Intracochlear schwannomas (ICS) are very rare benign tumours of the inner ear. We present histopathological proof of the extremely rare bilateral occurrence of intracochlear schwannomas with negative blood genetic testing for neurofibromatosis type 2 (NF2). Bilateral schwannomas are typically associated with the condition NF2 and this case is presumed to have either mosaicism for NF2 or sporadic development of bilateral tumours. For progressive bilateral tumour growth and associated profound hearing loss, surgical intervention via partial cochleoectomy, tumour removal, preservation of the modiolus, and simultaneous cochlear implantation with lateral wall electrode carrier with basal double electrode contacts was performed. The right side was operated on first with a 14-month gap between each side. The hearing in aided speech recognition for consonant-nucleus-consonant (CNC) phonemes in quiet improved from 57% to 83% 12 months after bilateral cochlear implantation (CI). Bilateral intracochlear schwannomas in non-NF2 patients are extremely rare but should be considered in cases of progressive bilateral hearing loss. Successful tumour removal and cochlear implantation utilizing a lateral wall electrode is possible and can achieve good hearing outcomes.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10663204 | PMC |
| http://dx.doi.org/10.1007/s00106-023-01379-7 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Pediatric Intracochlear Schwannoma: Case Series and Review of the Literature.
J Int Adv Otol
November 2024
The Department of Medicine and Surgery, University of Parma, Parma, Italy.
Background: Intracochlear schwannomas (ICSs) are a subtype of intralabyrinthine schwannomas, completely located in the cochlear lumen. ICSs are particularly rare in the pediatric population. Putative diagnosis is made on the basis of magnetic resonance findings with signal characteristics that should remain the same at follow-up imaging.
View Article and Find Full Text PDFClinico-Radiological Manifestations of Cochlear Schwannomas.
J Audiol Otol
October 2024
Departments of Otorhinolaryngology, Yonsei University College of Medicine, Seoul, Korea.
Background And Objectives: Cochlear schwannomas, which are categorized into intracochlear and intravestibulocochlear schwannomas (ICs and IVCs, respectively) are rare and may cause hearing loss (HL). The affected region is invariably correlated with tumor location, which can be detected on magnetic resonance imaging (MRI). We describe the cochleovestibular manifestations of ICs and IVCs.
View Article and Find Full Text PDFRevised Classification of Inner Ear Schwannomas.
Otol Neurotol
January 2025
Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota, USA.
Article Synopsis
- Over the past two decades, there has been a growing focus on diagnosing and managing inner ear schwannomas, especially regarding hearing rehabilitation through cochlear implants, but the terminology used to classify these tumors has been inconsistent and complex.
- At the Ninth Quadrennial Conference in May 2023 in Bergen, Norway, experts reached a consensus to standardize terminology, ultimately agreeing on the term "inner ear schwannoma (IES)" to clearly describe tumors affecting the eighth nerve in the cochlea, vestibule, or semicircular canals.
- The newly established classifications break down these tumors into three complexity levels: low (intravestibular, intracochlear), intermediate (with internal
Detecting endolymphatic hydrops in patients with cranial nerve VIII schwannoma using hydrops MR examination.
Eur Arch Otorhinolaryngol
October 2024
Departments of Otolaryngology, National Taiwan University Hospital, Taipei, Taiwan.
Purpose: This study utilized Hydrops MRI in patients with cranial nerve (CN) VIII schwannoma to assess the concomitance with endolymphatic hydrops (EH), aiming to elucidate the mechanism of hydrops formation in these patients.
Methods: Twenty-six patients diagnosed as CN VIII schwannoma including vestibular schwannoma (VS) in 24 and intracochlear schwannoma (ICS) in 2 were enrolled. Fifteen patients received radiosurgery and 11 patients opted for a wait-and-scan approach.
Electrocochleography-Based Tonotopic Map: I. Place Coding of the Human Cochlea With Hearing Loss.
Ear Hear
December 2024
Department of Otolaryngology-Head and Neck Surgery, Washington University School of Medicine in St. Louis, St Louis, Missouri, USA.
Article Synopsis
- - The study aims to map the tonotopy (frequency organization) of the human cochlea in vivo using cochlear implant electrodes, addressing challenges of past research that relied on cadaver and animal models.
- - Fifty patients with hearing loss underwent cochlear implantation, where their responses to sound stimuli were recorded to analyze how sound intensity and an artificial "third window" influence the tonotopic map.
- - Results showed notable deviations from the expected Greenwood model in the frequency-position function, especially at higher sound levels, indicating complexities in how the cochlea processes sound intensity.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!