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PubMed · 42391975

Cochlear size variation in a large-scale international multicentre cohort.

Andrea Schreier·Mario Zernotti·Aanand Acharya·Robert Eisenberg·Maryam Sahwan·Mohamed Alshehabi·Vincent Van Rompaey·Marc Lammers·Anandhan Dhanasingh·Suijun Chen·Lian Hui·Ahmed Mustafa·Nader El Feky·Ahmed Alshamsi·Ahmad Alamadi·Sebastian Schmerber·Benoit Godey·Estella Bilson-Amoah·Ramandeep Virk·Himanshu Swami·Abha Kumari·Shin-Ichi Usami·Hidekane Yoshimura·Grace Mwangi·Serah Ndegwa·Mutlaq Al-Sihan·Mariam Alkandari·Adekunle Adeyemo·Khalid Al Zaabi·Nasser Al Tamami·Piotr Henryk Skarzynski·Adam Walkowiak·Sima Sugarova·Vladislav Kuzovkov·Fida Almuhawas·Fahad N Altamami·Cristina Alonso·Luis Lassaletta·Louis Hofmeyr·Mohamed Razwi Ismail Ahmed·Elnemarie Burden·Johan Grobbelaar·Shaban Mawala·Ali F Jaffer·Amina Seguya·Linor Jones·Edward Flook·Anne Morgan Selleck·Camille Dunn·James Lin·Justus Müller-Goebel·Joachim Müller
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Abstract

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.

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HumansCochleaOrgan SizeCochlear duct lengthCochlear shapeCochlear sizeOTOPLAN®Journal ArticleScoping Review

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Andrea Schreier, Mario Zernotti, Aanand Acharya, Robert Eisenberg, Maryam Sahwan, Mohamed Alshehabi, Vincent Van Rompaey, Marc Lammers, Anandhan Dhanasingh, Suijun Chen, Lian Hui, Ahmed Mustafa, Nader El Feky, Ahmed Alshamsi, Ahmad Alamadi, Sebastian Schmerber, Benoit Godey, Estella Bilson-Amoah, Ramandeep Virk, Himanshu Swami, Abha Kumari, Shin-Ichi Usami, Hidekane Yoshimura, Grace Mwangi, Serah Ndegwa, Mutlaq Al-Sihan, Mariam Alkandari, Adekunle Adeyemo, Khalid Al Zaabi, Nasser Al Tamami, Piotr Henryk Skarzynski, Adam Walkowiak, Sima Sugarova, Vladislav Kuzovkov, Fida Almuhawas, Fahad N Altamami, Cristina Alonso, Luis Lassaletta, Louis Hofmeyr, Mohamed Razwi Ismail Ahmed, Elnemarie Burden, Johan Grobbelaar, Shaban Mawala, Ali F Jaffer, Amina Seguya, Linor Jones, Edward Flook, Anne Morgan Selleck, Camille Dunn, James Lin, Justus Müller-Goebel, Joachim Müller. 2026-06-14. Cochlear size variation in a large-scale international multicentre cohort.. https://doi.org/10.1016/j.heares.2026.109708

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Cochlear Implantation in Sickle Cell Disease: A Systematic Review of Feasibility and Outcomes.

INTRODUCTION: Sickle cell disease (SCD) is associated with systemic complications, including sensorineural hearing loss (SNHL) from microvascular occlusion and chronic inflammation. Although reports link SCD to higher rates of SNHL, current evidence is limited by small sample size, varied audiologic methods, and lack of standardized screening. This systematic review summarizes available literature on SNHL and cochlear implantation (CI) in SCD. METHODS: A literature search of PubMed MEDLINE, Embase, Scopus, Web of Science, and CINAHL identified 79 citations. After removal of duplicates, 35 records were screened in Rayyan. Studies published between January 1, 2000, and June 30, 2025, were eligible if they reported patients with SCD who developed hearing loss and underwent CI. Nine full texts were reviewed, and 4 met the inclusion criteria. Screening and review were performed independently by 2 authors per PRISMA guidelines. RESULTS: Across 4 case reports, a total of 5 patients with SCD underwent CI, ranging in age from 2 to 42 years. Four presented with bilateral severe-to-profound SNHL and one with unilateral loss. Implantation was technically feasible in all cases, including patients with cochlear fibrosis or ossification requiring modified insertion techniques. Postoperative outcomes were favorable: all patients demonstrated reliable device function and low impedances. Only 60% showed meaningful auditory benefit, characterized by improved functional speech perception in 2 patients (40%) and access to the speech frequency range with hearing testing going from moderate/profound hearing loss to mild hearing loss in 3 patients (60%). Complications occurred in 2 patients (40%): one developed unilateral middle ear infection leading to meningitis, and another experienced a postoperative pulmonary embolism requiring anticoagulation. The remaining 3 patients (60%) had uncomplicated recoveries with reported improved hearing from moderate/profound hearing loss to mild hearing loss post implantation. CONCLUSION: While CI appears feasible in SCD, our findings suggest that additional data are needed to assess its effectiveness in this patient population. However, evidence is limited to case reports, and complications such as thromboembolism and rapid cochlear fibrosis highlight the need for close perioperative management. More comprehensive studies with larger sample size are required to define surgical risk, optimize management, and establish best practices for timely implantation in this population.

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PubMed2026

Differential contributions of mt-Tr and Cs variants to developmental cochlear defects and mitochondrial dysfunction in A/J mice.

A/J mice exhibit early-onset hearing loss linked to Cdh23, mitochondrial tRNA-Arg (mt-Tr), and citrate synthase (Cs) variants. Although developmental cochlear defects have been identified in juvenile A/J mice, the hierarchical contributions of mt-Tr versus Cs remain unclear. Using reciprocal intercross-derived strains to decouple mitochondrial haplotypes from nuclear factors, we demonstrate that the mitochondrial background is the primary determinant of auditory dysfunction. Mice with A/J mtDNA (AXB strains) displayed significantly higher ABR thresholds, accelerated hair cell attrition, and severe stereocilia dysmorphology compared to those with B6 mtDNA (BXA strains), occurring largely independently of the Cs genotype. While the Cs mutation exacerbated hearing loss, its impact was secondary to that of the dominant mitochondrial background. Systemic behavioral assessments and mitochondrial assays confirmed that A/J mitochondria exert a more profound metabolic impact than the Cs mutation. Our findings establish that the mitochondrial genomic background, with the mt-Tr locus as a prominent candidate variant, serves as the principal driver of developmental cochlear defects and early-onset hearing loss in A/J mice, while the nuclear Cs mutation acts as a synergistic modifier. This study underscores the critical role of mitonuclear crosstalk in inner ear maturation and provides new insights into the etiology of hereditary hearing loss.

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Auditory brainstem response in the identification of cochlear synaptopathy in aged rodents: a systematic review with meta-analysis.

PURPOSE: This systematic review and meta-analysis evaluated the diagnostic performance of auditory brainstem response (ABR) for identifying age-related cochlear synaptopathies in rodents. METHOD: Following PRISMA guidelines, searches were conducted in PubMed/MEDLINE, Cochrane Library, Scopus, Embase, Web of Science, SciELO, LILACS, and gray literature. Studies evaluating CS in naturally aged rodents using short-latency auditory evoked potentials (AEPs) were included. Study selection, data extraction, risk-of-bias (JBI Critical Appraisal Checklist for Analytical Cross-Sectional Studies), and certainty of the evidence (GRADE® system) assessment were conducted independently by two reviewers. Meta-analyses were performed using a random-effects model, with standardized mean differences and 95% confidence intervals. ABR wave I amplitudes were analyzed for click (80 and 90 dB SPL) and tone-burst stimuli stratified by frequency. RESULTS: Among 3,008 identified records, 12 studies were included in the review and five in the meta-analysis. All included studies used ABR measures to investigate CS, with wave I amplitude being the most frequently evaluated biomarker. Meta-analysis demonstrated a significant reduction in ABR wave I amplitude in aged rodents compared with young controls for both click- and tone-burst-evoked responses. Tone-burst ABR showed no significant differences among the evaluated frequencies. These findings should be interpreted with caution due to the limited number of studies and the methodological heterogeneity, which may have reduced statistical power and comparability. CONCLUSIONS: The evidence supports ABR wave I amplitude as a sensitive electrophysiological marker of age-related CS in rodents. Nevertheless, further studies with standardized protocols are needed to strengthen its diagnostic utility and improve comparability across studies.

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