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Perilymphatic fistula with characteristic findings of the inner ear by contrast-enhanced magnetic resonance imaging: a case report

A perilymphatic fistula (PLF) presents with abnormal traffic in the otic capsule, causing cochlear and vestibular symptoms. However, the mechanisms underlying symptom recurrence remain controversial. Herein, we report the case of a 27-year-old female who complained of hearing disturbance in her righ...

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Autores principales: Ito, Yusuke, Seo, Toru, Sasano, Yoshiyuki, Mochizuki, Fumihiro, Koizuka, Izumi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10625405/
https://www.ncbi.nlm.nih.gov/pubmed/37928144
http://dx.doi.org/10.3389/fneur.2023.1276991
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author Ito, Yusuke
Seo, Toru
Sasano, Yoshiyuki
Mochizuki, Fumihiro
Koizuka, Izumi
author_facet Ito, Yusuke
Seo, Toru
Sasano, Yoshiyuki
Mochizuki, Fumihiro
Koizuka, Izumi
author_sort Ito, Yusuke
collection PubMed
description A perilymphatic fistula (PLF) presents with abnormal traffic in the otic capsule, causing cochlear and vestibular symptoms. However, the mechanisms underlying symptom recurrence remain controversial. Herein, we report the case of a 27-year-old female who complained of hearing disturbance in her right ear and recurrent vertigo after sudden onset of hearing loss with vertigo. The caloric test revealed unilateral weakness in the right ear, and the video head impulse test (vHIT) showed decreased vestibulo-ocular reflex (VOR) gain. Contrast-enhanced magnetic resonance imaging (MRI) using hybrid of reversed image of positive endolymph signal and negative image of perilymph signal (HYDROPS) indicated a collapsed endolymphatic space. As the vestibular symptoms did not improve, an exploratory tympanotomy was performed on the right ear. Although perilymph leakage was not noted in the oval or round windows, both windows were sealed with connective tissue. The patient's vestibular symptoms rapidly improved after surgery, and postoperative contrast-enhanced MRI showed improvement in the collapsed endolymphatic space. Although the caloric test revealed unilateral weakness, the VOR gain on the vHIT improved to normal on the right side. Thus, these findings indicated that recurrent symptoms caused by PLF are associated with a collapsed endolymphatic space. We speculate that the collapsed endolymphatic space was due to a ruptured Reissner's membrane. We hypothesized that sealing the fistula would promote normalization of perilymph pressure. The ruptured Reissner's membrane may have been gradually repaired as vestibular symptoms improved. This case adds to the existing literature on the occurrence of the “double-membrane break syndrome”. Collapse of the endolymph due to a ruptured Reissner's membrane may be the cause of PLF symptoms.
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spelling pubmed-106254052023-11-05 Perilymphatic fistula with characteristic findings of the inner ear by contrast-enhanced magnetic resonance imaging: a case report Ito, Yusuke Seo, Toru Sasano, Yoshiyuki Mochizuki, Fumihiro Koizuka, Izumi Front Neurol Neurology A perilymphatic fistula (PLF) presents with abnormal traffic in the otic capsule, causing cochlear and vestibular symptoms. However, the mechanisms underlying symptom recurrence remain controversial. Herein, we report the case of a 27-year-old female who complained of hearing disturbance in her right ear and recurrent vertigo after sudden onset of hearing loss with vertigo. The caloric test revealed unilateral weakness in the right ear, and the video head impulse test (vHIT) showed decreased vestibulo-ocular reflex (VOR) gain. Contrast-enhanced magnetic resonance imaging (MRI) using hybrid of reversed image of positive endolymph signal and negative image of perilymph signal (HYDROPS) indicated a collapsed endolymphatic space. As the vestibular symptoms did not improve, an exploratory tympanotomy was performed on the right ear. Although perilymph leakage was not noted in the oval or round windows, both windows were sealed with connective tissue. The patient's vestibular symptoms rapidly improved after surgery, and postoperative contrast-enhanced MRI showed improvement in the collapsed endolymphatic space. Although the caloric test revealed unilateral weakness, the VOR gain on the vHIT improved to normal on the right side. Thus, these findings indicated that recurrent symptoms caused by PLF are associated with a collapsed endolymphatic space. We speculate that the collapsed endolymphatic space was due to a ruptured Reissner's membrane. We hypothesized that sealing the fistula would promote normalization of perilymph pressure. The ruptured Reissner's membrane may have been gradually repaired as vestibular symptoms improved. This case adds to the existing literature on the occurrence of the “double-membrane break syndrome”. Collapse of the endolymph due to a ruptured Reissner's membrane may be the cause of PLF symptoms. Frontiers Media S.A. 2023-10-19 /pmc/articles/PMC10625405/ /pubmed/37928144 http://dx.doi.org/10.3389/fneur.2023.1276991 Text en Copyright © 2023 Ito, Seo, Sasano, Mochizuki and Koizuka. https://creativecommons.org/licenses/by/4.0/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) and the copyright owner(s) 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.
spellingShingle Neurology
Ito, Yusuke
Seo, Toru
Sasano, Yoshiyuki
Mochizuki, Fumihiro
Koizuka, Izumi
Perilymphatic fistula with characteristic findings of the inner ear by contrast-enhanced magnetic resonance imaging: a case report
title Perilymphatic fistula with characteristic findings of the inner ear by contrast-enhanced magnetic resonance imaging: a case report
title_full Perilymphatic fistula with characteristic findings of the inner ear by contrast-enhanced magnetic resonance imaging: a case report
title_fullStr Perilymphatic fistula with characteristic findings of the inner ear by contrast-enhanced magnetic resonance imaging: a case report
title_full_unstemmed Perilymphatic fistula with characteristic findings of the inner ear by contrast-enhanced magnetic resonance imaging: a case report
title_short Perilymphatic fistula with characteristic findings of the inner ear by contrast-enhanced magnetic resonance imaging: a case report
title_sort perilymphatic fistula with characteristic findings of the inner ear by contrast-enhanced magnetic resonance imaging: a case report
topic Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10625405/
https://www.ncbi.nlm.nih.gov/pubmed/37928144
http://dx.doi.org/10.3389/fneur.2023.1276991
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