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Objective: The objectives of this study are twofold: first, to visualize the structure of malformed cochleae through image reconstruction; and second, to develop a predictive model for postoperative outcomes of cochlear implantation (CI) in patients diagnosed with cochlear hypoplasia (CH) and incomplete partition (IP) malformation.
Methods: The clinical data from patients diagnosed with cochlear hypoplasia (CH) and incomplete partition (IP) malformation who underwent cochlear implantation (CI) at Beijing Tongren Hospital between January 2016 and August 2020 were collected. Radiological features were analyzed through 3D segmentation of the cochlea. Postoperative auditory speech rehabilitation outcomes were evaluated using the Categories of Auditory Performance (CAP) and the Speech Intelligibility Rating (SIR). This study aimed to investigate the relationship between cochlear parameters and postoperative outcomes. Additionally, a predictive model for postoperative outcomes was developed using the K-nearest neighbors (KNN) algorithm.
Results: In our study, we conducted feature selection by using patients' imaging and audiological attributes. This process involved methods such as the removal of missing values, correlation analysis, and chi-square tests. The findings indicated that two specific features, cochlear volume (V) and cochlear canal length (CDL), significantly contributed to predicting the outcomes of hearing and speech rehabilitation for patients with inner ear malformations. In terms of hearing rehabilitation, the KNN classification achieved an accuracy of 93.3%. Likewise, for speech rehabilitation, the KNN classification demonstrated an accuracy of 86.7%.
Conclusion: The measurements obtained from the 3D reconstruction model hold significant clinical relevance. Despite the considerable variability in cochlear morphology across individuals, radiological features remain effective in predicting cochlear implantation (CI) prognosis for patients with inner ear malformations. The utilization of 3D segmentation techniques and the developed predictive model can assist surgeons in conducting preoperative cochlear structural measurements for patients with inner ear malformations. This, in turn, can offer a more informed perspective on the anticipated outcomes of cochlear implantation.
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http://dx.doi.org/10.1007/s00405-024-08463-w | DOI Listing |
Hum Genet
September 2025
College of Otolaryngology Head and Neck Surgery, Chinese PLA General Hospital, Chinese PLA Medical School, 28 Fuxing Road, Beijing, 100853, China.
Recessive variants in TWNK cause syndromes arising from mitochondrial DNA (mtDNA) depletion. Hearing loss is the most prevalent manifestation in individuals with these disorders. However, the clinical and pathophysiological features have not been fully elucidated.
View Article and Find Full Text PDFBraz J Otorhinolaryngol
September 2025
Clinical Research Department, MED-EL GmbH, Innsbruck, Austria.
Objectives: Healthcare systems contribute significantly to global greenhouse gas emissions through energy consumption and waste generation. This study aims to explore strategies to make cochlear implantation processes more environmentally sustainable and aligned with the United Nations' Sustainable Development Goals.
Methods: We examined various approaches including the use of bio-based and biodegradable materials, sustainable energy solutions, greener anesthetic practices, effective waste separation and recycling in operating rooms, and patient-centered strategies such as reducing travel and promoting early activation and fitting of cochlear implants.
Am J Case Rep
September 2025
Oto-Rhino-Laryngology Surgery Clinic, Institute of Physiology and Pathology of Hearing - World Hearing Center, Kajetany, Poland.
BACKGROUND Duplicated internal auditory canal (dIAC) is a rare congenital temporal bone anomaly associated with ipsilateral sensorineural hearing loss (SNHL). The Bonebridge bone conduction implant has a magnet, an internal transducer, and an external audio processor. This report is of a 14-year-old girl with unilateral SNHL and vestibulocochlear nerve (VIII cranial nerve) aplasia due to dIAC who was treated with a Bonebridge bone conduction implant.
View Article and Find Full Text PDFObjective: This study aims to evaluate long-term auditory outcomes in patients with inner ear malformations (IEMs) treated with cochlear or auditory brainstem implants (CI/ABI), and to assess the influence of anatomical subtype, electrode design, insertion depth, and genetic/syndromic background on hearing performance over a 10-year follow-up.
Methods: We conducted a prospective cohort study including patients with radiologically confirmed IEMs and bilateral severe-to-profound hearing loss, all of whom underwent implantation and completed at least 10 years of follow-up. Outcomes were assessed using pure-tone average (PTA) and speech recognition scores (SRS) at defined intervals.
Curr Opin Otolaryngol Head Neck Surg
October 2025
Department of Otolaryngology Head and Neck Surgery, University of Vienna, Vienna, Austria.
Purpose Of Review: The recent successful otoferlin gene therapy trials have refocused the fields attention on the potential of gene therapy to cure hearing loss. With over 100 known monogenetic causes of hearing loss, the key question is which will be the next set of disorders that are treatable. The current review addresses potentially targetable hearing disorders that can be addressed with current gene therapy technologies.
View Article and Find Full Text PDF