Perilymphatic Fistula Research Articles

Perilymphatic fistula: a historical overview of publication trends

BackgroundPerilymph fistula (PLF) can cause symptoms of dizziness, vertigo, and fluctuating hearing. We hypothesized that publications on PLF have decreased in recent years relative to other inner ear disorders with overlapping symptoms.MethodsWe performed a Scopus search using the terms “perilymphatic fistula” OR “perilymph fistula,” limited to original studies or reviews involving human subjects published in English before 2022. We noted the senior author's institution, country affiliation, and publishing journal. The Kendall rank correlation coefficient test was used to analyze the trend of each variable over the past 30 years. Using the same search strategy, we compared these data to those from vestibular neuritis (VN), Meniere's disease (MD), superior semicircular canal dehiscence (SSCD), and benign paroxysmal positional vertigo (BPPV).ResultsSix hundred and ninety-eight PLF publications were returned. The top publishing country was the United States (n = 334), the top publishing journal was the American Journal of Otology/Otology and Neurotology (n = 68), and the top publishing institution was the University of Pittsburgh (n = 15). In the past 30 years (n = 501), there was no trend in the frequency of annual PLF publications (τ = −0.150, p = 0.265). Still there were positive trends in publications on vestibular neuritis (τ = 0.724, p < 0.001), Meniere's disease (τ = 0.587, p < 0.001), superior canal dehiscence syndrome (τ = 0.840, p < 0.001), and benign paroxysmal positional vertigo (τ = 0.882, p < 0.001) 5,398 PLF cases were identified, of which 4,356 specified the etiology; the majority identified an inciting insult (74.8%, n = 3,257).ConclusionsThe absence of diagnostic criteria for PLF may hinder its appeal to researchers as similar syndromes have had diagnostic consensus documents set forth. Given the greater number of cases with inciting insults, efforts to define a diagnostic criterion should focus on this subtype.

Ear diseases due to diving with an aqualung

Due to the growing popularity of diving, more and more people suffer from injuries related to this activity and ear injuries are among the most common ones. During diving, dysfunction of both the external, middle and inner ear are likely to occur. Inflammation is the most common disease of the external ear, which, in most cases, requires only local treatment with ear drops. Barotrauma of the middle and inner ear may occur due to Eustachian tube failure and difficulties in equalizing underwater pressure, also manifested as hearing loss and dizziness. In most cases, conservative treatment is applied, however, under specific conditions such as perforation of the tympanic membrane or the presence of persistent perilymphatic fistulas, surgical treatment may be necessary. In the case of severe dizziness or hearing loss during ascent after diving at great depths, a preliminary diagnosis of inner ear decompression sickness should be made and the patient should be placed in a hyperbaric oxygen chamber as a matter of urgency.

Audio-Vestibular Evaluation of Pediatric Pseudo-Conductive Hearing Loss: Third Window Syndromes.

Conductive hearing loss caused by external or middle ear problems prevents the transmission of sound waves from the external auditory canal to the cochlea, and it is a common condition, especially in pediatric patients aged 1-5 years. The most common etiological factors are otitis media and cerumen during childhood. In some patients, external and middle ear functions and structures may be normal bilaterally despite the air-bone gap on the audiogram. This condition, which is often a missed diagnosis in children, is defined as a pseudo-conductive hearing loss (PCHL) caused by third window syndromes (TWSs) such as semicircular canal dehiscence, inner ear malformations with third window effect, and perilymphatic fistula. In this review of the literature, the authors emphasize the pitfalls of pediatric audio-vestibular evaluation on TWSs as well as the key aspects of this evaluation for the differential diagnosis of PCHL brought on by TWSs. This literature review will provide audiologists and otologists with early diagnostic guidance for TWSs in pediatric patients.

Prognostic Factors in Idiopathic Sudden Sensorineural Hearing Loss: The Experience of Two Audiology Tertiary Referral Centres.

Background and Objectives: Although different hypotheses have been proposed over time, there is a dearth of information on factors able to predict the response to treatment for idiopathic sudden sensorineural hearing loss (ISSNHL) and hearing recovery. The aim of this study was to apply univariate and multivariate statistical models in a retrospective clinical setting of patients given therapy for ISSNHL at our tertiary academic audiological centers to investigate the prognostic value of clinical signs, symptoms, and comorbidities in relation to hearing recovery. Materials and Methods: The inclusion criteria were: history of ISSNHL diagnosed and treated at the Padova or Modena tertiary academic audiological centers; age ≥ 18 years; availability of clinical and audiological outcome data. The exclusion criteria were: hearing loss in acoustic schwannoma, endolymphatic hydrops, meningitis, trauma (head trauma, temporal bone fracture, acoustic trauma), barotrauma, perilymphatic fistula; exposure to noise levels ≥ 80 dB in the work environment; any unilateral or bilateral hearing loss (except for presbycusis) prior to ISSNHL diagnosis; any disorders affecting the external or middle ear; any previous ear surgery; refusal to make medical data available for research purposes. Eighty-six consecutive patients (38 females, 48 males; median age: 58 years; interquartile range: 47.00-69.00 years) were included. A systemic steroid therapy was administered to all patients, either orally with prednisone or intravenously with methylprednisolone. Second-line therapy included intratympanic steroid injections and/or hyperbaric oxygen therapy. Results: A multivariate logistic regression model was used, including the non-multicollinear clinical and audiological variables, which showed a p-value < 0.10 at the univariate analyses (namely age at diagnosis, time to diagnosis, oral steroid dose, and PTA on the affected side). Only PTA on the affected side retained its statistical significance (OR: 1.0615, 95% CI: 1.0185-1.1063, p = 0.005). Conclusions: The analysis of our data showed an association between the hearing threshold before treatment and the recovery from ISSNHL. Further studies on larger cohorts (especially in a prospective setting) are needed to shed more light on the prognostic role of clinical parameters in patients with ISSNHL. In a correct counseling setting, with regard to the patient's concern about not being able to recover hearing, it is important to offer perspectives of appropriate hearing rehabilitation approaches.

MRI-Based Inner Ear Assessment and Cochlin Tomoprotein-Based Evaluation of Perilymphatic Fistula in Patients with Sudden Hearing Loss.

To study the correlation between positive cochlin tomoprotein testing (CTP), magnetic resonance (MR) imaging, and the auditory and vestibular function amongst patients with sudden hearing loss. Prospective case series. We prospectively examined eight patients who presented with sudden hearing loss (>60 dB) with or without vertigo or tinnitus. We performed an ELISA-based CTP detection test using middle ear lavage samples. In addition to the CTP examination, a magnetic resonance imaging (MRI) examination was performed using different sequences (T1 and a T1 sequence with a contrast medium (CM), a T2 sequence, 4 h delayed intravenous gadolinium-enhanced three-dimensional fluid-attenuated inversion recovery (3D FLAIR)). All patients with sudden hearing loss (>60 dB) presented a non-specific contrast enhancement in the cochlea and vestibulum on the affected side on delayed 3D-FLAIR MRI. Four patients had a positive CTP test, suggesting a perilymphatic fistula (PLF). However, no specific MRI signal for a PLF was observed. Using multimodal diagnostic measures, such as CTP testing and different MRI sequences, no correlation could be found in patients with a PLF.

Factors Influencing Treatment Success in Cholesteatoma Management: A Cross-Sectional Study.

Background/Objectives: Cholesteatoma presents significant management challenges in otolaryngology. This study aimed to delineate the influence of demographic and clinical characteristics, preoperative imaging, and surgical approaches on treatment success in cholesteatoma management. Methods: A cross-sectional analytical study was conducted at the Otolaryngology Department of the University Hospital from January 2021 to December 2022. It included 68 patients diagnosed with cholesteatoma, focusing on three objectives: assessing the impact of demographic and clinical characteristics on treatment outcomes, evaluating the predictive value of preoperative imaging findings, and analyzing the influence of surgical factors. Results: The study population predominantly consisted of male (56%) and Saudi (81%) patients, with an average age of 45 years. Logistic regression revealed that older age (OR: 1.05), male gender (OR: 0.63), and non-Saudi Arab ethnicity (OR: 2.14) significantly impacted treatment outcomes. Clinical characteristics such as severe disease severity (OR: 3.00) and longer symptom duration (OR: 0.96) also influenced treatment success. In preoperative imaging, labyrinthine fistula (Regression Coefficient: 0.63) and epidural extension (Coefficient: 0.55) emerged as key predictors. The surgical factors that significantly affected the outcomes included the extent of surgery (Complete Removal OR: 3.32) and the use of endoscopic approaches (OR: 1.42). Conclusions: This study highlights that patient demographics, clinical profiles, specific preoperative imaging features, and surgical strategies multifactorially determine cholesteatoma treatment success. These findings suggest the necessity for a tailored approach in cholesteatoma management, reinforcing the importance of individualized treatment plans based on comprehensive preoperative assessments.

3D printed temporal bones for preoperative simulation and planning

ObjectiveDemonstrate the utility of 3D printed temporal bone models in individual patient preoperative planning and simulation. Methods3D models of the temporal bone were made from 5 pediatric and adult patients at a tertiary academic hospital with challenging surgical anatomy planned for cochlear implantation or exteriorization of cholesteatoma with complex labyrinthine fistula. The 3D models were created from CT scan used for preoperative planning, simulation and intraoperative reference. The utility of models was assessed for ease of segmentation and production and impact on surgery in regard to reducing intraoperative time and costs, improving safety and efficacy. ResultsThree patients received cochlear implants, two exteriorization of advanced cholesteatoma with fistulas (1 internal auditory canal/cochlea, 1 all three semicircular canals). Surgical planning and intraoperative referencing to the simulations by the attending surgeon and trainees significantly altered original surgical plans. In a case of X-linked hereditary deafness, optimal angles and rotation maneuvers for cochlear implant insertion reduced operating time by 93 min compared to the previous contralateral side surgery. Two cochlear implant cases planned for subtotal petrosectomy approach due to aberrant anatomy were successfully approached through routine mastoidectomy. The cholesteatoma cases were successfully exteriorized without necessitating partial labyrinthectomy or labyrinthine injury. There were no complications. Conclusion3D printed models for simulation training, surgical planning and use intraoperatively in temporal bone surgery demonstrated significant benefits in designing approaches, development of patient-specific techniques, avoidance of potential or actual complications encountered in previous or current surgery, and reduced surgical time and costs.

The Safety and Efficacy of Hydroxyapatite Repair of Cholesteatoma Skull Base Defects.

To investigate the possibility of hydroxyapatite as a safe and effective alternative to currently used bioavailable materials for repair of tegmen defects and labyrinthine fistulas in the setting of cholesteatoma. Retrospective study. Tertiary-level-care hospital. Electronic medical records of patients 18+ years undergoing cholesteatoma-removal surgery between 2013 and 2022 were reviewed. Twenty-two patients diagnosed with cholesteatoma who underwent repair of either a tegmen defect or labyrinthine fistula using hydroxyapatite were evaluated. There were 17 canal wall up (CWU) and 5 canal wall down (CWD) surgeries. The cholesteatoma recidivism rate was 18.2% (n = 4) and the recurrence rate was 4.5% (n = 1). To ensure that these rates were similar to cholesteatoma-removal surgeries in which hydroxyapatite was not used, a 22 age, gender, and operative technique-matched cohort was evaluated. For patients with CWU surgeries, the rate of recurrence and recidivism were identical between both cohorts (0% and 23.5%, respectively; P = 1 for both). While in CWD surgeries, there was a nonstatistically significant difference in the recurrence as there was only 1 patient with recurrence in the hydroxyapatite group (P = 1). Three (13.5%) patients in the hydroxyapatite group had a local infection and 1 (4.5%) had a subacute mastoid infection. All patients with semicircular canal fistulas had consistent bone lines on postoperative audiograms, with no worsening sensorineural hearing loss. In our cohort, hydroxyapatite was safe and successful in repairing skull base defects and inner-ear fistulas in the setting of cholesteatoma with a low rate of postoperative infection and no evidence of a higher rate of cholesteatoma recurrence. Further studies are needed to assess population generalizability.

A Case of Middle Ear Adenomatous Neuroendocrine Tumors: Proposal of a Staging System

A 49-year-old man visited a local otolaryngology clinic with left hearing loss. He was diagnosed as left middle ear tumor and referred to us. The tumor was protruding from the left tympanic membrane. Computed tomography (CT) revealed thickening of the tympanic membrane, space-occupying lesions in the middle ear cavity and mastoid air cells, and no bone destruction in the left middle ear. Magnetic resonance imaging confirmed the CT findings, and the lesions were hyperintense on T1- and T2-weighted imaging. He was diagnosed as middle ear adenomatous neuroendocrine tumor (MEANT) with a pathological result. The patient underwent postauricular approach and canal wall-down mastoidectomy. The tumor occupied the mastoid air cells and extended into the middle ear mucosa. To prevent perilymphatic fistula, the tumor was excised as extensively as possible, while preserving the tumor around and adjacent to the stapes footplate. MEANT is extremely rare and long-term follow-up is necessary due to be potential for tumor regrowth or recurrence. Herein, we propose a new staging system for MEANT.

Postoperative Late Hearing Deterioration in Cholesteatoma with Labyrinthine Fistulas.

A labyrinthine fistula is a severe complication of middle ear cholesteatoma that can cause profound sensorineural hearing loss and vertigo. However, there is no consensus regarding the transition to postoperative hearing. Although hearing deteriorates gradually with a delay in some cases of labyrinthine fistula, insufficient consideration has been given to this point. We examined perioperative changes in cases of middle ear cholesteatoma with labyrinthine fistulas. We retrospectively reviewed the medical records of 578 patients with middle ear cholesteatoma who underwent tympanoplasty at our hospital between 2016 and 2021. Patients with labyrinthine fistulas were selected; their perioperative bone-conduction hearing was assessed. Fistula depth was determined following the classification reported by Dornhoffer et al. The hearing was compared preoperatively, early postoperatively (3-6 months), and 1 year postoperatively. Forty-eight patients (8.3%) had labyrinthine fistulas. Regarding depth, 21 cases were type I, 14 were type IIa, 3 were type IIb, and 10 were type III. Preoperative bone-conduction hearing was significantly poor in invasion type IIb or deeper cases. Cases with type IIb or deeper fistulas, multiple fistulas, or vertigo deteriorated postoperatively. Type III cases or those with multiple fistulas deteriorated further from the early postoperative period to 1 year postoperatively. Concerning frequency, 500 and 2000 Hz showed a delayed deterioration. This is a valuable report of delayed hearing loss after surgery in patients with a labyrinthine fistula. This change is associated with the labyrinthine fistula's depth and multiple fistulas-this is important during preoperative counseling of patients undergoing surgery.

Clinical course of five patients definitively diagnosed with idiopathic perilymphatic fistula treated with transcanal endoscopic ear surgery.

An idiopathic perilymphatic fistula (PLF) can be difficult to diagnose because patients present with sudden sensorineural hearing loss (SSHL) and/or vestibular symptoms without any preceding events. In such cases, we currently test for cochlin-tomoprotein (CTP) to confirm the diagnosis of idiopathic PLF because CTP is only detected in the perilymph. In this study, we report the clinical course of five patients definitively diagnosed with idiopathic PLF who underwent PLF repair surgery using transcanal endoscopic ear surgery (TEES). Five patients were initially treated with intratympanic dexamethasone for SSHL, at which time a CTP test was also performed (preoperative CTP test). Due to refractory hearing loss and/or fluctuating disequilibrium, PLF repair surgery using TEES was performed to seal the oval and round windows using connective tissue and fibrin glue. These patients were diagnosed with definite idiopathic PLF based on pre- or intra-operative CTP test results (negative, < 0.4 ng/mL; intermediate, 0.4-< 0.8 ng/mL; and positive, > 0.8 ng/mL). We evaluated pre- and intra-operative CTP values, intraoperative surgical findings via a magnified endoscopic view, and pre- and post-operative changes in averaged hearing level and vestibular symptoms. Pre- and intra-operative CTP values were positive and intermediate in three patients, positive and negative in one patient, and negative and positive in one patient. None of the patients had intraoperative findings consistent with a fistula between the inner and middle ears or leakage of perilymph. Only two patients showed a slight postoperative recovery in hearing. Four patients complained of disequilibrium preoperatively, of whom two had resolution of disequilibrium postoperatively. A positive CTP test confirms PLF in patients without obvious intraoperative findings. The CTP test is considered more sensitive than endoscopic fistula confirmation. We consider that CTP test results are important indicators to decide the surgical indication for idiopathic PLF repair surgery. In our experience with the five cases, two of them showed improvements in both hearing and vestibular symptoms.

Registration of preoperative temporal bone CT-scan to otoendoscopic video for augmented-reality based on convolutional neural networks.

Patient-to-image registration is a preliminary step required in surgical navigation based on preoperative images. Human intervention and fiducial markers hamper this task as they are time-consuming and introduce potential errors. We aimed to develop a fully automatic 2D registration system for augmented reality in ear surgery. CT-scans and corresponding oto-endoscopic videos were collected from 41 patients (58 ears) undergoing ear examination (vestibular schwannoma before surgery, profound hearing loss requiring cochlear implant, suspicion of perilymphatic fistula, contralateral ears in cases of unilateral chronic otitis media). Two to four images were selected from each case. For the training phase, data from patients (75% of the dataset) and 11 cadaveric specimens were used. Tympanic membranes and malleus handles were contoured on both video images and CT-scans by expert surgeons. The algorithm used a U-Net network for detecting the contours of the tympanic membrane and the malleus on both preoperative CT-scans and endoscopic video frames. Then, contours were processed and registered through an iterative closest point algorithm. Validation was performed on 4 cases and testing on 6 cases. Registration error was measured by overlaying both images and measuring the average and Hausdorff distances. The proposed registration method yielded a precision compatible with ear surgery with a 2D mean overlay error of mm for the incus and mm for the round window. The average Hausdorff distance for these 2 targets was mm and mm respectively. An outlier case with higher errors (2.3 mm and 1.5 mm average Hausdorff distance for incus and round window respectively) was observed in relation to a high discrepancy between the projection angle of the reconstructed CT-scan and the video image. The maximum duration for the overall process was 18s. A fully automatic 2D registration method based on a convolutional neural network and applied to ear surgery was developed. The method did not rely on any external fiducial markers nor human intervention for landmark recognition. The method was fast and its precision was compatible with ear surgery.

Suspicion and Treatment of Perilymphatic Fistula: A Prospective Clinical Study.

Background: Since the discovery of the perilymphatic fistula (PLF), the diagnosis and treatment remain controversial. If successfully recognized, the PLF is surgically repairable with an obliteration of the fistula site. Successful treatment has a major impact on patient's quality of life with an improvement in their audiological and vestibular symptoms. Objective: To prospectively investigate patients' clinical and audiological evolution with PLF suspicion after middle ear exploration and obliteration of the round and oval window. Study Design: Prospective comparative study. Setting: Tertiary care center. Methods: Patients were divided into two groups: Group I consisted of patients where no PLF had been identified intraoperatively at the oval and/or at the round window, and Group II consisted of patients where a fistula had been visualized. Patient assessment was a combination of past medical history, the presence of any risk factors, cochlear and vestibular symptoms, a physical examination, temporal bone imaging, audiograms, and a videonystagmogram (VNG). Results: A total of 98 patients were divided into two groups: 62 in Group I and 36 in Group II. A statistically significant difference regarding gender was observed in Group II (83.3% of males vs. 16.7% of females, p = 0.008). A total of 14 cases (4 and 10 in Groups I and II, respectively) were operated for a recurrent PLF. Fat graft material was used in the majority of their previous surgery; however, no difference was found when comparing fat to other materials. In addition, no statistically significant difference was noted between Groups I and II concerning predisposing factors, imaging, VNG, symptom evolution, or a physical exam before the surgery and at 12 months post-operative. However, both groups showed statistically significant hearing and vestibular improvement. On the other hand, the air conduction (AC) and bone conduction (BC) at each frequency were not statistically different between the two groups before surgery but showed statistically significant improvement at 12 months post-operatively, especially for the BC at the frequencies 250 (p = 0.02), 500 (p = 0.0008), and 1000 Hz (p = 0.04). Conclusions: Whenever you suspect a perilymphatic fistula, do not hesitate to explore middle ear and do window obliterations using a tragal perichondrium material. Our data showed that cochlear and vestibular symptoms improved whether a fistula had been identified or not.

Chronic Suppurative Otitis Media Accompanied by Facial Nerve Paresis: A Case Report

Introduction: Chronic suppurative otitis media (CSOM) is marked by ear discharge (otorrhea) that lasts for more than six to eight weeks, middle ear inflammation that doesn't go away, and a ruptured eardrum. The purpose of this study is to describe examples of CSOM with facial nerve paresis and how such cases are managed.&#x0D; Case presentation: A thirty-year-old man complained to the Mataram Community Health Center two days before admission that his mouth was turning to the left, despite the fact that he was still able to speak well. Additionally, for four months, the patient complained of a constant discharge from the right ear. The fluid that emerges from the ear is sticky, yellow, and odorous; blood is not mixed with it. Upon inspection, a cholesteatoma and a flat-edge attic perforation on the tympanic membrane were discovered, together with a purulent and odorous discharge in the ear canal of the right ear. After a facial nerve examination, it was discovered that the forehead wrinkles were uneven and that the face was asymmetrical, with the right eyebrow unable to be raised. The patient was identified as having peripheral facial nerve paresis and persistent suppurative malignant otitis media of the auricula dextra. The patient is being treated with irrigation of the ear canal with 0.9% NaCl, cleaning of the cholesteatoma, and antibiotic administration: two drops of ofloxacin ear drops given twice a day, three times a day ciprofloxacin tablets 500 mg, three times a day methylprednisolone 16 mg, and advice to undergo a mastoidectomy.&#x0D; Conclusion: Complications including hearing loss, facial nerve palsy, subperiosteal abscess, petrositis, meningitis, cerebral abscess, and labyrinthine fistula are indicated for mastectomy surgical therapy.

Prevalence of perilymphatic fistula in patients with sudden-onset sensorineural hearing loss as diagnosed by Cochlin-tomoprotein (CTP) biomarker detection: its association with age, hearing severity, and treatment outcomes

PurposeTo determine the prevalence of perilymphatic fistula (PLF) in sudden-onset sensorineural hearing loss (SSNHL) patients by employing the Cochlin-tomoprotein (CTP) detection test, a specific diagnostic marker for perilymph. We also analyzed the clinical characteristics associated with hearing outcomes in this cohort.MethodsA total of 74 eligible patients were prospectively enrolled. Following myringotomy, middle ear lavage (MEL) samples underwent the CTP test to identify perilymph leakage. Intratympanic dexamethasone (IT-DEX) therapy was administered, and hearing outcomes were assessed. Control groups comprised patients with chronic otitis media (n = 40) and non-inflammatory middle ears (n = 51) with concurrent MEL sample collection.ResultsCTP was positive in 16 (22%) patients. No control samples showed positive results. Multiple regression analysis indicated that age and pre-treatment hearing levels significantly contributed to the CTP value. We found a positive correlation between CTP values, age, and pre-treatment pure-tone averages. Notably, CTP values in SSNHL cases aged 60 and above were significantly higher than in those below 60 years. Patients with positive CTP had significantly worse recovery rates after IT-DEX treatment.ConclusionThis study is the first prospective investigation demonstrating a positive relationship between CTP values, age, and hearing severity in SSNHL, indicating that PLF might be the essential cause of SSNHL, particularly in the elderly. Our findings suggest that IT-DEX may be less effective for PLF-associated SSNHL. Future research could reveal that PLF repair surgery is a viable treatment strategy for SSNHL. This study was registered under the UMIN Clinical Trials Registry (UMIN000010837) on 30/May/2013.

Multi-Disciplinary Approach to Skull Base Paragangliomas.

The treatment of skull base paragangliomas has moved towards the use of cranial nerve preservation strategies, using radiation therapy and subtotal resection in instances when aiming for gross total resection would be expected to cause increased morbidity compared to the natural history of the tumor itself. The goal of this study was to analyze the role of surgery in patients with skull base paragangliomas treated with CyberKnife stereotactic radiosurgery (SRS) for definitive tumor control. A retrospective review identified 22 patients (median age 65.5 years, 50% female) treated with SRS from 2010-2022. Fourteen patients (63.6%) underwent microsurgical resection. Gross total resection was performed in four patients for tympanic paraganglioma (n = 2), contralateral paraganglioma (n = 1), and intracranial tumor with multiple cranial neuropathies (n = 1). Partial/subtotal resection was performed for the treatment of pulsatile tinnitus and conductive hearing loss (n = 6), chronic otitis and otorrhea (n = 2), intracranial extension (n = 1), or episodic vertigo due to perilymphatic fistula (n = 1). Eighteen patients had clinical and imaging follow-up for a mean (SD) of 4.5 (3.4) years after SRS, with all patients having clinical and radiological tumor control and no mortalities. Surgery remains an important component in the multidisciplinary treatment of skull base paraganglioma when considering other outcomes besides local tumor control.

Objective Assessment of Perilymphatic Fistula in Cases of Postoperative Vertigo after Cochlear Implantation by Cochlin Tomoprotein (CTP).

Vertigo is a quite frequent complication after cochlear implantation. Perilymphatic fistula (PLF) is assumed to be one cause of this problem. Cochlin tomoprotein (CTP) is a newly introduced marker for PLF. The present aim was to evaluate the rate of positive CTP testing in cases of newly occurring vertigo after cochlear implantation. Twelve patients with vertigo after cochlear implantation and a revisional electrode-sealing procedure underwent intraoperative rinsing of their middle ear. The sample was evaluated for CTP with monoclonal antibody testing. Sixteen controls from six CI patients were taken. 4 out of 12 (33%) cases showed positive CTP testing, indicating that a PLF could be evaluated. In all of the positive CTP cases, surgery decreased the vertigo symptoms. A relation between the subjective visual assessment of a fistula and a positive CTP value was not observed. Controls confirmed the value of the testing. CTP detection objectively shows that PLF can occur in patients with vertigo after CI.

Assessing the efficacy of perilymphatic fistula repair surgery in alleviating vestibular symptoms and associated auditory impairments.

Perilymph Fistula (PLF), abnormal communication between the fluid-filled space of the inner ear and the air-filled space of the middle ear, is a significant cause of vestibular and auditory symptoms. This is a retrospective study of 22 cases treated with PLF repair surgery, selected based on our surgical indication. We analyzed the characteristics of these 22 cases and evaluated the efficacy of PLF repair surgery in treating vestibular and auditory symptoms. Cases with antecedent events had significantly shorter intervals before surgery. The postoperative recovery from vestibular symptoms following PLF repair surgery was strikingly rapid, with 82% of cases demonstrating marked improvement within a week, even in chronic cases. Despite the notable absence of a control group in the study, the marked improvements in vestibular symptoms and substantial reductions in Dizziness Handicap Inventory (DHI) scores suggest that the observed benefits are attributable to the surgical intervention. Further, timely surgery showed improvements in hearing, with some benefits also seen in late-stage surgeries. Using the perilymph-specific protein Cochlin-tomoprotein (CTP) as a diagnostic biomarker, we could prove that PLF could be responsible for disequilibrium and related auditory disturbances in these patients. A new hypothesis is proposed that the chronic disequilibrium experienced by many PLF patients is due to enhanced mobility of the utricle and not to endolymphatic hydrops. Further research is needed to fully elucidate PLF's symptoms and treatment efficacy using the surgical indication we developed.

An expanding mechanical role for the tiny mammalian endolymphatic duct: 2. An explanation for the beaching of whales–failure of osmotic pressure counterbalancing mechanism in the cochlea causing acute vertigo attack

Endolymphatic Hydrops (EH) describes a swelling of the endolymphatic duct (ED). It is a key correlate of human Ménière’s disease featuring incapacitating giddy attacks. Direct measurements by micropuncture to be presented support the hypothesis that swelling occurs because water flows through aquaporins into the ED under an osmotic gradient to stabilize basilar membrane position against deep dive pressures. Patients having vestibular attacks often display nystagmus if not also perilymphatic fistulae or dehiscences. As shown by Ketten et al. (2004), stranded whales also exhibit nystagmus and also intracochlear bleeding. Here, EH is modelled as a normal rate-limited osmotic mechanism which likely evolved to cope with synoptic variation. It has been extensively adapted by beaked whales to allow diving to feed on squid at great depths—where they still need to hear. Their more-robust EDs normally cope with very slow equalization against megapascal pressures. An unexpected undersea pressure transient may trigger perilymph-endolymph membrane rupture and consequent vertigo attack, well documented in humans. Due to acoustic spread, whole pods may be variously affected. Humans need to remain stationary while waiting hours for their attack to subside. Marine mammals with limited oxygen stores may panic and surface too quickly, accounting for life-threatening decompression sickness.

Real-life study of the use of oto-acoustic emissions in the diagnosis of intracranial hypotension

BackgroundThe diagnosis of spontaneous or post-traumatic intracranial hypotension (IH) mainly relies on clinical features and neuro-imaging. However, the results of brain and spine magnetic resonance imaging are not always contributive. There is an interest for other non-invasive procedures, able to confirm or refute the diagnosis. The use of oto-acoustic emissions (OAE) was previously reported on isolated cases of IH associated with endolymphatic hydrops (ELH). The aim of this study was to assess the real-life utilization of this electrophysiological method in a larger population of suspected IH. MethodsA retro-prospective cohort study was conducted from November 2013 to July 2022 in patients with a suspected or doubtful diagnosis of IH. They were assessed for ELH by recording bilateral distortion product of oto-acoustic emissions (DPOAE) in sitting then in supine position. ResultsAmong the 32 patients assessed, the diagnostic of IH was confirmed in 18 patients. An ELH was shown in 15 of them (83%), but also in seven other patients. They had several differential diagnoses: chronic migraine, Chiari malformation, rebound intracranial hypertension and perilymph fistula. ConclusionsThis procedure seems to be insufficient to exclude differential diagnosis when intracranial hypotension is suspected.