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CASE REPORT article

Front. Oncol.
Sec. Neuro-Oncology and Neurosurgical Oncology
Volume 14 - 2024 | doi: 10.3389/fonc.2024.1443370
This article is part of the Research Topic Molecular Biology and Treatment Strategies for Tumors of Middle and Inner Ear View all 3 articles

Cochlear implantation in deafness due to an endolymphatic sac tumor in von Hippel-Lindau syndrome patients

Provisionally accepted
Oskar Rosiak Oskar Rosiak 1*Nikodem Pietrzak Nikodem Pietrzak 1Katarzyna Jankowska Katarzyna Jankowska 1Andrzej Kucharski Andrzej Kucharski 2Wiesław Konopka Wiesław Konopka 1Marcin Szymanski Marcin Szymanski 2
  • 1 Department of Otolaryngology, Polish Mother’s Memorial Hospital Research Institute, Łódź, Poland
  • 2 Department of Otolaryngology Head and Neck Surgery, Medical University of Lublin, Lublin, Lublin, Poland

The final, formatted version of the article will be published soon.

    Introduction: Endolymphatic sac tumors (ELSTs) are rare neuroectodermal neoplasms originating in the endolymphatic sac and duct, exhibiting no specific age or gender predilection, albeit more prevalent in Von Hippel-Lindau syndrome patients. Material and Methods: Manuscript preparation adhered to CARE guidelines for clinical case standardization and PRISMA guidelines for scientific reviews. Three studies meeting inclusion criteria were selected from analysis of 15 articles, supplemented by two additional studies identified through citation review. Results: The presented case describes a 16-year-old male diagnosed with unilateral sensorineural hearing loss secondary to bilateral ELST. Surgical excision of the right ear tumor with simultaneous cochlear implantation was performed, followed by left ear surgery. Hearing restoration was achieved in the implanted right ear, with normal hearing in the left ear, and no postoperative vestibular symptoms observed. Conclusion: Prompt surgical intervention remains the cornerstone of ELST treatment. Cochlear implantation, though sporadically reported in scientific literature, presents an option for hearing improvement in patients afflicted with deafness due to the disease.

    Keywords: Endolymphatic sac tumor, Hearing Loss, Deafness, Von Hippel Lindau, Cochlear Impalnt

    Received: 03 Jun 2024; Accepted: 21 Oct 2024.

    Copyright: © 2024 Rosiak, Pietrzak, Jankowska, Kucharski, Konopka and Szymanski. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence: Oskar Rosiak, Department of Otolaryngology, Polish Mother’s Memorial Hospital Research Institute, Łódź, Poland

    Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.