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Objective: Intralabyrinthine schwannomas (ILSs) are a rare cause of deafness. Patients with ILS confined to the semicircular canals and the vestibule (intravestibular schwannomas) are potential candidates for cochlear implantation for hearing rehabilitation, a new option for patients with unilateral hearing loss since the 2019 FDA approval of cochlear implant (CI) for single-sided deafness. In this report, we describe an evolving management approach for ILSs causing hearing loss.
Patients: Adults (≥18 years) who underwent simultaneous ILS resection and CI between January 2019 and June 2023 (n = 3).
Intervention: Transmastoid labyrinthectomy with simultaneous cochlear implantation.
Main Outcome Measures: Hearing performance with cochlear implantation measured as CNC Word Recognition scores and AzBio Sentence scores.
Results: Three patients with ILS confined to the semicircular canals and vestibule underwent simultaneous tumor resection via labyrinthectomy with CI placement. In all cases, complete tumor resection and full CI insertion were achieved. No patients experienced postoperative complications. Patients 1 and 2 underwent 6- and 9-month postactivation testing, respectively, with CNC scores 64% to 80% and AzBio 81% to 99% in the implanted ears. Patient 3 scored 0% on CNC and AzBio testing at 3 months and deferred her 6-month audiometry.
Conclusions: Patients with ILS confined to the vestibule and semicircular canals can be considered for simultaneous tumor resection and CI placement.
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http://dx.doi.org/10.1097/MAO.0000000000004140 | 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.
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