AIM:This study aims to (1) review the literature on cochlear size, measured by the A-value, to assess reported variation across studies, and (2) consolidate cochlear parameters-specifically basal turn diameter (A-value) and width (B-value)-measured using the standardized DICOM viewer OTOPLAN®, and compare these with published data. METHODS:A scoping literature review was conducted using the search term "diameter of cochlear basal turn" to identify studies reporting A-value measurements. To complement this, cochlear measurements obtained with OTOPLAN® were collected from clinical collaborators across multiple international centres. The ratio of B- to A-values, which defines the cochlear basal turn shape, was calculated as previously described by Khurayzi et al. (2021). RESULTS:The literature review identified 38 studies comprising 5,206 A-value measurements (average: 9.08 mm; range: 8.01-10.03 mm). Additionally, 5,580 OTOPLAN® measurements were collected from 43 clinics across 25 countries (mean: 9.07 mm; range: 6.67-11.45 mm). Overall, 68.7% of cochleae exhibited an elliptical basal turn shape. CONCLUSION:Using a large, international multicentre dataset and a standardized measurement tool, this study demonstrates substantial variation in cochlear size and shape across clinical cohorts from different regions. The mean A-value (9.07 mm) is consistent with previously published literature, while the wider observed range highlights greater anatomical variability within a multicentre clinical population than previously reported. The majority of cochleae demonstrate an elliptical basal turn configuration.

Background:Otitis media (OM) is a common pediatric infection worldwide. Conventionally, accurate diagnosis depends on in-person pneumatic otoscopy, which is not always accessible, contributing to delayed care and inappropriate prescribing, especially in underserved settings. Rapid advances in telemedicine and digital tools have accelerated the development of remote approaches for assessing pediatric ear diseases, while diagnostic quality maintenance, care models, and real-world outcomes have not been comprehensively mapped in children. Objective:This study aimed to map existing telehealth technologies and operational models used for pediatric OM and report their diagnostic and implementation outcomes to guide practice and further research. Methods:PRISMA-ScR (Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews) guided this scoping review (protocol not registered). MEDLINE (via PubMed), Scopus, Web of Science, and the Cochrane Central Register of Controlled Trials were searched for original English-language studies (published between January 1, 2010, and February 9, 2026) reporting the clinical implementation of telemedicine or digital health in children (<18 years) with suspected or confirmed OM. We excluded reviews, editorials, and protocols; conference abstracts; adult-only studies; and purely technical evaluations. Two reviewers independently extracted and charted the study characteristics: telemedicine model, technology, users, comparators, outcomes, and limitations. Findings were synthesized using practice-oriented mapping that aligned technologies with care models and implementation conditions. Results:Fifty-two studies across 18 countries and various settings met the inclusion criteria. Sample sizes ranged from 6 to 3950, with heterogeneous reporting units (children, ears, episodes, or screening assessments). Asynchronous store-and-forward tele-otoscopy was the most common approach. The rest used synchronous or hybrid models. Image capture by trained personnel and review by experienced clinicians yielded substantial diagnostic consistency with in-person microscopy (κ 0.68-0.89, sensitivity 72%-94%, specificity 93%-98%, where available). However, the diagnostic yield was highly dependent on the training level: structured instruction improved video capture by parents and nonspecialists, whereas brief or written-only guidance resulted in low rates of diagnostically useful videos. Telemedicine approaches improved access, supported perioperative follow-up, and, in some contexts, reduced reexaminations and promoted more judicious antibiotic use. Televisits without otoscopy were associated with lower confirmation rates of middle ear effusion during tympanostomy tube placement. Evidence was heterogeneous, with predominantly small single-site studies, variable reference standards and operator training, and rapidly evolving device ecosystems. Conclusions:This review provides a practice-oriented map of telehealth approaches for pediatric OM. Tele-otoscopy and adjunct digital tools are feasible, achieving diagnostic accuracy comparable to in-person assessments while enhancing access and service efficiency. However, important evidence gaps remain, including the need for large multisite trials, evaluation of long-term child outcomes, economic evaluations, and robust external validation of artificial intelligence-based diagnostic tools. Standardization of image capture protocols and integration into hybrid care models should be prioritized for scaling up.

OBJECTIVE:Otological diseases impair patients' physical, mental, and social quality of life. Surgical intervention against otologic diseases can improve the deterioration of the patient's quality of life and the recovery of hearing loss; however, no large-scale multicenter studies in Japan have investigated patients' quality of life after otologic surgery. To conduct a prospective investigation on the quality of life of patients who underwent otologic surgery. STUDY DESIGN:Case-control study. SETTING:A multicenter study involving 21 hospitals in Japan. PATIENTS:We enrolled 199 patients with cholesteatoma, 137 patients with chronic otitis media, 72 patients with otosclerosis, 126 patients before cochlear implantations, 12 patients with middle ear malformation, and 27 patients with other otologic diseases. INTERVENTIONS:We compared the impairment in patients' quality of life between different otologic diseases and examined the effects of surgical intervention using generic and disease-specific quality-of-life questionnaires. MAIN OUTCOME MEASURES:Improvement of the quality of life of the patients measured with general or disease-specific questionnaires. RESULTS:Our results revealed that the patients' preoperative quality of life was lower than that of the control group. Patients who underwent cochlear implantation had a significantly lower quality of life compared with other groups. The results also revealed that surgical intervention could improve postoperative quality of life. In addition, the study revealed the characteristic features of changes in the quality of life in each otologic disease group. CONCLUSIONS:These findings will be useful for patients when deciding on surgical intervention. It would also benefit otologic surgeons when explaining surgical indications or recommending surgical interventions.

BACKGROUND:Branchio-oto-renal (BOR) syndrome is characterized by branchiogenic malformation, hearing loss, and renal anomalies, with EYA1, SIX1, and SIX5 known as the causative genes. As BOR syndrome presents with various clinical phenotypes, its characteristics and genotype-phenotype correlations remain unknown. AIMS/OBJECTIVES:In this study, we aimed to clarify the detailed hearing loss phenotypes and genotype-phenotype correlations of BOR syndrome. MATERIAL AND METHODS:In this study, we performed an etiological analysis of 169 BOR syndrome patients from 129 families. We also performed genetic testing for 78 probands. RESULTS:In all, 66.7% of BOR patients carried EYA1 variants, whereas 17.9% carried SIX1 variants. We also clarified the detailed clinical features including the prevalence of major and minor symptoms, asymmetrical hearing loss, type of hearing loss, severity of hearing loss and detailed clinical characteristics of auricular, external ear, and middle ear and inner ear anomalies. In terms of genotype-phenotype correlations, patients with SIX1 variants had no kidney anomalies and fewer middle ear anomalies. CONCLUSIONS AND SIGNIFICANCE:We clarified the detailed hearing loss phenotypes of BOR syndrome patients. Our study results will contribute to a better understanding and clinical management of BOR syndrome patients.

OBJECTIVE:Clinical trials on cochlear gene therapy for patients with autosomal recessive deafness 9 through transcanal and transmastoid approaches were reported in 2024. While both are clinically feasible in older patients, the small size of the external auditory canal (EAC) in younger patients may limit the applicability of transcanal endoscopic approaches. Therefore, we aimed to evaluate pediatric EAC anatomy by using temporal bone computed tomography (CT). STUDY DESIGN:Retrospective study. SETTING:Tertiary center. PATIENTS:Children aged 0 to 18 years. INTERVENTIONS:We analyzed 624 temporal bone CT scans obtained between 2017 and 2024. MAIN OUTCOME MEASURES:Measurements included the full, bony, and cartilaginous lengths of the posterior and inferior EAC walls, angle between the inferior EAC wall and tympanic membrane, diameter at the narrowest point of the EAC, and tympanic membrane diameter, all assessed using a standardized method. RESULTS:The full and bony lengths of the inferior EAC wall, the angle between the inferior EAC wall and tympanic membrane, and the diameter at its narrowest point increased dramatically between 0 and 1 year of age. During this period, the annual increases in these measurements were 2.21 mm, 8.59 mm, 33.0 degrees, and 1.43 mm, respectively. In contrast, the tympanic membrane diameter remained unchanged throughout childhood. CONCLUSIONS:The feasibility of transcanal endoscopic ear surgery depends on the diameter at the narrowest point of the EAC, the endoscope and surgical instrument dimensions, and the length of the bony part of the EAC needed to elevate a tympanomeatal flap. Based on our findings, a transmastoid microscopic approach should be considered instead of transcanal approach in patients under 1 year of age.

Approximately 200 genes have been identified as causative in hereditary hearing loss. Genetic testing is increasingly important, not only for accurate diagnosis but also for predicting audiometric profiles, prognoses, and potential syndromic features. Hereditary hearing loss can be syndromic or nonsyndromic, with nonsyndromic forms further classified by inheritance: autosomal-dominant or autosomal-recessive. In autosomal-dominant cases, three pathological mechanisms—haploinsufficiency, dominant-negative effects, and gain of function—are often implicated. Moreover, specific genes correlate with distinct audiometric patterns: WFS1 variants typically cause low-frequency hearing loss, whereas KCNQ4 and POU4F3 variants are linked to high-frequency loss. To investigate the underlying mechanisms of these frequency-dependent patterns, gene expression across cochlear turns was compared in mice, but interpretations of the results were limited because of inherent structural differences between rodent and primate cochleae. Therefore, the common marmoset (Callithrix jacchus), which offers closer anatomical and functional similarity to human cochleae, was utilized herein as an improved model. Using RNA sequencing (RNA-seq) across cochlear turns of common marmosets, the present study aimed to uncover gene expression and alternative splicing patterns that may explain tonotopic manifestations in hereditary hearing loss, including those caused by WFS1 variants, the present study being one such using common marmoset cochlear RNA-seq data, and these findings are highly valuable for genetic diagnosis and the development of gene therapies.

Hearing preservation during surgery for large vestibular schwannomas (VS) remains challenging, and reports of simultaneous cochlear implantation (CI) via the retrosigmoid approach are exceedingly rare. We report, through close collaboration between neurosurgery and otolaryngology, what is, to our knowledge, the first case of simultaneous CI during retrosigmoid resection of a large sporadic VS under continuous intraoperative electrically evoked auditory brainstem response (EABR) monitoring. A 70-year-old woman with a 30-mm Koos grade 4 sporadic VS and contralateral profound hearing loss presented with right-sided scale-out audiometry (bilateral speech discrimination 0%). Retrosigmoid resection with continuous EABR monitoring via an intracochlear test electrode was performed, followed by immediate CI. Resection was terminated when wave V amplitude reached approximately 20% of baseline, achieving subtotal removal with preserved facial nerve function. The CI was activated postoperatively; aided thresholds reached 35-40 dB HL at 3 months, with stable tumor at 9 months. Preservation of EABR-not maximal tumor removal-should guide the extent of safe resection in CI-dependent candidates. Close interdisciplinary collaboration can extend hearing rehabilitation to patients with large VS, a population previously considered beyond the reach of hearing-preservation strategies.

OBJECTIVE:This study aimed to present and evaluate a surgical approach for placing a Vibrant Soundbridge® (VSB) device prior to auricular reconstruction in patients with microtia atresia. The goal was to determine whether an incision line for VSB implantation, located sufficiently posterior to the planned area of auricular reconstruction, could prevent interference with subsequent auricular surgeries and ensure stable hearing outcomes. METHODS:We retrospectively examined four patients with unilateral microtia who underwent VSB implantation before auricular reconstruction. The incision line was placed approximately 5 cm posterior to the posterior edge of the temporomandibular joint, ensuring that it remained at least 2 cm away from the future area of auricular reconstruction. The VSB implantation procedure involved skin incision, subperiosteal dissection, mastoidectomy, and placement of the VSB implant, with a floating mass transducer attached to the head of the stapes. More than a year later, two-stage auricular reconstruction was performed. RESULTS:All patients underwent successful auricular reconstruction after VSB implantation. During the first stage of costal cartilage grafting, subcutaneous pockets were created without encountering congestion or compromised skin flaps, and the grafted cartilage maintained a good contour. In the second stage of auricular elevation, the mastoid fascial flap was elevated with adequate blood flow, and careful dissection prevented exposure or damage to the VSB device. Postoperatively, the reconstructed auricles exhibited stable contour. Audiometric evaluation revealed no deterioration in hearing outcomes, indicating that the VSB device worked well. CONCLUSION:This method allows safe and effective VSB implantation prior to auricular reconstruction, without hindering subsequent procedures. By maintaining an appropriate distance between the incision line and planned area of reconstruction, early hearing rehabilitation and successful auricular reconstruction can be achieved. This approach may serve as a practical standard for treating patients with microtia atresia who require early hearing intervention.

Otitis media (OM) is one of the most common pediatric infections worldwide. Conventionally, accurate diagnosis depends on in-person pneumatic otoscopy, which is not always accessible, contributing to delayed care and inappropriate prescribing, especially in underserved settings. Rapid advances in telemedicine and digital tool have accelerated the development of remote approaches for assessing pediatric ear diseases, while maintaining diagnostic quality, care models, and real-world outcomes have not been comprehensively mapped in children. To map existing technologies and operational models employed in telehealth for pediatric OM and report their diagnostic and implementation outcomes to guide practice and further research. Between January 1, 2010, and August 31, 2025, a scoping review guided by Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR) was performed (protocol not registered). PubMed, MEDLINE, Web of Science, and Cochrane CENTRAL were systematically searched for studies that reported on the clinical implementation of telemedicine or digital health in children (<18 years) with suspected or confirmed OM. Two reviewers independently extracted and charted the following study characteristics: telemedicine model, technology, users, comparators, outcomes, and limitations. Of the 805 records searched, 46 met the inclusion criteria. These studies have been performed in diverse countries and settings. Asynchronous store-and-forward tele-otoscopy was the most frequently used approach. The rest used either synchronous or hybrid models. When images were captured by trained personnel and reviewed by experienced clinicians, diagnostic consistency with in-person microscopy was substantial. However, the diagnostic yield was highly dependent on the level of training: structured instruction improved video capture by parents and nonspecialists, whereas brief or written-only guidance resulted in low rates of diagnostically useful videos. Telemedicine approaches improved access, supported perioperative follow-up, and, in some contexts, reduced re-examinations and promoted more judicious antibiotic use. However, televisits without otoscopy were associated with lower confirmation rates of middle-ear effusion during tympanostomy tube placement. Tele-otoscopy and adjunct digital tools have been demonstrated to be feasible, achieving diagnostic accuracy comparable to in-person assessments, while enhancing access and service efficiency. However, important evidence gaps remain, including the need for large multisite trials, evaluation of long-term child outcomes, economic evaluations, and robust external validation of artificial intelligence (AI)-based diagnostic tools. Standardization of image capture protocols and integration into hybrid care models should be prioritized for scaling up.

Background/Objectives: A heterozygous mutation in the WFS1 gene is responsible for autosomal dominant non-syndromic hearing loss (DFNA6/14/38) and Wolfram-like syndrome, which is characterized by bilateral sensorineural hearing loss with optic atrophy and/or diabetes mellitus. However, detailed clinical features for the patients with the heterozygous p.A684V variant remain unknown. Methods: We report the clinical details of 14 cases with a heterozygous p.A684V variant in the WFS1 gene identified from target resequencing analysis of 63 previously reported deafness genes by next-generation sequencing of 15,684 hearing loss patients (mean age 27.5 ± 23.1 years old, 6574 male, 8612 female and 498 for whom information was unavailable). Results: Among the 14 patients from 13 families with the p.A684V variant, nine were sporadic cases. In addition, we confirmed de novo occurrence of this variant in seven families. This result strongly supports the notion that this variant was located on a mutational hotspot. When comparing previously reported cases of autosomal dominant WFS1 gene-associated hearing loss, most of the patients in this study showed severe-to-profound bilateral sensorineural hearing loss (genotype–phenotype correlation). Two patients had optic atrophy, while the others did not have any other complications. Conclusions: The identified heterozygous p.A684V variant appears to be a hotspot mutation and likely to cause severe-to-profound hearing loss in early childhood. Cochlear implantation is considered favorable in cases of hearing impairment due to this variant.

Background/Objectives: Hearing loss is one of the most common sensorineural impairments, and approximately 60% of early-onset cases are due to genetic variations. The otogelin-like protein, encoded by the OTOGL gene, is a component of the acellular membranes of the inner ear, such as the tectorial membrane, and is thought to play an important role in cochlear amplification. OTOGL gene variants are a rare cause of hearing loss such as DFNB84B, a mild-to-moderate sensorineural hearing loss presenting in early childhood with autosomal recessive inheritance. In this study, we aim to enhance our comprehension of the phenotypes of hearing loss caused by OTOGL variants. Methods: A total of 7056 Japanese patients with hearing loss were recruited, and based on massively parallel DNA sequencing on 158 target genes, we selected patients with biallelic OTOGL variants. Results: Ten affected individuals with OTOGL gene variants were detected, the largest group of patients yet to be reported, and eight of the eleven variants were novel. Our results showed that variations in this gene led to mild-to-moderate non-progressive hearing loss, and the accompanying symptoms, mainly vestibular symptoms, were speculated to present in adulthood. Conclusions: Determination of the phenotypes of genes causative of hearing loss is expected to greatly benefit patients with hearing loss as it can assist in predicting outcomes and lead to appropriate intervention, which, in OTOGL-associated hearing loss cases, is based around the fact that the patients need not be concerned with deterioration in hearing, but require careful follow-up for vestibular symptoms.

Background/Objectives: During and after electric–acoustic stimulation (EAS) surgery (as well as regular cochlear implant surgery), the oral and/or intravenous administration of steroids is recommended to prevent acute inflammatory reactions and subsequent fibrosis. However, the effect does not last long. Therefore, with the hope of providing a sustained effect, a new dexamethasone (DEX)-eluting electrode (FLEX28 DEX) has recently been developed. Methods: A case study was performed at Shinshu University in February 2024 in which a DEX-eluting electrode array was utilized for a patient presenting with high-frequency hearing loss with a defined etiology (hearing loss due to a mitochondrial m.1555A > G variant). Results: Residual hearing was well preserved after EAS surgery, and post-operative impedance field telemetry was maintained at a very low level in contrast with a historical/retrospective control group (FLEX28 electrodes without DEX); therefore, it is expected that post-operative fibrosis will be minimized. Further, it was shown that the DEX-eluting electrode can also be applied to EAS. Conclusions: The DEX-eluting electrode was useful in maintaining post-operative impedance at a very low level, indicating that post-operative fibrosis could be minimized even after EAS surgery.

Background The integration of artificial intelligence (AI), particularly deep learning models, has transformed the landscape of medical technology, especially in the field of diagnosis using imaging and physiological data. In otolaryngology, AI has shown promise in image classification for middle ear diseases. However, existing models often lack patient-specific data and clinical context, limiting their universal applicability. The emergence of GPT-4 Vision (GPT-4V) has enabled a multimodal diagnostic approach, integrating language processing with image analysis. Objective In this study, we investigated the effectiveness of GPT-4V in diagnosing middle ear diseases by integrating patient-specific data with otoscopic images of the tympanic membrane. Methods The design of this study was divided into two phases: (1) establishing a model with appropriate prompts and (2) validating the ability of the optimal prompt model to classify images. In total, 305 otoscopic images of 4 middle ear diseases (acute otitis media, middle ear cholesteatoma, chronic otitis media, and otitis media with effusion) were obtained from patients who visited Shinshu University or Jichi Medical University between April 2010 and December 2023. The optimized GPT-4V settings were established using prompts and patients’ data, and the model created with the optimal prompt was used to verify the diagnostic accuracy of GPT-4V on 190 images. To compare the diagnostic accuracy of GPT-4V with that of physicians, 30 clinicians completed a web-based questionnaire consisting of 190 images. Results The multimodal AI approach achieved an accuracy of 82.1%, which is superior to that of certified pediatricians at 70.6%, but trailing behind that of otolaryngologists at more than 95%. The model’s disease-specific accuracy rates were 89.2% for acute otitis media, 76.5% for chronic otitis media, 79.3% for middle ear cholesteatoma, and 85.7% for otitis media with effusion, which highlights the need for disease-specific optimization. Comparisons with physicians revealed promising results, suggesting the potential of GPT-4V to augment clinical decision-making. Conclusions Despite its advantages, challenges such as data privacy and ethical considerations must be addressed. Overall, this study underscores the potential of multimodal AI for enhancing diagnostic accuracy and improving patient care in otolaryngology. Further research is warranted to optimize and validate this approach in diverse clinical settings.

OBJECTIVE:To investigate the frequency, treatment, and outcomes of postoperative delayed-onset swelling around cochlear implants. STUDY DESIGN:Retrospective, observational, nonrandomized group study. SETTING:Academic medical center. PATIENTS/INTERVENTIONS:Among 354 patients (516 ears) who underwent cochlear implantation (CI) at our hospital between May 2009 and October 2022, 329 (472 ears: 138 children [246 ears] and 191 adults [226 ears]) with a follow-up period of >3 months were included. MAIN OUTCOME MEASURES:Physical examination and computed tomography of the head were performed. RESULTS:In total, 5.5% (26/472 ears) had a history of delayed-onset swelling around the implant. This complication occurred in 9.8% (24/246 ears) of children and 0.9% (2/226 ears) of adults. The mean time to onset of swelling was 50 (range, 5.5-147) months following CI. In 60% (21/35) of the cases, the cause was unknown, whereas in 25.7% (9/35) and 11.5% (4/35) of cases, it was head trauma and acute inflammation, respectively. Conservative treatment (observation, antibiotics, and/or strong magnetic compression) was adapted in 91.4% (32/35) of cases. After conservative treatment, revision CI surgery was performed in one ear. Additionally, recurrent swelling was observed in 23.1% (6/26 ears) of swelling cases. CONCLUSIONS:The results suggest that delayed-onset swelling around implants occurs more frequently in children than in adults because of the higher incidence rates of head trauma and acute otitis media in children. In most cases, conservative treatment was adequate; however, careful follow-up is necessary. Our findings can serve as a reference for optimizing care and intervention options after CI.

Electric-acoustic stimulation (EAS) is a promising treatment to improve hearing ability in patients with high-frequency hearing loss (HL). In EAS surgeries, shorter electrodes have been preferred to avoid the presence of an electrode covering the residual hearing region. However, our earlier studies showed that EAS with longer electrodes (28 mm) could preserve acoustic hearing. Additionally, we reported that the hearing preservation (HP) scores were independent of the length of the inserted electrodes, consistent with the systematic review. As most EAS patients gradually lose residual hearing over time due to the natural course of HL, in these cases, providing broader cochlear coverage using longer electrodes was beneficial toward better place-pitch matching. In addition to preparing for the deterioration in hearing in the future, EAS with longer electrodes could offer various types of map strategies. Herein, we show the pre-, intra-, and post-procedures for EAS surgery. Appropriate preoperative evaluation, less invasive surgery, flexible lateral-wall electrodes, and steroid administration resulted in good HP following EAS with longer electrodes.