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Diagn Interv Imaging 2022 Apr 22;103(4):225-229. Epub 2021 Oct 22.

Department of Neuroradiology, Lariboisière University Hospital, Assistance Publique Hôpitaux de Paris, 75010 Paris, France; Université de Paris, Faculté de Médecine, 75010 Paris, France. Electronic address:

Purpose: The purpose of this study was to compare the degree of perilymphatic enhancement between 4 hour post-contrast constant flip angle three-dimensional fluid attenuated inversion recovery (3D-FLAIR) images obtained with short repetition time (TR) and those obtained with long TR.

Materials And Methods: This single-center, prospective study included patients who underwent MRI of the inner ear with heavily T2-weighted sequence, 3D-FLAIR sequence with a "short" TR of 10,000 ms (s3D-FLAIR) and with a "long" TR of 16,000 ms (l3D-FLAIR). Signal intensity ratio (SIR) and contrast-to-noise ratio (CNR) obtained with s3D-FLAIR and l3D-FLAIR were quantitatively assessed using region of interest (ROI) method and compared. Read More

Vestn Otorinolaringol 2021 ;86(4):17-22

Moscow Regional Scientific Research Clinical Institute MF Vladimirsky, Moscow, Russia.

The Purpose Of The Study: To determine the value of the results of various audiological and vestibulometric studies for the differential diagnosis of non-inflammatory perilymphatic fistulas of the labyrinth windows (PFOLW).

Material And Methods: The retrospective and prospective analysis of the informative value of the results of audiological and vestibulometric studies was carried out in 124 people with different combinations of cochleovestibular complaints, who had different pathology of the inner and middle ear, with different terms of the disease - from several days to 30 years. To assess the informativeness of the applied testing, the following operational characteristics were determined: general sensitivity (Se), specificity (Sp) and the prognostic value of a positive result (PPV=positive predictive value). Read More

Continuum (Minneap Minn) 2021 04;27(2):468-490

Purpose Of Review: This article details updated clinical presentations and current treatment paradigms of the common otologic disorders that may present to the neurologist for vertigo, including Ménière disease, superior semicircular canal dehiscence syndrome, perilymphatic fistula, barotrauma, cholesteatoma, Ramsay Hunt syndrome, enlarged vestibular aqueduct syndrome, and autoimmune inner ear disease including Cogan syndrome.

Recent Findings: The recent data on modern imaging techniques with three-dimensional delayed IV contrast in Ménière disease, findings on the clinical and testing parameters to diagnose semicircular canal dehiscence and barotrauma, and clinical findings in Ramsay Hunt syndrome, cholesteatoma, and enlarged vestibular aqueduct syndrome are discussed in the article. The most recent findings on the treatment and evaluation of autoimmune inner ear disease and Cogan syndrome are also covered. Read More

Laryngoscope 2021 09 11;131(9):2088-2090. Epub 2021 May 11.

Department of Otolaryngology - Head and Neck Surgery, University of Washington, Seattle, Washington, U.S.A.

Intratympanic (IT) steroid therapy is a mainstay treatment for sudden sensorineural hearing loss (SSNHL) for both initial therapy and salvage therapy. We report a rare case of iatrogenic perilymphatic fistula that resulted from trauma during an IT steroid injection for SSNHL. We discuss the diagnosis and treatment in the current case and compare it with previous reports from the literature. Read More

J Int Adv Otol 2021 Mar;17(2):182-185

Department of Otolaryngology, Harvard Medical School, Boston, MA, USA;Department of Otolaryngology and Communication Enhancement, Boston Children's Hospital, Boston, MA, USA.

Traumatic perilymphatic fistula (PLF) is an uncommon cause of acute vestibular symptoms and hearing loss following head injury in children. We describe the management of 3 pediatric patients with traumatic PLF using an endoscopic ear surgery (EES) approach. Three pediatric patients with traumatic PLF underwent repair via an EES approach between August and October 2018. Read More

J Int Adv Otol 2021 Mar;17(2):156-161

Department of Otolaryngology-Head and Neck Surgery, Nara Medical University, Kashihara City, Nara, Japan.

It has been revealed that the pure-tone audiometry demonstrates large air-bone gaps at low pitches due to the presence of inner ear fistulae. When a third mobile window resulting from an inner ear fistula is present, in addition to the 2 normally present windows consisting of the oval window and the round window, a portion of the air-conducted waves escape from the scala vestibuli through the inner ear fistula. On the other hand, bone-conducted waves traveling to the scala vestibuli are reduced by an inner ear fistula; however, bone-conducted waves traveling to the scala tympani are not affected by an inner ear fistula. Read More

Otol Neurotol 2021 08;42(7):1051-1057

Michigan Ear Institute, Farmington Hills, MI.

Introduction: Oval window (OW) and round window (RW) reinforcement surgery has been used for symptomatic treatment of multiple clinical entities, most commonly perilymphatic fistula and superior semicircular canal dehiscence. Owing to the theoretical acoustically negative effect of stiffening the windows, there has been concern of an unfavorable effect on audiologic outcomes due to the procedure. The purpose of this study is to specifically evaluate audiologic outcomes after OW and RW reinforcement. Read More

Am J Otolaryngol 2021 Mar-Apr;42(2):102753. Epub 2020 Oct 17.

Department of Otolaryngology, Juntendo University Nerima Hospital, Tokyo, Japan; Department of Otolaryngology, Juntendo University, Tokyo, Japan; Department of Neurotology, Mejiro University, Saitama, Japan.

We introduce our horrible experience of lateral semicircular canal exposure due to unintended drilling during left facial nerve decompression. Nearly half of the canal was drilled-out, however, the membranous labyrinth was preserved and the defect was covered with temporal fascia. Immediately after surgery, the patient complained of vertigo with right beating nystagmus. Read More

Med Hypotheses 2020 Nov 28;144:110223. Epub 2020 Aug 28.

Department of Otorhinolaryngology-Head and Neck Surgery, Konkuk University Medical Center, Research Institute of Medical Science, Konkuk University School of Medicine, Seoul, Republic of Korea. Electronic address:

Chronic otitis media with cholesteatoma can cause erosion of the dense labyrinthine bone overlying the inner ear organs, and this labyrinthine fistula allows pressure or mass-induced motion of the underlying perilymphatic and endolymphatic compartment, evoking vestibular symptoms. While the mechanism of a positive fistula test, which is conducted by increasing or decreasing the external auditory canal pressure, has been well established, the mechanism underlying positional nystagmus in labyrinthine fistula has not been discussed yet. In the present study, we propose a new hypothesis accounting for positional nystagmus in labyrinthine fistula involving the lateral semicircular canal (LSCC), i. Read More

Front Neurol 2020 10;11:585747. Epub 2020 Nov 10.

Department of Otorhinolaryngology, Saitama Medical University, Saitama, Japan.

Recent third window syndrome studies have revealed that the intact bony labyrinth and differences in the stiffness of the oval and round windows are essential for proper cochlear and vestibular function. Herein we report a patient with a congenital dehiscence of the right stapes footplate. This dehiscence caused long-standing episodic pressure-induced vertigo (Hennebert sign). Read More

Front Neurol 2020 15;11:1046. Epub 2020 Sep 15.

Department of Otolaryngology-Head and Neck Surgery, University of California, Irvine, CA, United States.

A perilymphatic fistula (PLF) is an abnormal communication between the perilymph-filled inner ear and the middle ear cavity, mastoid, or intracranial cavity. A PLF most commonly forms when the integrity of the oval or round window is compromised, and it may be trauma-induced or may occur with no known cause (idiopathic). Controversy regarding the diagnosis of idiopathic PLF has persisted for decades, and the presenting symptoms may be vague. Read More

Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2020 Sep;34(9):857-861

Perilymphatic fistula（PLF） is defined as an abnormal communication between the fluid（perilymph） -filled space of the inner ear and the air-filled space of the middle ear and mastoid, or cranial spaces. At present the diagnostic criteria for perilymphatic fistula is the fistula hole confirmed by the microscope and endoscope between the middle ear and inner ear, the hole is located in the round or oval window, fractured bony labyrinth, microfissures, anomalous footplate, and can occur after head trauma or barotrauma, chronic inflammation, or in otic capsule dehiscence. Recently, the cochlin-tomoprotein（CTP） detected from the middle ear. Read More

Laryngoscope 2021 04 17;131(4):E1301-E1307. Epub 2020 Aug 17.

Department of Otorhinolaryngology, The Jikei University School of Medicine, Tokyo, Japan.

Objectives/hypothesis: Disturbed perilymph behind a labyrinth fistula can lead to hearing deterioration; thus, delicate manipulation is required during surgery for cholesteatomatous fistulae with matrix extension to the perilymphatic space (EPS). However, it remains challenging to identify the EPS preoperatively. This study aimed to evaluate the diagnostic value of computed tomography (CT) for preoperative prediction of the EPS of cholesteatomatous fistulae. Read More

J Laryngol Otol 2020 Jun;134(6):501-508

Department of Otolaryngology, Hannover Medical School, Hannover, Germany.

Objective: The diagnostic value of exploratory tympanotomy in sudden sensorineural hearing loss remains controversial. This study and review were performed to identify the incidence of perilymphatic fistula in patients with sudden sensorineural hearing loss. The effectiveness of tympanotomy for sealing of the cochlear windows in cases with perilymphatic fistula was evaluated. Read More

Eur Radiol 2020 Nov 28;30(11):6303-6310. Epub 2020 May 28.

Otology and Oto-neurosurgery Department, Salengro Hospital, CHU Lille, 59037, Lille, France.

Objectives: The aim of this study is to assess the diagnostic performance of a new MR sign, named the round window sign (RWS), to diagnose perilymphatic fistula (PLF) in a population of patients with chronic cochleo-vestibular symptoms, classified as definite or probable Menière's disease (MD).

Methods: A total of 164 patients (mean age 52 ± 35 years) with chronic cochleo-vestibular symptoms underwent MRI, between 4 and 5 h after intravenous gadoteric acid injection (Dotarem®, 0.1 mmol/kg). Read More

Case Rep Otolaryngol 2020 9;2020:9476915. Epub 2020 Mar 9.

Department of Otolaryngology, Saitama Medical University, Saitama, Japan.

We present a case of perilymphatic fistula (PLF) with inner ear anomalies having sudden, progressive sensorineural hearing loss and describe the fistula repair surgeries. We focus on the diagnosis methods of PLF and clinical course of PLF with inner ear anomaly. The cochlin-tomoprotein (CTP) detection test is very useful for the surgeons to encourage the earlier operation to sudden hearing loss cases. Read More

Eur Arch Otorhinolaryngol 2020 Apr 10;277(4):1045-1051. Epub 2020 Feb 10.

Service de Radiologie 1, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.

Background: We evaluated the usefulness of CT and MRI for the diagnosis of perilymphatic fistula (PLF) of the round (RW) and/or oval (OW) windows, with surgery as gold standard.

Methods: We retrospectively enrolled 17 patients who presented a surgically confirmed PLF of the round (RW) or oval (OW) windows. All patients were imaged by CT + MRI (T2W SSFP without contrast) prior to surgery (= gold standard). Read More

Acta Otolaryngol 2020 Apr 21;140(4):262-269. Epub 2020 Jan 21.

Department of Head and Neck Surgery, Lariboisière University Hospital, Paris, France.

Delayed 3D-FLAIR sequences enable the distinction between the utricle and the saccule. We sought to evaluate the clinical and radiological findings in patients with no visible saccule (NVS) on 4-hour post-contrast MRI. We retrospectively assessed the presence of NVS signs in 400 patients who underwent delayed inner ear MRI. Read More

Vestn Otorinolaringol 2019 ;84(3):61-64

ENT department of Rostov State Medical University, Rostov-on-Don, Russia, 344022.

A case of perilymphatic fistula with the luxation of the stapes into vestibule was described, which was caused by a direct damage of the eardrum (the patient cleaned the right ear with a cotton swab and received a strong blow to the arm). The moment of injury was characterized by severe pain, a significant hearing loss, the appearance of tympanophonia and vertigo. Clinical examination revealed the eardrum rupture. Read More

Clin Pract Cases Emerg Med 2019 May 4;3(2):115-118. Epub 2019 Mar 4.

David Geffen School of Medicine at the University of California Los Angeles, Department of Head and Neck Surgery, Los Angeles, California.

Pneumolabyrinth, defined as air within the labyrinth on high-resolution computed tomography, suggests that a perilymphatic fistula (PLF) is present. PLF describes an abnormal communication between the middle and inner ear, and can result in deafness, vertigo, and imbalance. In the setting of a penetrating injury to the temporal bone or inner ear, pneumolabyrinth should trigger prompt otolaryngology consultation and urgent surgical exploration. Read More

Adv Otorhinolaryngol 2019 15;82:93-100. Epub 2019 Jan 15.

Perilymphatic fistulas (PLF) and superior semi-circular canal dehiscence syndrome (SCDS) are 2 conditions that can present with sound and/or pressure-induced vertigo. PLF should be suspected in cases of trauma or surgery, while a spontaneous PLF is a diagnosis of exclusion. Research is ongoing to identify an ideal biomarker for perilymph. Read More

Otol Neurotol 2019 04;40(4):e356-e363

Department of Otorhinolaryngology-Head and Neck Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul.

Objective: To analyze surgical results on hearing and vestibular symptoms in patients with barotraumatic perilymphatic fistula (PLF) according to diagnostic criteria.

Methods: A total of 39 patients (41 ears) who underwent surgery on suspicion of barotraumatic PLF from January 2005 to December 2017 were included. Pure tone audiometry and videonystagmography (VNG) recording for spontaneous nystagmus and positional tests were performed preoperatively and postoperatively at 1 week and 1 month. Read More

BMJ Case Rep 2019 Mar 1;12(3). Epub 2019 Mar 1.

Otorhinolaryngology - Head & Neck Surgery, Hospital de Braga, Braga, Portugal.

Post-traumatic pneumolabyrinth is an uncommon clinical entity, particularly in the absence of temporal bone fracture. We report the case of a patient who presented to our emergency department with a headache, sudden left hearing loss and severe dizziness which began after a traumatic brain injury 3 days earlier. On examination, the patient presented signs of left vestibulopathy, left sensorineural hearing loss and positive fistula test, normal otoscopy and without focal neurological signs. Read More

Front Neurol 2019 30;10:47. Epub 2019 Jan 30.

Department of Otolaryngology, Kindai University Faculty of Medicine, Osaka, Japan.

An 8-year-old boy was referred to the ENT department for further evaluation of right-sided conductive hearing loss. A small cyst anterior to the oval window and fixation of the stapes footplate were observed during an exploratory tympanotomy. The concentration of a perilymph-specific protein, cochlin-tomoprotein (CTP), in the middle ear lavage fluid was measured with an ELISA-based CTP detection kit. Read More

Adv Otorhinolaryngol 2018 6;81:133-145. Epub 2018 Apr 6.

Perilymphatic fistula (PLF) is defined as an abnormal communication between the fluid (perilymph)-filled space of the inner ear and the air-filled space of the middle ear and mastoid, or cranial spaces. PLF is located in the round or oval window, fractured bony labyrinth, microfissures, anomalous footplate, and can occur after head trauma or barotrauma, chronic inflammation, or in otic capsule dehiscence. This clinical entity was initially proposed more than a century ago, yet it has remained a topic of controversy for more than 50 years. Read More

J Int Adv Otol 2018 Apr;14(1):106-111

Department of Neurosciences, Institute of Otolaryngology, Padova University School of Medicine, Padova, Italy.

The goal of the present study was to describe a case of perilymphatic fistula (PLF) of the round window (RW) that occurred after sneezing, along with a review of the literature. We report a case of PLF of RW, which was provoked by sneezing, and its consequent medical and surgical treatments. With respect to the review of the literature, articles were initially selected based on their titles or abstracts, followed by methodological evaluation. Read More

Am J Otolaryngol 2018 Jan - Feb;39(1):46-49. Epub 2017 Oct 9.

Department of Otolaryngology - Head and Neck Surgery, Soroka University Medical Center and the Faculty of Health Sciences, Ben-Gurion University in the Negev, Beer-Sheva, Israel. Electronic address:

Purpose: The purpose of this study was to describe the role of explorative tympanotomy in patients with Profound Sudden Sensorineural Hearing Loss (SSNHL) without clinical evidence of perilymphatic or labyrinthine fistula and to compare intraoperative findings with the postoperative hearing outcome.

Study Design: Retrospective study of all patients diagnosed with SSNHL who underwent explorative tympanotomy between 2002 and 2005.

Settings: Tertiary care university-affiliated hospital. Read More

Diagn Interv Imaging 2017 Oct 20;98(10):699-706. Epub 2017 Jun 20.

Department of neuroradiology and MRI, Grenoble Alpes university hospital-SFR RMN neurosciences, 38000 Grenoble, France; University Grenoble Alpes, IRMaGe, 38000 Grenoble, France.

Purpose: The goal of this retrospective study was to investigate the differential diagnosis of endolymphatic hydrops in patients with Meniere's disease (MD) symptoms by using magnetic resonance imaging (MRI) with intravenous injection of gadolinium chelate and delayed acquisition.

Material And Method: Two hundred patients (133 women, 67 men; mean age=67.2±11 ([SD] years) with unilateral MD underwent MRI at 3-T, between 4. Read More

Acta Otolaryngol 2017 3;137(sup565):S53-S59. Epub 2017 Apr 3.

a Department of Otorhinolaryngology , Saitama Medical University , Moroyama , Japan.

Objective: To investigate the positive rate for the Cochlin tomo-protein (CTP: an inner ear-specific protein) detection test among patients with inner ear-related clinical manifestations and evaluate the clinical characteristics of definite perilymphatic fistula (PLF).

Methods: We have performed an ELISA-based CTP detection test using middle ear lavage (MEL) samples from 497 cases of suspected PLF enrolled from 70 clinical centers nationwide between 2014 and 2015. In addition to the CTP-positive rate, audio-vestibular symptoms were compared between CTP-positive and -negative cases. Read More