of a busy restaurant. Participants completed SIN and localization in two conditions: (a) normal-hearing ear and a CI on the contralateral SSD side (CI-ON) and (b) normal-hearing ear and unaided on the contralateral SSD side (CI-OFF). Overall, CI benefits for SIN and localization within the VR test environment were improved for some participants, although not all. CI benefit for SIN and localization was dependent on speaker location. VR test environments present new opportunities for studying SIN and localization abilities in participants with CIs. This pilot study shows that, within a VR test environment, degree of CI benefit among SSD participants for SIN and localization varies across speaker location and across participants.

Auditory Localization Performance in Cochlear Implant Recipients With Single-Sided Deafness: The Challenges and Limitations of Current Outcome Metrics.

Acoustic localization accuracy metrics currently employed in clinical literature both overestimate and underestimate performance benefit of cochlear implantation (CI) for single-sided deafness (SSD). Although localization in SSD with CI has been investigated, performance characterization has relied heavily on average error. Although attractively concise, this measure may misrepresent performance. Here, we characterize frequency-specific localization on a granular level in subjects with CI for SSD as a critical analysis of localization outcome metrics. Eight CI recipients with SSD were recruited. Stimuli of broadband (BBN) and narrowband noise (NBN) at low (500 Hz), mid (1000 Hz), and high (4000 Hz) frequencies were presented in a semianechoic chamber. Localization accuracy was quantified in mean angular error (MAE) and linear regression slope. Use of a CI for SSD subjects improved localization performance by slope for all stimuli ( p ≤ 0.0033) to a level that was equal to normal-hearing controls at 1 and 4 kHz ( p ≥ 0.2281). MAE was also significantly improved for SSD subjects using CI for BBN stimuli ( p ≪ 0.0001); however, no statistically significant improvement in MAE was seen for NBN ( p ≥ 0.5773) with CI use. Descriptive analysis of individual subject performance highlights the reasons for contradictory results. There is inherent challenge in characterizing localization benefit for individuals with CI for SSD. Our data demonstrate the limitations of utilization of average error as the sole metric for outcome benefit. We emphasize the importance of continued research investigating alternative outcome measures as we work toward a more refined understanding of the potential benefits and limitations of cochlear implantation for SSD.

Sound Localization in Active Transcutaneous Bone Conduction Implant Users with Single-Sided Deafness.

Evaluate sound localization accuracy of subjects with single-sided deafness (SSD) with active transcutaneous bone conduction implants (atBCIs). Prospective case-control study. Tertiary referral center. Ten SSD patients (with ATBCIS) and 10 controls. Localization was assessed in a semianechoic chamber using a 24-speaker array. Stimuli included broadband noise (BBN) and narrowband noise (NBN). Perceived stimulus angle was recorded and compared with presented location. Statistical analyses were performed using ANOVA and Wilcoxon rank sum tests. The primary outcome measures were as follows: 1) mean angular error (MAE) error (°) and regression slope and 2) subjective benefit assessment (Speech Spatial Qualities questionnaire). Subjects with SSD demonstrated worse localization by MAE and regression slope compared with controls for both broadband noise (p < 0.0001) and narrowband noise at 500 Hz and 1000 kHz (p < 0.0001). There was no statistically significant difference (p = 0.1090) in slope between all groups at 4000 Hz. There was no significant difference in slope or MAE aided compared with unaided. Localization ability varied widely within the SSD cohort, with some individuals showing some ability in the unaided condition, best at 4000 Hz. Although SSQ confirmed particular difficulty in the spatial hearing domain, all domains improved with device use. Localization ability for individuals with SSD falls into a somewhat bimodal distribution. Some have fair localization, particularly at high frequencies, that is preserved but not improved with the atBCI. Others have minimal to no localization ability at any frequency, with no apparent device benefit.

From the expert opinion practice: Cochlear implant (CI) versus CROS fitting for single-sided deafness (SSD)

From the expert opinion practice: Cochlear implant (CI) versus CROS fitting for single-sided deafness (SSD)

Single-Sided Deafness: A Narrative Review

Background and Aims: Single-sided deafness (SSD) is a severe type of hearing loss affecting one ear, and it frequently presents social difficulties for those affected. Head shadow effect and sound localisation play an important role in speech perception. Methods: Research articles on SSD were identified through an online search of PubMed, Scopus, Medline, and Google Scholar databases. Results: SSD affects daily activities and emotional or social well-being. It is necessary to get the early identification of the aetiology of SSD for prompt intervention. The aetiology of SSD can be determined with the use of investigations such as as genetic testing, serological testing, computed tomography (CT) scan, magnetic resonance imaging, and DNA testing for viral infections. Rehabilitation options for patients with SSD include cochlear implants, bone-anchored hearing aids, and contralateral routing of sound. Individuals with SSD cochlear implants have strong auditory perception. The objective of this review article is to provide aetiology, clinical presentations, diagnosis and current treatment of SSD. Conclusion: Individuals with SSD commonly experience communication issues that have a substantial negative effect on their quality of life. Word discrimination is hampered by SSD, and this impairment varies depending on the affected ear’s residual hearing. Speech comprehension is frequently difficult for patients with severe SSD, especially in noisy environments.

Enhancing Liver Disease Detection and Management with Advanced Machine Learning Models

The prevalence of hearing loss has risen making it a significant public health issue. Hearing loss is caused by complicated pathophysiological pathways, with various risk factors identified, such as hereditary factors, inflammatory processes, systemic disorders, noise exposure, medicines, oxidative stress, and age. Metabolic syndrome is a medical condition characterized by the presence of hypertension, central obesity, hyperlipidemia, and diabetes. Metabolic syndrome has been linked to several clinical diseases, such as stroke, heart attack, cardiovascular disease-related death, and diabetes. A cross-sectional study was done on 100 patients with metabolic syndrome which used specific cut-off points of waist circumference, fasting glucose levels, blood pressure, triglyceride, and high-density lipoprotein cholesterol levels to diagnose the condition. Among these five criteria, at least three had to be met, and the presence of additional criteria indicated greater severity. Audiological evaluation with pure tone audiometry was done and recorded. Statistical analysis was performed to determine the significance of the results. The majority of the patients (62%) had unilateral hearing loss, amongst which sensory-neural type and moderately severe hearing loss were the most common type (67%) and severity (61%) of hearing loss respectively. Chi-square tests were done for the comparison of type, severity, and laterality of hearing loss with age, gender of the patients, and criteria fulfilled for metabolic syndrome. The severity of hearing loss had a statistically significant association with the age of the patients and the number of criteria fulfilled for metabolic syndrome with a p-value of 0.003. There was a statistically significant association between the severity of hearing loss and the age of the patients and the number of criteria fulfilled for metabolic syndrome with a p-value of 0.004. Metabolic syndrome affects the auditory system in several ways. It damages hearing and exacerbates presbycusis. Hearing loss worsens as components of the metabolic syndrome increase.

Benefits of Cochlear Implantation for Older Adults With Asymmetric Hearing Loss.

FDA-approved indications for cochlear implantation include patients with severe-to-profound unilateral hearing loss (UHL) or asymmetric hearing loss (AHL); however, these indications are not covered for Medicare beneficiaries. We assessed the outcomes of cochlear implant (CI) use for older adults with UHL or AHL. Eighteen older adults (≥65 years of age at surgery) with UHL/AHL participated in a prospective, longitudinal investigation evaluating outcomes of CI use. Speech recognition for the affected ear was evaluated with consonant-nucleus-consonant (CNC) words. Spatial hearing was assessed with measures of sound source localization and sentence recognition in noise. The target sentence was presented from the front and the masker was either co-located with the target (SoNo), presented toward the affected ear (SoNci) or contralateral ear (SoNcontra). Perceived benefit was assessed with the Speech, Spatial, and Qualities of Hearing scale (SSQ) and the Tinnitus Handicap Inventory (THI). Participants experienced significant improvements with CI use for CNC words (mean [SD]; preop: 8% [10%], 1 yr: 51% [22%], 5 yr: 50% [19%]), masked sentence recognition (SoNcontra preop: 5% [6%], 1 yr: 22% [15%], 5 yr: 41% [14%]), and localization (preop: 76° [18°], 1 yr: 40° [11°], 5 yr: 41° [14°]),and reported significant improvements in hearing abilities (SSQ Spatial Hearing preop: 3 [1], 1 yr: 6 [2], 5 yr: 6 [2]) and tinnitus severity (THI preop: 16 [18], 1 yr: 4 [14], 5 yr: 6 [12]). Older adults with UHL/AHL experience significant improvements in speech recognition, spatial hearing, and subjective perceptions (e.g., hearing abilities and tinnitus severity) with a CI as compared to pre-operative abilities. IV Laryngoscope, 2024.

Do Otologists and Other Otolaryngologists Manage Single-Sided Deafness Differently?

The aim of this study was to survey the knowledge and treatment management practices for single-sided deafness (SSD) among different subspecialties of otolaryngology. A questionnaire was sent via Google Sheets to members of the Turkish and Egyptian Otorhinolaryngology Societies between December 2021 and February 2022. For the statistical analysis, the respondents were divided into 3 groups as otologists, non-otologists, and residents at the department of otolaryngology-head and neck department. There were no statistically significant differences between otologists and non-otologists in radiological imaging (child P = .469, adult P = .140) and preferred treatment method (child P = .546, adult P = .106). However, otolaryngologists showed significant differences in radiological evaluation (P <.001), vestibular evaluation (P = .000), and frequency of treatment options recommended for pediatric and adult SSD patients (P = .000). There were no significant differences in SSD diagnosis, treatment, and rehabilitation between otologists and non-otologists. However, when comparing pediatric and adult patients, there was a difference in the treatment management of SSD patients.

Unilateral Hearing Loss and Auditory Asymmetry in Mitochondrial Disease: A Scoping Review.

Background/Objectives: Mitochondrial transfer RNA mutations are one of the most important causes of hereditary hearing loss in humans. In most cases, its presentation is bilateral and symmetrical; however, there are numerous cases of single-sided presentation or asymmetrical onset described in the literature that may represent a diagnostic challenge. The aim of this review is to present the evidence of auditory asymmetry in mitochondrial diseases, highlighting the possible presence of cases with atypical presentation. Methods: A review of the English literature to date on hearing loss and mitochondrial diseases was performed using PubMed, Scopus, and Google Scholar databases. The literature review was performed using the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) guidelines for scoping review. Results: A total of 10 full-text articles were included in this review, comprising 25 patients with single-sided or asymmetrical hearing loss associated with mitochondrial disease. Conclusions: Sensorineural hearing loss due to mitochondrial disease can represent a complex diagnostic challenge in cases of asymmetric or unilateral presentation. It is critical to recognize this clinical variant and to diagnose it in daily clinical practice.

P1-N1-P2 Cortical Auditory Evoked Potentials in Chronic Unilateral Acquired Conductive Hearing Loss in Adults.

Chronic unilateral hearing loss causes imbalanced auditory input to the brain that triggers cortical reorganization. The effect of sensorineural hearing loss on the central auditory system (CAS) has been thoroughly studied, while there is a paucity of research on the effect of conductive hearing loss (CHL). The aim of this study was to assess the P1-N1-P2 cortical auditory evoked response potential (CAEP) in adult individuals with chronic acquired unilateral CHL. This study included 108 participants of both genders: 54 patients with unilateral chronic CHL who were compared to well-matched 54 controls. All were subjected to history-taking, otologic examination, basic audiological evaluation, and bone conduction N1-P2 CAEP. The affected ears of the cases showed highly statistically significant shorter CAEPs N1, P2, N1-P2 latencies but not P1, and showed highly statistically significant larger N1, P2, N1P2, amplitude than the control group. Latencies decreased and amplitudes increased as the degree of CHL increased, but were not affected by patients' age, side, or duration of the CHL. Cases with tinnitus had statistically significant and worse results than those without tinnitus. Unilateral chronic CHL might enhance neurocortical plasticity, with greater changes occurring at greater degrees of the CHL.

P1 and N1 Characteristics in Individuals with Normal Hearing and Hearing Loss, and Cochlear Implant Users: A Pilot Study.

Background: It has been reported in many previous studies that the lack of auditory input due to hearing loss (HL) can induce changes in the brain. However, most of these studies have focused on individuals with pre-lingual HL and have predominantly compared the characteristics of those with normal hearing (NH) to cochlear implant (CI) users in children. This study examined the visual and auditory evoked potential characteristics in NH listeners, individuals with bilateral HL, and CI users, including those with single-sided deafness. Methods: A total of sixteen participants (seven NH listeners, four individuals with bilateral sensorineural HL, and five CI users) completed speech testing in quiet and noise and evoked potential testing. For speech testing, the Korean version of the Hearing in Noise Test was used to assess individuals' speech understanding ability in quiet and in noise (noise from the front, +90 degrees, and -90 degrees). For evoked potential testing, visual and auditory (1000 Hz, /ba/, and /da/) evoked potentials were measured. Results: The results showed that CI users understood speech better than those with HL in all conditions except for the noise from +90 and -90 degrees. In the CI group, a decrease in P1 amplitudes was noted across all channels after implantation. The NH group exhibited the highest amplitudes, followed by the HL group, with the CI group (post-CI) showing the lowest amplitudes. In terms of auditory evoked potentials, the smallest amplitude was observed in the pre-CI condition regardless of the type of stimulus. Conclusions: To the best of our knowledge, this is the first study that examined visual and auditory evoked potentials based on various hearing profiles. The characteristics of evoked potentials varied across participant groups, and further studies with CI users are necessary, as there are significant challenges in collecting and analyzing evoked potentials due to artifact issues on the CI side.

Speech Recognition and Subjective Hearing Abilities for Electric-Acoustic Stimulation Users With Unilateral Hearing Loss.

Some cochlear implant (CI) recipients with unilateral hearing loss (UHL) have functional acoustic hearing in the implanted ear, warranting the fitting of an ipsilateral electric-acoustic stimulation (EAS) device. The present study assessed speech recognition and subjective hearing abilities over time for EAS users with UHL. Prospective, repeated-measures. Tertiary referral center. Adult CI recipients with normal-to-moderate low-frequency acoustic thresholds in the implanted ear and a contralateral pure-tone average (0.5, 1, and 2 kHz) ≤25 dB HL. Participants were evaluated preoperatively and at 1, 3, and 6 months post-activation. Speech recognition for the affected ear was evaluated with CNC words in quiet. Masked speech recognition in the bilateral condition was evaluated with AzBio sentences in a 10-talker masker (0 dB SNR) for three spatial configurations: target from the front and masker either colocated with the target or presented 90 degrees toward the affected or contralateral ear. Responses to the Speech, Spatial, and Qualities of Hearing Scale subscales were used to assess subjective hearing abilities. Participants experienced significant improvements in CNC scores ( F(3,13) = 14.90, p < 0.001), and masked speech recognition in the colocated ( F(3,11) = 3.79, p = 0.043) and masker toward the contralateral ear ( F(3,11) = 4.75, p = 0.023) configurations. They also reported significantly better abilities on the Speech Hearing ( F(3,13) = 5.19, p = 0.014) and Spatial Hearing ( F(3,13) = 10.22, p = 0.001) subscales. Adults with UHL and functional acoustic hearing in the implanted ear experience significant improvements in speech recognition and subjective hearing abilities within the initial months of EAS use as compared with preoperative performance and perceptions.

Sound-seeking before and after hearing loss in mice

How we move our bodies affects how we perceive sound. For instance, head movements help us to better localize the source of a sound and to compensate for asymmetric hearing loss. However, many auditory experiments are designed to restrict head and body movements. To study the role of movement in hearing, we developed a behavioral task called sound-seeking that rewarded freely moving mice for tracking down an ongoing sound source. Over the course of learning, mice more efficiently navigated to the sound. Next, we asked how sound-seeking was affected by hearing loss induced by surgical removal of the malleus from the middle ear. After bilateral hearing loss sound-seeking performance drastically declined and did not recover. In striking contrast, after unilateral hearing loss mice were only transiently impaired and then recovered their sound-seek ability over about a week. Throughout recovery, unilateral mice increasingly relied on a movement strategy of sequentially checking potential locations for the sound source. In contrast, the startle reflex (an innate auditory behavior) was preserved after unilateral hearing loss and abolished by bilateral hearing loss without recovery over time. In sum, mice compensate with body movement for permanent unilateral damage to the peripheral auditory system. Looking forward, this paradigm provides an opportunity to examine how movement enhances perception and enables resilient adaptation to sensory disorders.

Factors Influencing Pediatric Cochlear Implant Use.

Cochlear implant device use, quantified by hearing hours percentage (HHP), is a known variable that impacts pediatric spoken language outcomes. Isolating specific factors that impact HHP could help clinicians intervene to reduce the implications of barriers and amplify the positive facets. The aim of this study is to identify variables that predict HHP in children. A retrospective chart review was completed using data collected from 2019 to 2023. Subjects were included if they were under the age of 18 years at the time of data collection and had data logging recorded in the clinical patient database. A mixed-effects model weighed the influence of year of the clinical visit (2019, 2020, 2021, 2022, and 2023), race/ethnicity (White, African American, Asian, Hispanic, Mixed Race, or Other), listener type (bilateral simultaneous, sequential, bimodal, unilateral hearing loss, or unilateral listener; one cochlear implant and a contralateral deaf ear), insurance type (private, Medicaid, or military, or none), age at surgery, presence of autism spectrum disorder (ASD) or an intellectual development delay (IDD), and age at test on HHP. There were a total of 5,106 data points from 958 subjects. The mean HHP of the cohort was 64.2% (SD = 26.94%). Lower HHP was associated with the presence of IDD or ASD, use of Medicaid, and older age at surgery. HHP increased with age. Subjects of color did not have a significantly different HHP than those who were White. There was an interaction between year of data collection and listener type. Each listener type's HHP was impacted differently by the year of data collection; however, years of the COVID-19 pandemic yielded lower HHP for all listener types. The group mean of 64.9% is lower than the recommended 80% HHP goal, indicating that pediatric cochlear implant recipients have slightly more than half the access to sound as their age-matched typically hearing peers. Several variables that impact HHP were identified in this study. Cochlear implant teams can utilize these data to support vulnerable patients to increase HHP. Additional investigation is needed to determine what interventions most effectively improve HHP.

Parent-Reported Ease of Listening in Preschool-Aged Children With Bilateral and Unilateral Hearing Loss.

Evidence from school-aged children suggests that the ease with which children listen varies with the presence of hearing loss and the acoustic environment despite the use of devices like hearing aids. However, little is known about the ease of listening in preschool-aged children with hearing loss-an age at which rapid learning occurs and increased listening difficulty or effort may diminish the required capacity to learn new skills. To this end, the objectives of the present study were to (i) assess parent-reported aided ease of listening as a function of hearing loss configuration (hearing loss in one versus both ears) and device configuration among children with hearing loss in one ear (unilateral hearing loss), and (ii) investigate factors that influence children's ease of listening. Parents of 83 children with normal hearing, 54 aided children with bilateral hearing loss (hearing loss in both ears), and 139 children with unilateral hearing loss participated in the study. Of the 139 children with unilateral loss, 72 were unaided, 54 were aided with a device on the ear with hearing loss (direct aiding) and 13 were aided with a device that routed signals to the contralateral normal hearing ear (indirect aiding). Mean age of children was 40.2 months (1 SD = 2.5; range: 36 to 51). Parents completed the two subscales of the Parents' Evaluation of Aural/Oral Performance of Children+ (PEACH+) questionnaire, namely functional listening and ease of listening. Individual percent scores were computed for quiet and noisy situations. Linear mixed-effects models were used to assess the effect of hearing loss configuration and device configuration in children with unilateral hearing loss. Multiple regression was used to assess factors that influenced ease of listening. Factors included hearing thresholds, age at first device fit, consistency in device use, condition (quiet/noise), presence of developmental disabilities, and functional listening abilities. Children with direct aiding for their hearing loss, either unilateral or bilateral, had similarly lower functional listening skills and ease of listening than their normal hearing peers. Unaided children with unilateral hearing loss had lower functional listening skills and ease of listening than their normal hearing peers in noise but not in quiet. All aided children with unilateral hearing loss, irrespective of direct or indirect aiding had lower functional listening skills and ease of listening relative to normal hearing children in both quiet and noise. Furthermore, relative to unaided children with unilateral hearing loss, those with indirect aiding had lower functional listening and ease of listening. Regression analyses revealed functional listening as a significant predictor of ease of listening in all children with hearing loss. In addition, worse degrees of hearing loss and presence of noise reduced ease of listening in unaided children with unilateral hearing loss. Bilateral hearing loss is associated with poorer-than-typical ease of listening in preschoolers even when aided. The impact of unilateral hearing loss on ease of listening is similar to that observed in children with bilateral hearing loss, despite good hearing in one ear and aiding. Given increased difficulties experienced by children with unilateral loss, with or without a device, additional strategies to facilitate communication abilities in noise should be a priority.

Speech Quality Perception in Unilateral Cochlear Implant Users With Single-Sided Deafness.

Cochlear implant (CI) users frequently complain about speech quality perception (SQP). In patients undergoing cochlear implantation for single-sided deafness, there is concern that poor SQP from the implanted ear will negatively impact binaural (CI + normal hearing [NH]) SQP. In this study, we investigate if binaural SQP is measurably different than unimplanted NH alone. Cross-sectional study. Tertiary care center. Fifteen unilateral CI users with NH in the contralateral ear completed the validated Columbia Speech Quality Instrument. This instrument consists of 9 audio clips rated across 14 specific speech qualities using a 10-point visual analog scale. SQP was assessed in 3 conditions: CI only, NH only, and CI + NH. Median speech quality scores were worse in the CI only condition compared to the NH only (50.0 vs 72.6, P = .0003) and binaural (50.0 vs 71.0, P = .007) conditions. Median speech quality scores were not significantly different between the NH only and binaural conditions (72.6 vs 71, P = .8). Compared to NH, CI speech quality sounded less clear, less natural, and more mechanical. Compared to NH, SQP is poorer with a CI alone. However, in contrast to expectation, there is no significant difference between NH and binaural SQP. This suggests poorer CI speech perception does not negatively impact binaural SQP in patients undergoing cochlear implantation for single-sided deafness.

Biomarkers of auditory cortical plasticity and development of binaural pathways in children with unilateral hearing loss using a hearing aid

Congenital or early-onset unilateral hearing loss (UHL) can disrupt the normal development of the auditory system. In extreme cases of UHL (i.e., single sided deafness), consistent cochlear implant use during sensitive periods resulted in cortical reorganization that partially reversed the detrimental effects of unilateral sensory deprivation. There is a gap in knowledge, however, regarding cortical plasticity i.e. the brain's capacity to adapt, reorganize, and develop binaural pathways in milder degrees of UHL rehabilitated by a hearing aid (HA). The current study was set to investigate early-stage cortical processing and electrophysiological manifestations of binaural processing by means of cortical auditory evoked potentials (CAEPs) to speech sounds, in children with moderate to severe-to-profound UHL using a HA. Fourteen children with UHL (CHwUHL), 6-14 years old consistently using a HA for 3.5 (±2.3) years participated in the study. CAEPs were elicited to the speech sounds /m/, /g/, and /t/ in three listening conditions: monaural [Normal hearing (NH), HA], and bilateral [BI (NH + HA)]. Results indicated age-appropriate CAEP morphology in the NH and BI listening conditions in all children. In the HA listening condition: (1) CAEPs showed similar morphology to that found in the NH listening condition, however, the mature morphology observed in older children in the NH listening condition was not evident; (2) P1 was elicited in all but two children with severe-to-profound hearing loss, to at least one speech stimuli, indicating effective audibility; (3) A significant mismatch in timing and synchrony between the NH and HA ear was found; (4) P1 was sensitive to the acoustic features of the eliciting stimulus and to the amplification characteristics of the HA. Finally, a cortical binaural interaction component (BIC) was derived in most children. In conclusion, the current study provides first-time evidence for cortical plasticity and partial reversal of the detrimental effects of moderate to severe-to-profound UHL rehabilitated by a HA. The derivation of a cortical biomarker of binaural processing implies that functional binaural pathways can develop when sufficient auditory input is provided to the affected ear. CAEPs may thus serve as a clinical tool for assessing, monitoring, and managing CHwUHL using a HA.

Elevated modiolus density on high-resolution computed tomography as an indicator of cochlear neurodysplasia: a retrospective case-control study.

Cochlear neurodysplasia (CND) is recognized as a contributing factor to sensorineural hearing loss in children. This study aimed to investigate the relationship between modiolus density on high-resolution computed tomography (HRCT) and CND, and to evaluate its performance in diagnosing CND. This retrospective study collected HRCT images of 34 patients diagnosed with unilateral neurological hearing loss in the Children's Hospital of Chongqing Medical University from March 2018 to December 2023, who were also diagnosed with unilateral CND by computed tomography (CT) and magnetic resonance imaging (MRI) hydroimaging. CT values of the modiolus and petrous bone were measured on the affected and healthy sides, in addition to determining the width of cochlear nerve foramina and the width of internal auditory tract. The receiver operator characteristic (ROC) curve was used to evaluate the diagnostic performance of these features. Simultaneously, comparisons were conducted with parameters obtained from normal children. A total of 29 patients without CND were randomly selected as a control group. The unilateral sensorineural hearing loss group had 34 patients, comprising 18 males and 16 females, with a median age of 4.5 years, ranging from 0.7 to 11 years. The normal children group consisted of 20 males and 9 females, with a median age of 5.9 years, ranging from 0.5 to 12.0 years. Statistically significant differences were observed in the CT values of the modiolus, modiolus/petrous bone CT value ratio, width of cochlear nerve foramina, and width of internal auditory tract between the affected and healthy sides in patients with unilateral sensorineural hearing loss (P<0.05). The area under the ROC curve (AUC) of the modiolus CT value and the width of cochlear nerve foramina for the diagnosis of unilateral sensorineural hearing loss was 0.98 [95% confidence interval (CI): 0.95-1.00] and 0.99 (95% CI: 0.98-1.00), respectively. the modiolus density was significantly elevated in the affected sides in patients with unilateral CND. The optimal cut-off value of modiolus CT values was 983 Hounsfield unit (HU). The elevated density of the modiolus on HRCT holds significant value in diagnosing CND.

Early Audiometric Intervention in Bacterial Meningitis: Cochlear Implantation in a 10-Week-Old Child.

Sensorineural hearing loss is a well-known complication of Streptococcus pneumoniae meningitis. Given the propensity for fibrosis and ossification of the cochlea in bacterial meningitis, implantation must be performed in a timely fashion because a delayed attempt at implantation can frustrate obtaining an optimal technical result or lead to an inability to implant. Obtaining optimal audiometric outcomes is reliant on early hearing screening in patients with streptococcal meningitis. In the absence of standardized protocols, audiometric testing is often overlooked or delayed in the workup and management of meningitis. Our institution implemented a meningitis protocol with a particular focus on timing of audiometric testing in patients with meningitis. We present a patient diagnosed with streptococcal meningitis in the first week of life. Early hearing screening allowed the diagnosis of profound unilateral sensorineural hearing loss and subsequent cochlear implantation at 10 weeks of age, the youngest described in the medical literature. Despite early implantation, there was cochlear fibrosis at the time of implantation. Fortunately, the majority of electrodes were implanted to achieve a serviceable hearing outcome. Serial magnetic resonance imaging scans were obtained because of her contralateral ventriculoperitoneal shunt that allowed unique visualization of the progression of cochlear fibrosis over time. This case demonstrates the importance of including audiometric testing in a standardized meningitis protocol to diagnose hearing loss in a timely and accurate way and to achieve optimal long-term hearing outcomes.