Search Results (468)

This research compares speech discrimination abilities between 17 children who are hard-of-hearing (CHH) and 13 children with normal hearing (CNH), aged 9 to 36 months, using either a conditioned head turn (CHT) or condition play paradigm, for two phoneme pairs /ba-da/ and /sa-ʃa/. As CHH were tested in the aided and unaided conditions, CNH were also tested on each phoneme contrast twice to control for learning effects. When speech discrimination abilities were compared between CHH, with hearing aids (HAs), and CNH, there were no statistical differences observed in performance on stop consonant discrimination, but a significant statistical difference was observed for fricative discrimination performance. Among CHH, significant benefits were observed for /ba-da/ speech discrimination while wearing HAs, compared to the no HA condition. All CHH were early-identified, early amplified, and were enrolled in parent-centered early intervention services. Under these conditions, CHH demonstrated the ability to discriminate speech comparable to CNH. Additionally, repeated testing within 1-month did not result in a change in speech discrimination scores, indicating good test–retest reliability of speech discrimination scores. Finally, this research explored the question of infant/toddler listening fatigue in the behavioral speech discrimination task. The CHT paradigm included returning to a contrast (i.e., /a-i/) previously shown to be easier for both CHH and CNH to discriminate to examine if failure to discriminate /ba-da/ or /sa-ʃa/ was due to listening fatigue or off-task behavior. Full article

Background: Thyroid eye disease (TED) is a rare autoimmune orbital disorder predominantly associated with Graves’ disease. It is characterized by orbital inflammation, tissue remodeling, and potential visual morbidity. Conventional therapies, particularly systemic glucocorticoids, offer only partial symptomatic relief, failing to reverse chronic structural changes such as proptosis and diplopia, and are associated with substantial adverse effects. This review aims to synthesize recent developments in understandings of TED pathogenesis and to critically evaluate emerging therapeutic strategies. Methods: A systematic literature review was conducted using MEDLINE, Embase, and international clinical trial registries focusing on pivotal clinical trials and investigational therapies targeting core molecular pathways involved in TED. Results: Current evidence suggests that TED pathogenesis is primarily driven by the autoimmune activation of orbital fibroblasts (OFs) through thyrotropin receptor (TSH-R) and insulin-like growth factor-1 receptor (IGF-1R) signaling. Teprotumumab, a monoclonal IGF-1R inhibitor and the first therapy approved by the U.S. Food and Drug Administration for TED, has demonstrated substantial clinical benefit, including improvements in proptosis, diplopia, and quality of life. However, concerns remain regarding relapse rates and treatment-associated adverse events, particularly hearing impairment. Investigational therapies, including next-generation IGF-1R inhibitors, small-molecule antagonists, TSH-R inhibitors, neonatal Fc receptor (FcRn) blockers, cytokine-targeting agents, and gene-based interventions, are under development. These novel approaches aim to address both inflammatory and fibrotic components of TED. Conclusions: Teprotumumab has changed TED management but sustained control and toxicity reduction remain challenges. Future therapies should focus on targeted, mechanism-based, personalized approaches to improve long-term outcomes and patient quality of life. Full article

Theory of Mind (ToM) is a construct that includes a range of connected abilities linked to the understanding of others’ mental states. During the last three decades, ToM development has been studied extensively in deaf and hard of hearing (DHH) individuals and performances compared to the typically hearing (TH) population. Given the advances in the early diagnosis of deafness, interventions, and hearing devices over this period, variations in task performance among DHH participants might have been reduced. The current systematic review aims to synthesize all studies of ToM in DHH individuals and answer the following question: Do DHH individuals (Population), compared to a control sample of TH and/or among themselves (Comparator), in an assessment of ToM (Intervention), have differentiated results (Outcome)? After a search of the literature, 97 papers were included. We found that, in general, TH participants outperformed their DHH peers in ToM measures; however, there was a wide range of results. Explanations for this variability included the quality of early interactions and early exposure to both signed and spoken language. The review also indicates that the understanding of false belief was the most studied component within ToM, while other components, such as understanding intention and irony, require further research. Implications of these findings for clinical practice are discussed. Full article

Objectives: Lermoyez syndrome (LS) is a rare variant of endolymphatic hydrops with a unique clinical presentation characterized by reversible sensorineural hearing loss preceding vertigo. This review aims to synthesize available literature on LS to clarify its clinical characteristics, diagnostic approach, management strategies, and outcomes, and to highlight the distinguishing features from Menière’s disease (MD). Methods: A systematic literature review according to PRISMA guidelines was conducted from 1919 to 2025. The extracted data included demographics, symptom profiles, audiovestibular testing, imaging findings, treatment approaches, and patient outcomes. Results: A total of 23 studies were identified, reporting 53 individual cases of LS. Patients ranged from 27 to 85 years of age, with a mean age of 50.34 years and a male predominance (64.1%). The hallmark of LS across cases was a reproducible clinical pattern of unilateral low-frequency hearing loss followed by vertigo and subsequent auditory recovery. Audiometry typically confirmed reversible sensorineural hearing loss, while vestibular tests and imaging were often unremarkable, primarily used to exclude alternative diagnoses. Treatment approaches varied and were often based on MD protocols, including dietary modifications, vasodilators, diuretics, and vestibular suppressants. Prognosis was generally favorable, with most patients experiencing both hearing recovery and symptom resolution. Conclusions: LS remains a clinically distinct but underrecognized inner ear disorder. Its defining feature—the paradoxical improvement in hearing after vertigo—distinguishes it from Menière’s disease and should prompt clinicians to consider LS in differential diagnosis. Due to the rarity of LS and the lack of standardized guidelines, diagnosis and treatment rely on careful clinical assessment and individualized management strategies. Full article

Objective: Hearing rehabilitation using hearing aids keeps increasing in the general population. Patient-related outcome measures are essential to evaluate benefits. Although the IOI-HA is routinely used in France, its translated version from 2002 has never been validated. This study aimed to assess the psychometric properties of the French version of the IOI-HA questionnaire. Design: Controlled, prospective, monocentric study performed between February 2024 and January 2025. The forward–backward technique was used for translation of the questionnaire. Study Sample: 100 patients fitted with hearing aids completed the questionnaire. Thirty-five patients were retested 15 days after first completion. Results: Internal consistency, assessed by Cronbach’s alpha, was 0.863. Mean IOI-HA item scores ranged from 3.3 to 4.57. All seven items had a high degree of consistency with the total score, except for item Q1 which had a moderate score (0.45). Cronbach’s alpha after item deletion confirmed internal consistency. Intra-class correlation coefficients ranged from 0.622 (Q7) to 0.767 (Q5) and were all statistically significant (p < 0.001), revealing high reliability over time. No significant correlation was found between item scores and age, unilateral or bilateral hearing aid use or accompanying symptoms (tinnitus, dizziness). Conclusions: The French translation of the IOI-HA questionnaire, published in 2002, is a valid and reliable questionnaire evaluating hearing aid satisfaction. This validated questionnaire can now be used in daily clinical practice. Full article

Background: Cochlear implantation (CI) in pediatric patients with cochlear nerve deficiencies (CND) remains controversial due to a highly variable clinical population, lack of evidence-based guidelines, and mixed research findings. This study assessed current clinical perspectives and practices regarding CI candidacy in children with CND among hearing healthcare professionals in the USA. Methods: An anonymous 19-question online survey was distributed to CI clinicians nationwide. The survey assessed professional background, experience with aplasia and hypoplasia, and perspectives on CI versus auditory brainstem implant (ABI) candidacy, including imaging practices and outcome expectations. Both multiple-choice and open-ended responses were analyzed to identify trends and reasoning. Results: Seventy-two responses were analyzed. Most clinicians supported CI for hypoplasia (60.2%) and, to a lesser extent, for aplasia (41.7%), with audiologists more likely than neurotologists to favor CI. Respondents cited lower risk, accessibility, and the potential for benefit as reasons to attempt CI before ABI. However, many emphasized a case-by-case approach, incorporating imaging, electrophysiological testing, and family counseling. Only 22.2% considered structural factors the best predictors of CI success. Conclusions: Overall, hearing health professionals in the USA tend to favor CI as a first-line option, while acknowledging the limitations of current diagnostic tools and the importance of individualized, multidisciplinary decision-making in CI candidacy for children with CND. Findings reveal a high variability in clinical perspectives on CI implantation for pediatric aplasia and hypoplasia and a lack of clinical consensus, highlighting the need for more standardized assessment and imaging protocols to provide greater consistency across centers and enable the development of evidence-based guidelines. Full article

Objective: To evaluate the outcomes of a modified infratemporal fossa approach (ITFA) that preserves the posterior external auditory canal (EAC) in patients with tumors in the infratemporal fossa and skull base, focusing on postoperative hearing and facial nerve function. Methods: This retrospective study included nine patients who underwent ITFA with posterior EAC preservation for tumor removal while minimizing facial nerve rerouting. All surgeries were performed by a single surgeon. Preoperative and postoperative hearing levels, facial nerve function, tumor characteristics, and surgical outcomes were analyzed. Air-bone gaps (ABG) were assessed using pure tone audiometry, and facial nerve function was assessed using the House–Brackmann grading system. Results: The cohort consisted of eight female patients and one male patient, with a mean tumor size of 3.0 cm. Surgical outcomes were promising, with no statistically significant increase in postoperative ABG and well-preserved facial nerve function. Only one patient developed postoperative grade II facial palsy. A residual tumor was identified in one case with extensive meningioma, which has remained stable, and no recurrence or regrowth was noted during the follow-up period (mean: 3.7 years). The modified approach minimized complications related to conductive hearing loss and facial nerve dysfunction. Conclusions: The modified ITFA with posterior EAC preservation provides a promising alternative to conventional ITFA for managing deep-seated tumors. It preserves both hearing and facial nerve function while ensuring adequate tumor resection. Full article

Background/Objectives: Cochlear implants (CIs) and hearing aids (HAs) have enhanced auditory rehabilitation in elderly individuals, yet limitations in musical perception and psychosocial integration persist. This systematic review aimed to evaluate the effects of music therapy (MT) on the quality of life (QoL), self-esteem, auditory perception, and cognition in older CI and HA users. Methods: A comprehensive search of PubMed was conducted up to March 2022 following PRISMA guidelines. Studies involving participants aged ≥ 60 years with CIs and/or HAs were included. Ten studies (n = 21,632) met eligibility criteria. Data were extracted and assessed using the Newcastle–Ottawa Scale. Results: MT led to improved sound quality, with HISQUI19 scores rising from 60.0 ± 21.8 to 74.2 ± 27.5. Early MT exposure was associated with significantly better MUMU outcomes (p = 0.02). Bilateral CI users showed enhanced stereo detection (52% to 86%), and CI + HA users achieved CNC scores exceeding 95%. Postlingual CI users outperformed prelingual peers in musical discrimination (9.81 vs. 3.48; p < 0.001). Long-term HA use was linked to better a QoL and reduced loneliness. Conclusions: While music therapy appears to support auditory and psychosocial functioning in hearing-impaired older adults, the absence of randomized controlled trials limits causal inference regarding its effects. These results support its integration into hearing rehabilitation strategies for older adults. Full article

This study investigates hearing outcomes of stapedotomy using two different types of prostheses: manually crimped MatriX and thermally activated NiTiBOND. The primary objective was to determine whether the method of prosthesis fixation to the long process of incus influences postoperative results. A retrospective analysis was conducted on 155 patients with otosclerosis; 90 received the NiTiBOND prosthesis and 65 received the MatriX prosthesis. Choice of prosthesis was determined intraoperatively based on position of chorda tympani. If the nerve was located near the incus and the prosthesis fixation site, the surgeon opted for MatriX prosthesis to avoid potential injury from activation of the NiTiBOND. Audiometric evaluations revealed no statistically significant differences in bone conduction thresholds on the first postoperative day (p = 0.275) or at six weeks (p = 0.899), postoperative air-bone gap (p = 0.810), air-bone gap closure (p = 0.489), overclosure (p = 0.436), or bone conduction at 4 kHz (p = 0.324). Chorda tympani nerve injury occurred in 9.2% of cases with MatriX prosthesis and 6.7% with NiTiBOND prosthesis (p = 0.556). Our findings highlight the theoretical and practical significance of comparing both prostheses, demonstrating that NiTiBOND can serve as an alternative in anatomically favorable cases, thereby guiding treatment choices. Full article

This paper offers an overview of a large study of language and cognitive development in deaf and hard of hearing children. Specifically, we investigated how acquiring a signed or spoken language (language modality) and when a child’s access to language begins (i.e., at birth or later in development) influence cognitive development. We conducted in-person behavioral assessments with 404 children 3–10 years old (280 deaf and hard of hearing; 124 typically hearing). The tasks measured a range of abilities along a continuum of how strongly they depend on language input, such as general vocabulary and number words (strongly dependent) vs. skills such as tracking sets of two to three objects and standardized ‘nonverbal’ picture-similarity tasks (relatively independent of language). Overall, the timing of children’s access to language predicted more variability in their performance than language modality. These findings help refine our theories about how language influences development and suggest how a STEAM pedagogical approach may ameliorate the impacts of later access to language. These results underscore children’s need for language early in development. That is, deaf and hard of hearing children must receive fully accessible language input as early as possible through sign language, accompanied by hearing technology aimed at improving access to spoken language, if desired. Full article

Background/Objectives: Menière’s disease (MD) is a debilitating disorder with episodic and variable ear symptoms. Diagnosis can be challenging, and evidence for therapeutic approaches is low. Furthermore, patients show a unique and fluctuating configuration of audiovestibular impairment. As a psychometric instrument to assess hearing-specific disability is currently lacking, we evaluated a short form of the Speech, Spatial, and Qualities of Hearing Scale (SSQ) in a cohort of patients with MD. Methods: Data was collected in the context of a multicenter prospective patient registry intended for the long-term follow up of MD patients. Hearing was assessed by pure tone and speech audiometry. The SSQ was applied in the German language version with 17 items. Results: In total, 97 consecutive patients with unilateral MD with a mean age of 56.2 ± 5.0 years were included. A total of 55 individuals (57.3%) were female, and 72 (75.0%) were categorized as having definite MD. The average total score of the SSQ was 6.0 ± 2.1. Cronbach’s alpha for internal consistency was 0.960 for the total score. We did not observe undue floor or ceiling effects. SSQ values showed a statistically negative correlation with hearing thresholds and a statistically positive correlation with speech recognition scores of affected ears. Conclusions: The short form of the SSQ provides insight into hearing-specific disability in patients with MD. Therefore, it may be informative regarding disease stage and rehabilitation needs. Full article

Background/Objectives: Congenital cytomegalovirus (cCMV) infection is the most common non-genetic cause of sensorineural hearing loss (SNHL) in children. In cases of severe-to-profound SNHL, cochlear implantation (CI) is a widely used intervention, but outcomes remain variable due to possible neurodevelopmental comorbidities. This study aimed to evaluate the long-term auditory and language outcomes in children with cCMV after CI and to explore clinical and radiological predictors of post-CI performance. Methods: Fifty-three children with cCMV and bilateral severe-to-profound SNHL who underwent CI at five tertiary referral centers in Italy were included in the study. Auditory and language outcomes were assessed pre- and post-implantation using the Categories of Auditory Performance II (CAP-II) scale, the Nottingham 3-Level Classification, and the Bates Language Development Scale. Brain MRI abnormalities were classified according to the Alarcón classification. Correlations were explored between outcome scores and symptomatic status at birth, MRI findings, and neurodevelopmental comorbidities. Results: At birth, 40 children (75.5%) were symptomatic and 13 (24.5%) asymptomatic. Neurodevelopmental comorbidities were present in 19 children (35.8%). MRI was normal in 15 (28.3%), mildly abnormal in 26 (49%), and moderately to severely abnormal in 12 (22.6%). Auditory and language outcomes improved significantly post-CI (p < 0.001), though the outcomes varied widely. Twenty-five children (47%) reached CAP level ≥ 6, and thirteen (23%) reached Bates Level 6. Symptomatic status at birth correlated weakly with worse CAP (ρ = −0.291, p = 0.038) and Bates (ρ = −0.310, p = 0.028) scores. Higher Alarcón scores were significantly associated with neurodevelopmental comorbidities, though not directly with post-CI auditory and language outcomes. Finally, the presence of neurodevelopmental disabilities was generally associated with lower results, even if without statistical significance. Conclusions: CI provides substantial auditory and language benefit in children with cCMV, even in cases of severe neurodevelopmental comorbidities. MRI and developmental assessments, as well as perinatal history for clinical signs and symptoms, are helpful in guiding expectations and personalizing post-implantation support. Full article

Platinum-induced ototoxicity constitutes a significant adverse effect in pediatric oncology, frequently resulting in permanent hearing impairment with profound implications for quality of life, language acquisition, and scholastic performance. This comprehensive review critically evaluates contemporary ototoxicity monitoring practices across various pediatric oncology settings, analyzes current guideline recommendations, and formulates strategies for implementing standardized surveillance protocols. Through examination of recent literature—encompassing retrospective cohort investigations, international consensus recommendations, and functional outcome assessments—we present an integrated analysis of challenges and opportunities in managing chemotherapy-associated hearing loss among childhood cancer survivors. Our findings demonstrate marked heterogeneity in monitoring methodologies, substantial implementation obstacles, and considerable impact on survivors’ functional status across multiple domains. Particularly concerning is the persistent absence of an evidence-based consensus regarding the appropriate duration of audiological surveillance for this vulnerable population. We propose a structured framework for comprehensive ototoxicity management emphasizing prompt detection, standardized assessment techniques, and integrated long-term follow-up care to minimize the developmental consequences of platinum-induced hearing impairment. This approach addresses critical gaps in current practice while acknowledging resource limitations across diverse healthcare environments. Full article

Usher syndrome, a rare genetic disorder causing both hearing and vision loss, presents significant diagnostic and therapeutic challenges due to its complex genetic basis. The identification of reliable biomarkers for early detection and intervention is crucial for improving patient outcomes. In this study, we present a machine learning-based hybrid sequential feature selection approach to identify key mRNA biomarkers associated with Usher syndrome. Beginning with a dataset of 42,334 mRNA features, our approach successfully reduced dimensionality and identified 58 top mRNA biomarkers that distinguish Usher syndrome from control samples. We employed a combination of feature selection techniques, including variance thresholding, recursive feature elimination, and Lasso regression, integrated within a nested cross-validation framework. The selected biomarkers were further validated using multiple machine learning models, including Logistic Regression, Random Forest, and Support Vector Machines, demonstrating robust classification performance. To assess the biological relevance of the computationally identified mRNA biomarkers, we experimentally validated candidates from the top 10 selected mRNAs using droplet digital PCR (ddPCR). The ddPCR results were consistent with expression patterns observed in the integrated transcriptomic metadata, reinforcing the credibility of our machine learning-driven biomarker discovery framework. Our findings highlight the potential of machine learning-driven biomarker discovery to enhance the detection of Usher syndrome. Full article

Background/Objectives: This study aimed to (i) cross-culturally adapt the Inventory of Hyperacusis (IHS) into Danish and (ii) assess its usability, validity, and reliability in Danish adults with hyperacusis. Methods: The translation followed established guidelines for adapting hearing-related questionnaires. A two-phase design ensured linguistic and cultural adaptation and evaluated test–retest reliability and construct validity. The IHS, consisting of 25 items, was translated and tested in seven participants through cognitive debriefing. In phase two, temporal consistency was assessed in 32 patients. Results: Thirty-two participants (twenty-eight female; mean age 49.8 years) completed the study over 2–4 weeks (mean 22 days). Eight used hearing aids, and twenty-four reported tinnitus. The Danish IHS showed good reliability (Cronbach’s alpha = 0.95) and acceptable test–retest reliability, except for the General Loudness factor. While no systematic score changes occurred, significant variability in score changes were noted. Conclusions: The Danish IHS appears to be a reliable and valid tool for assessing hyperacusis. Further research is needed, but the IHS-DK shows potential as an effective clinical and research tool for evaluating hyperacusis impact and treatment outcomes. Full article