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Objective: To discuss the link between inner ear decompression sickness and patent foramen ovale., Materials and Methods: Monocentric and retrospective study on decompression sickness of the inner ear requiring hyperbaric chamber treatment, from 2014 to 2021., Results: Sixty-one patients of inner ear decompression sickness were included in this study. Twenty-four patients had vestibular injuries, 28 cochlear injuries and 9 cochleo-vestibular injuries. Compression chamber treatment was given, using an oxygen-helium mixture with oxygen partial pressure (PIO2) limited to 2.8 atmosphere absolute (ATA). All vestibular accidents completely recovered without clinical sequelae. For cochlear accident only 10 out of 37 patients (27%) recovered completely. A right-left shunt (patent foramen oval or intra-pulmonary shunt) was found in 31.1% of patients with inner ear decompression sickness (p > 0.05)., Conclusion: The presence of patent foramen oval in patients with inner ear decompression was not statistically significant in our study. Understanding of the pathophysiology of decompression illness and the physiology and anatomy of the labyrinth would suggest a mechanism of supersaturation with degassing in intra-labyrinthine liquids., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Various inner ear diseases such as sensorineural deafness and Meniere's disease bring about problems such as speech communication disorders and decreased work efficiency, which seriously affect the life quality of patients. Due to the special anatomical structure and blood-labyrinth barrier in the inner ear, the current drug administration methods are often unable to achieve satisfactory results. Nanocarriers are the forefront and hot spot of nanotechnology research. In recent years, a lot of research progress has been made in the field of targeted delivery of the inner ear, which is expected to be eventually applied to the treatment of clinical diseases of the inner ear. This review focuses on the advantages, main research achievements and limitations of various nanocarriers in the targeted delivery of the inner ear, hoping to provide new ideas for related research., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Background: Manières disease (MD) is a chronic inner ear disease characterized by recurrent vertigo and fluctuation in auditory symptoms. Vertigo spells have a sudden onset and are difficult for patients to handle. Therefore, treating a patient with MD is still a challenge for clinicians., Aims: This study aims to analyse the short-term effects of intratympanic dexamethasone (ITD) on the various symptoms of unilateral MD., Materials and Methods: The study comprised 27 patients with unilateral MD and severe vertigo who failed medication therapy. Treatment was with ITD as an alternative to destructive therapy. Treatment is evaluated after four months., Results: Significant improvements were measured with Dizziness Handicap Inventory (DHI), Tinnitus Handicap Inventory (THI), frequency of vertigo attacks longer than 20 min, Functional Level Scale (FLS), and tinnitus sensation measured by the Analog Visual Scale (AVS). Patients with severe symptoms grading with DHI and THI experienced the most improvement. Patients have achieved substantial vertigo control in 73%., Conclusion: ITD application shows improvement in controlling vertigo and tinnitus in patients under exacerbation in MD., Significance: It is a promising non-destructive addition to the 'stepwise treatment concept' in MD and can be used as a first-line treatment in vertigo control.

This prospective pilot study aimed to evaluate whether cerebral inflow and outflow abnormalities assessed by ultrasonographic examination could be associated with recurrent benign paroxysmal positional vertigo (BPPV). Twenty-four patients with recurrent BPPV, affected by at least two episodes, and diagnosed according to American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) criteria, evaluated at our University Hospital, between 1 February 2020 and 30 November 2021, have been included. At the ultrasonographic examination, 22 of 24 patients (92%) reported one or more alterations of the extracranial venous circulation, among those considered for the diagnosis of chronic cerebrospinal venous insufficiency (CCSVI), although none of the studied patients were found to have alterations in the arterial circulation. The present study confirms the presence of alterations of the extracranial venous circulation in recurrent BPPV; these anomalies (such as stenosis, blockages or regurgitation of flow, or abnormal valves, as per the CCSVI) could cause a disruption in the venous inner ear drainage, hampering the inner ear microcirculation and then possibly causing recurrent otolith detachment.

A 27-year-old female patient suffering endolymphatic sac tumor with intralabyrinthine hemorrhage was reported. The patient had hearing loss in the left ear with continuous tinnitus, and MRI showed the soft tissue shadow of endolymphatic sac. Considering that the tumor involved semicircular canal and vestibule,endolymphatic cyst tumor resection was performed by labyrinth route. After surgery, there was no cerebrospinal fluid leakage and facial nerve function was normal. More importantly, enhanced MRI of temporal bone showed no tumor recurrence 1 year after surgery., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Objective: To evaluate otolith function by comparing the findings of the Subjective Visual Vertical, the cervical Vestibular Evoked Myogenic Potential and the ocular Vestibular Evoked Myogenic Potential tests in patients in the inter-crisis period of unilateral definite Meniere's disease., Methods: The sample consisted of an experimental group (n=22; 10 men and 12 women, mean age 47.32±12.82 years) with definite unilateral Meniere's disease and a control group (n=14; 5 men and 9 women, with a mean age of 41.64±13.45 years). They all underwent vestibular evaluation by means of Subjective Visual Vertical with the bucket method and, cervical and ocular Vestibular Evoked Myogenic Potential tests. The data were collected and subjected to statistical analysis., Results: The results of the comparison of the Subjective Visual Vertical, the cervical Vestibular Evoked Myogenic Potential, the ocular Vestibular Evoked Myogenic Potential and, the association of cervical Vestibular Evoked Myogenic Potential with the ocular Vestibular Evoked Myogenic Potential showed no significant difference, indicating concordance among the tests., Conclusion: The identified abnormalities and the concordance between the combined proportion of the Subjective Visual Vertical, the cervical Vestibular Evoked Myogenic Potential and the ocular Vestibular Evoked Myogenic Potential findings indicate that the association of these three tests contributes to the identification of sustained and transient otolith dysfunction in the inter-crisis of unilateral definite Meniere's disease., (Copyright © 2022. Published by Elsevier España, S.L.U.)

Background/purpose: Concussion sequelae in the elderly is under recognized and negatively impacts quality of life. Labyrinthine concussion (LC) is an uncommon diagnosis, as is multiple canal (MC) benign paroxysmal positional vertigo (BPPV). This case report highlights physical therapist (PT) evaluation and treatment of an elderly male misdiagnosed with LC and successfully treated for MC BPPV., Case Description: A 72 year old male presented to his PT 23 days after falling off a ladder, resulting in a mild traumatic brain injury (mTBI). Diagnosed with LC, he was referred to PT due to ongoing symptoms of "falling backwards," poor gait, and diminished mobility. PT examination revealed an atypical BPPV. Thus, the patient was treated in two PT visits, which included canalith repositioning techniques and neuromuscular reeducation., Outcomes: The PT diagnosis was MC BPPV, including the right lateral and left posterior semicircular canals. Initial positive findings of Head Impulse Test, Bow and Lean Test, Dix-Hallpike, and Roll Test were negative on the last visit. Patient-Specific Functional Scale improved from 0 to 9.9 (10 being no limitations). The patient progressed from minimum assistance to independence in bed mobility, transfers, gait, and previous activities., Discussion: The patient's presentation was atypical in signs and symptoms with a diagnosis of LC. PT examination and intervention successfully resolved the patient's signs and symptoms within two visits. Further research is needed regarding identification and treatment of elderly individuals with head injuries, such as MC BPPV, as well as the efficacy of a PT seeing patients shortly after mTBI.

Purpose: Most ELST data in the literature are case studies or limited to small cohorts (< 16 patients). We evaluated the main clinical signs observed at endolymphatic sac tumor (ELST) diagnosis in patients with or without Von Hippel-Lindau disease., Methods: We conducted a comprehensive literature search in PubMed, Scopus, and Web of Science. We included studies with at least 1 patient, of any age, affected by sporadic or VHL-related ELSTs reporting levels of hearing loss and facial nerve function and a comprehensive description of presenting symptoms at ELST diagnosis. We combined data for proportional meta-analysis. p values of 0.05 were considered statistically significant. Methodological quality was evaluated. Analyses were performed with MedCalc 14.8.1 software., Results: A total of 26 studies, including 113 patients and 118 cases of ELSTs were included. Pooled proportion rates (95% CI) of overall hearing loss was 88.7%, (82.4-93.4), severe hearing loss was 21.6% (12.8-32.1) profound hearing loss was 39.8% (28.7-51.5), vertigo/imbalance was 42.0% (33.8-50.5), tinnitus was 61.8% (53.4-69.8) and facial nerve palsy was 30.6% (23.2-38.9). Generally, symptoms were homogeneous or moderately heterogeneous among included studies., Conclusion: This is the first systematic review of clinical presentations at ELST diagnosis. The most serious clinical events include profound hearing loss and facial impairment. Fluctuating hearing loss, tinnitus and vertigo are frequently reported and may confound correct and prompt ELST diagnosis., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

We present a topical drug delivery mechanism through the ear canal to the middle and inner ear using liposomal nanoparticles without disrupting the integrity of the tympanic membrane. The current delivery method provides a noninvasive and safer alternative to transtympanic membrane injections, ear tubes followed by ear drops administration, and systemic drug formulations. We investigate the capability of liposomal NPs, particularly transfersomes (TLipo), used as drug delivery vesicles to penetrate the tympanic membrane (TM) and round window membrane (RWM) with high affinity, specificity, and retention time. The TLipo is applied to the ear canal and found to pass through the tympanic membrane quickly in 3 h post drug administration. They are identified in the middle ear cavity 6 h and in the inner ear 24 h after drug administration. We performed cytotoxicity in vitro and ototoxicity in vivo studies. Cell viability shows no significant difference between the applied TLipo concentration and control. Furthermore, auditory brainstem response (ABR) reveals no hearing loss in 1 week and 1 month post-administration. Immunohistochemistry results demonstrate no evidence of hair cell loss in the cochlea at 1 month following TLipo administration. Together, the data suggested that TLipo can be used as a vehicle for topical drug delivery to the middle ear and inner ear.

Objective: Recurrent dizziness, vertigo, and imbalance, as well as nausea or vomiting, can be induced by central or peripheral vestibular pathology. Vestibular migraine (VM) is a central pathology in which migraines reciprocally interact with vestibular nuclei. Vestibular neuritis and labyrinthitis (VN/L) are peripheral diseases involving inflammation of structures in the vestibular nerve or labyrinth. Because VM and VN/L in early stages can produce similar symptoms, diagnosis may require prolonged clinical evaluation. It has been suggested that differences in a patient's Vestibulo-Ocular Reflex (VOR) measured during the rotary chair step rotation test (RCS Test) can differentiate VM from peripheral pathologies, allowing for faster diagnosis. We sought to compare VOR time constants (VORT c s) in patients with VM versus VN/L, with the goal of exploring potential diagnostic value., Study Design: Retrospective chart review of patients seen at a hospital balance center between January 2010 and June 2019., Setting: Tertiary referral center., Patients: 68 patients (mean age = 58.15, 65 % female, 35 % male) were placed into two groups based on clinical diagnosis codes: 1) VM or 2) VN/L., Main Outcome Measure(s): Primary variable of VORT c , evoked by rotational step testing., Results: Patients in the VM group demonstrated statistically significant elevated VOR T c s outside the normed range of 10-19.5 compared to those in the VN/L group (p = 0.0003) while patients in the VN/L group demonstrated statistically significant shortened VOR T c s (p = 0.0443) with the two populations having distinctly different VOR T c profiles., Conclusions: Our results support the conclusion that elevated VOR T c s are a sign of central vestibular pathology and highlight the benefit of utilizing rotary chair testing early in the diagnostic process as a powerful diagnostic tool when evaluating dizzy patients for central versus peripheral vestibular dysfunction. Our results suggest more particularly that elevated VOR T c s can be utilized to identify patients with vestibular migraine, thus offering differentiation from patients with peripheral vestibular dysfunction earlier in the process of diagnosis, with an opportunity for earlier intervention., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objective: This study reports subjective and objective audiovestibular function in patients diagnosed with an endolymphatic sac tumor (ELST), to evaluate the clinical implication of vestibular testing in ELST patients., Study Design: Retrospective cohort study., Setting: Tertiary referral center/university hospital., Patients: Five patients diagnosed with unilateral ELST., Intervention: Subjective and objective audiovestibular tests; pure-tone average; speech discrimination score; the Video Head Impulse Test; the cervical vestibular evoked myogenic potential test; the caloric irrigation test; the Speech, Spatial and Qualities of Hearing Scale; the Tinnitus Handicap Inventory; and the Dizziness Handicap Inventory., Main Outcome Measure: Findings and characteristics of both subjective and objective audiovestibular functions., Results: The mean pure-tone average and the mean speech discrimination score were 49 dB HL and 68% on the tumor ear and 13 dB HL and 99% contralaterally. The mean Speech, Spatial and Qualities of Hearing Scale score was 84, and the mean Tinnitus Handicap Inventory score was 64. Caloric testing showed a mean unilateral weakness of 80%. The Video Head Impulse Test of the lateral semicircular canal revealed refixation saccades on all tumor ears and an average gain value of 0.75 on the tumor ear versus 0.94 contralaterally. No cervical vestibular evoked myogenic potentials were elicited on the tumor side, whereas all but one was elicited contralaterally. The average Dizziness Handicap Inventory score was 47., Conclusion: A characteristic pattern of abnormal caloric findings, refixation saccades, and normal vestibulo-ocular reflex gain alongside sensorineural hearing loss on the tumor ear was identified. Asymmetric sensorineural hearing loss in any patient should lead to objective vestibular evaluation to strengthen not only a targeted tumor surveillance strategy and the indication for imaging, but also the design of vestibular rehabilitation. In addition, vestibular findings may guide the choice of surgical approach., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Auditory and vestibular function detection technology is the premise and key to the diagnosis and management for inner ear diseases. Concurrent damage to the auditory and vestibular system occurs in many inner ear diseases. The general points and issues on hearing and vestibular function tests, as well as the clinical significance of refined and individualized comprehensive evaluation of inner ear function are described in this paper., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

A clinical diagnosis of Ménière's disease (MD) is made based on medical history and audiometry findings. The 1995 American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) guidelines requires histopathological confirmation of endolymphatic hydrops (EH) for a diagnosis of "certain" MD. Symptoms such as dizziness and ear fullness are important diagnostic features; however, the descriptions provided by patients are frequently vague and non-specific. A recently developed magnetic resonance imaging (MRI) protocol to document EH is, therefore, useful for the evaluation of inner ear status in patients with MD. In this study, patients with MD were assessed using MRI and the HYDROPS (HYbriD of Reversed image Of Positive endolymph signal and native image of positive perilymph Signal) protocol to investigate the effectiveness of MRI for visualization of the endolymphatic space in the diagnosis of MD by correlating clinical laboratory parameters with the grade of EH. Of the 123 patients with MD recruited in this study, 80 had definite MD, 11 had probable MD, and 32 had possible MD based on the 1995 AAO-HNS guidelines. The EH grade based on HYDROPS MRI was determined independently by two otorhinolaryngologists and compared with several clinical parameters, including the diagnostic scale of MD (1995 AAO-HNS guidelines), pure tone average (PTA), low tone average (LTA), canal paresis (CP) on the caloric test, and disease duration. Cochlear hydrops and vestibular hydrops were detected in 58 and 80% of 80 definite MD ears, in 33 and 58% of 12 probable MD ears, and in 5 and 27% of 37 possible MD ears, respectively. The proportion of higher hydrops grades increased significantly with grade according to the MD diagnostic scale ( p < 0.0001). Both PTA and LTA were significantly higher in patients with hydrops grade 2 than hydrops grade 0 in both the cochlea and the vestibule. CP was significantly higher in patients with grade 2 than grade 0 vestibular hydrops. Disease duration was not associated with hydrops grade. Radiological evaluation of MD using the HYDROPS protocol is useful for evaluation of the extent and severity of EH in the diagnosis of MD based on its pathophysiological mechanism., (Copyright © 2022 Han, Kim, Kim, Lee, Song, Choi, Kim, Bae and Koo.)

Background: Endolymphatic sac tumour (ELST) is a rare low-grade locally aggressive neoplasm arising from the endolymphatic duct or sac. It presents mostly with vestibulo-cochlear symptoms either sporadically or as part of von Hippel-Lindau (VHL) syndrome. Micro-neurosurgical excision remains the cornerstone of therapy with the role of radiotherapy (RT) being controversial. This is a clinico-pathological analysis of consecutive ELST patients presenting to a single-institution in India., Methods: Neuropathology database of a tertiary-care comprehensive cancer centre was searched electronically to identify consecutive patients with histopathological diagnosis of ELST registered at the institute over last one decade. Data regarding demographic profile, clinical presentation, histopathological features, treatment details and outcomes were retrieved from electronic medical records for this retrospective analysis., Results: Electronic search identified seven unique patients with biopsy-proven ELST registered at the institute between 2009 and 2020. Median age of the study cohort was 39 years (range 24-65 years) with strong male predilection (5:2 ratio) and left-sided preponderance (71%). Most common presenting symptoms were hearing loss (86%) and earache (71%) on affected side followed by headache (43%). All patients underwent maximal safe resection at initial diagnosis and were followed-up closely with periodic surveillance imaging. Two patients underwent salvage RT using high-precision conformal techniques at recurrence/progression., Conclusion: ELST is a rare low-grade locally aggressive neoplasm that arises generally as part of VHL syndrome or sometimes sporadically. Gross total resection provides the best chance of cure with RT being reserved for unresectable disease, large residue, medical inoperability, or as salvage therapy for recurrent/progressive tumor., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Labyrinthine fistula (LF) is one of the most important complications of cholesteatoma and is defined as an abnormal communication between the inner and the middle ear. This study aims to describe our experience with the partial labyrinthectomy evaluating the post-operative hearing results. Twenty-one patients who presented labyrinthine fistula in the semicircular canals were included in the present study. Hearing impairment was present in 48% of patients (10/21). A pre-operative assessment using the Gardner-Robertson hearing classification showed the following: 52%, Class I; and 48%, Class II. A post-surgical Gardner-Robertson hearing classification evidenced the following: 43%, Class I; and 57%, Class II. The presence of LF is usually considered a negative prognostic factor for hearing preservation. The key point of partial labyrinthectomy surgery is the preservation of structures, keeping them wet with Ringer's solution throughout the procedures, and not performing suction that is close to the opened LF. The bony labyrinth is drilled underwater without suction, removing the entire cholesteatoma matrix and quickly plugging the site before and after the LF. This faster plugging of the labyrinth makes it possible to preserve the peri-lymph and the endo-lymph fluid and the hearing function. This study showed that a partial labyrinthectomy is useful for maintaining serviceable hearing in patients with LF., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Pace, Milani, Messineo, Rossetti, Cocuzza, Maniaci, Vicini, Iannella and Magliulo.)

Objective: Immune-mediated inner ear disease (IMIED) might cause severe/profound hearing loss and these patients are considered ideal candidates to cochlear implant (CI) surgery. The aim of the study was to evaluate impedance changes over time., Method: The Study Group (SG) was composed of CI IMIED patients (31 ears) and a Control Group (CG) of CI patients with hearing loss not related to their immune system (31 ears). Audiological performance and impedance values were measured and compared amongst groups at 3, 6, 12 and 18 months following the fitting sessions., Results: Speech perception was significantly better for SG in word and sentence recognition in quiet. Impedance values were, on average, significantly higher for apical and middle electrode segments in SG compared to CG at the 3- month follow-up and were maintained over time. Additionally, a subset of SG patients (active patients) experienced significantly greater impedance fluctuation corresponding to clinical symptom reactivation., Conclusion: IMIED patients achieve good audiological performance. However, the relapsing inflammation could change the inner ear environment, causing impedance fluctuations and, consequently, more frequent CI fittings. Additionally, impedance evaluation could be utilized as an early warning sign of IMIED recurrence and as an aid to therapeutic decision-making.

Competing Interests: I have no commercial associations, contractual relations, or proprietary considerations that might pose a conflict of interest related or unrelated with the submitted manuscript, and have had no involvements that might raise the question of bias in the work reported or in the conclusions, implications, or opinions stated. The authors disclose no conflicts of interest.

Competing Interests: The authors disclose no conflicts of interest.

Objectives: Hypothesized causes of vestibular neuritis/labyrinthitis include neuroinflammatory or vascular disorders, yet vascular disorders of the inner ear are poorly understood. Guided by known microvascular diseases of the retina, we developed 2 hypotheses: (1) there exist vascular vulnerabilities of artery channels in cases of hypothetical nerve swelling for the superior, inferior, and vestibulocochlear artery and (2) there are arteriovenous crossings that could compromise vascular flow in disease states., Methods: Two fully mounted and stained temporal bones were used to render three-dimensional reconstructions of the labyrinth blood supply. Using these maps, areas of potential vascular compression were quantified in 50 human temporal bones., Results: Although inner ear arteries and veins mostly travel within their own bony channels, they may be exposed (1) at the entrance into the otic capsule, and (2) where the superior vestibular vein crosses the inferior vestibular artery. At the entry into the otic capsule, the ratio of the soft tissue to total space for the superior vestibular artery was significantly greater than the inferior vestibular artery/cochleovestibular artery (median 44, interquartile range 34-55 vs. 14 [9-17], p < 0.0001)., Conclusions: Three-dimensional reconstruction of human temporal bone histopathology can guide vascular studies of the human inner ear. Studies of retinal microvascular disease helped identify areas of vascular vulnerability in cases of hypothetical nerve swelling at the entrance into the otic capsule and at an arteriovenous crossing near the saccular macula. These data may help explain patterns of clinical findings in peripheral vestibular lesions., (© 2021 The Author(s). Published by S. Karger AG, Basel.)

Objectives: (1) To determine how otologic/neurotologic surgeons counsel patients with superior semicircular canal dehiscence (SSCD). (2) To understand the plethora of presenting symptoms associated with SSCD and appropriate management. (3) To suggest appropriate management; oftentimes avoiding surgery., Methods: This was a survey study of both community and academic physicians. A 23-question survey was distributed to all members of the American Neurotological (ANS) and American Otologic Societies (AOS) via email in the Fall of 2018. A total of 54 responses were received from a possible pool of 279 for a response rate of 19.4%. Inferences were made about the population through sample proportions and confidence intervals., Results: All respondents use computed tomography (CT) in diagnosing SSCD and 11.1% use CT exclusively. Cervical vestibular evoked myogenic potential (VEMP; 77.8%) are used more often than ocular VEMPs (38.9%). Magnetic resonance imaging (7.4%) is used infrequently; 96.3% of surgeons surveyed have seen patients with SSCD on imaging that are asymptomatic. Following surgical treatment, respondents reported balance issues and mild-to-moderate high-frequency sensorineural hearing loss (88.4%); 32.6% reported that the majority (>50%) of their patients needed further intervention after surgery, typically aggressive vestibular rehabilitation., Conclusions: There is a discrepancy in the systematic approach to SSCD between both the surgeons and the published literature. Patients with SSCD on ultra-high-resolution CT may have myriad symptoms while others are asymptomatic, and surgery may lead to additional complications. We will present a methodical recommendation to assist in the management of patients with SSCD depending upon their symptoms. This may improve patient selection, counseling, and outcomes.

Objectives: Even 250 years after Beethoven's birth, the irrevocable cause of his hearing impairment remains unclear despite multiple publications by different professional groups. This study aimed to analyse the development of the most likely aetiologies during the last 100 years by a systematic review of the relevant medical literature., Methods: A systematic review of medical literature in PubMed ® , PubMed Central ® , and Web of Science ® for the period 1920-2020 was conducted. Medical publications between 1920 and 1935 were additionally searched manually by review of reference lists. Studies were eligible when a statement regarding the most likely aetiology of the hearing loss of Beethoven was the aimed objective of the publication., Results: 48 publications were included. The following aetiologies were supposed: otosclerosis (n = 10), syphilis (n = 9), Paget's disease (n = 6), neural deafness (n = 5), immunopathy with inflammatory bowel disease, neural deafness with otosclerosis, sarcoidosis or lead intoxication (n = 2), and systemic lupus erythematosus, trauma, labyrinthitis or inner ear disease (n = 1). There is an ongoing effort with a mean publication frequency in this topic of 0.48/year. From 1920 to 1970, otolaryngologists were the group with the highest interest in this field (67%), whereas since 1971 most authors have belonged to non-otolaryngologic subspecialities (81%)., Conclusion: Over the past 100 years, otosclerosis and syphilis were predominantly supposed to be the underlying causes. The hypothesis of syphilis-although rejected for a long time-has had a remarkable revival during the past 20 years. Regarding the outcome following therapeutic intervention by cochlear implantation, the differential diagnosis of neural deafness would be relevant today.

Competing Interests: The authors disclose no conflicts of interest.

An infracochlear cholesteatoma of the petrous apex with direct contact to the internal carotid artery (ICA) is rare. Due to the risk of cochlear injury with consecutive deafness or injury of the ICA, precise preoperative planning of the approach and strategy is recommended, as well as thorough preoperative counseling of the patient for their informed consent. This case report presents navigated endoscopically controlled transtympanic resection of such a cholesteatoma recurrence. Hearing capacity was not impaired and the patient shows no signs of recurrence.

Despite the low overall prevalence of individual rare diseases, cochlear dysfunction leading to hearing loss represents a symptom in a large proportion. The aim of this work was to provide a clear overview of rare cochlear diseases, taking into account the embryonic development of the cochlea and the systematic presentation of the different disorders. Although rapid biotechnological and bioinformatic advances may facilitate the diagnosis of a rare disease, an interdisciplinary exchange is often required to raise the suspicion of a rare disease. It is important to recognize that the phenotype of rare inner ear diseases can vary greatly not only in non-syndromic but also in syndromic hearing disorders. Finally, it becomes clear that the phenotype of the individual rare diseases cannot be determined exclusively by classical genetics even in monogenetic disorders., Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).)

Objective: Concomitant otosclerosis (OTS) and superior semicircular canal dehiscence (SSCD) is a rare, but difficult-to-identify and treat diagnosis. A systematic review of the literature was performed to analyze the diagnostic and therapeutic approaches of concurrent OTS and SSCD cases and to identify possible factors that may help in predicting the surgical outcome., Data Sources: PubMed, Scopus, Medscape, Ovid databases., Study Selection: Studies showing diagnosis of OTS documented by audiometric test with or without associated radiological signs (computed tomography), and concomitant diagnosis of SSCD, documented at least by high-resolution computed tomography (and possibly supported by neurophysiological testing) were included. Both surgically treated and untreated patients were considered for data analysis., Data Extraction: The general characteristics of each study were recorded, when available. Clinical, audiological, vestibular testing, surgical, and radiological data were extracted from the published case reports and series, and recorded on a database. For each case, the diagnostic steps that confirmed OTS and concomitant SSCD in the same ear were extracted., Data Synthesis: Twelve studies were included in the review and 18 cases affected simultaneously by the 2 conditions were extracted and analyzed. Seven of 12 ears (58.3%) undergoing stapes surgery experienced a hearing improvement. In four (33.3%) cases, vestibular symptoms (with or without associated acoustic symptoms) of SSCD were unmasked or worsened by stapes surgery., Conclusions: A reliable preoperative diagnosis of the two concomitant conditions is possible with a proper interpretation of clinical signs, audiometric, and vestibular testing, in association with the radiologic assessment. Despite that the length and the location of the dehiscence may guide the surgical decision, definitive conclusions regarding the appropriate indications for surgical treatment cannot be drawn due to the limited number of cases with adequate data reported in the literature.

Objective: Part of the recent progress in the labyrinth imaging has been made possible by the rise of contrast-free T2-weighted and delayed (1h) FLAIR sequences. The aim of this article is to review evidence for the use of these two sequences to image the inner ear, especially the posterior membranous labyrinth., Material and Methods: We analyzed MRI-based papers (2007-2020)using high-resolution T2-weighted or contrast-enhanced FLAIR (1h) sequences to image the inner ear., Results: T2-weighted sequences (3T MRI)enabled the visualization of the posterior membranous labyrinth with good correlation when compared to corresponding histological slices.Significant progress has been made, especially in terms of scanning time, aiming at reducing it, in order to decrease motions artifacts. The saccule is visible on a 3T MRI without significant motion artifacts. Its shape is ovoid, with a maximum height and width of 1.6 and 1.4 mm, respectively. An enlarged saccule was observed in 84%of patients with unilateral Meniere's disease, in 28%of patients with vestibular schwannomas (VS) and 47%of patients with intralabyrinthine schwannomas. VS obstructing the internal auditory canal caused a decrease of the perilymphatic signal (more moderate decrease in meningiomas) on T2 gradient-echo images. Contrast-enhanced FLAIR sequences are useful to image vestibular/facial neuritis and inflammatory inner ear diseases., Conclusion: Precise analysis of the posterior membranous labyrinth, in terms of size, shape and signal intensity, is possible on a 3T MRI using high-resolution gradient-echo T2-weighted sequences. Such sequences are an interesting add-on to delayed (4h30) FLAIR-based protocols for labyrinth imaging.

An infracochlear cholesteatoma of the petrous apex with direct contact to the internal carotid artery (ICA) is rare. Due to the risk of cochlear injury with consecutive deafness or injury of the ICA, precise preoperative planning of the approach and strategy is recommended, as well as thorough preoperative counseling of the patient for their informed consent. This case report presents navigated endoscopically controlled transtympanic resection of such a cholesteatoma recurrence. Hearing capacity was not impaired and the patient shows no signs of recurrence.

Introduction: Superior canal dehiscence syndrome (SCDS) is a condition characterized by a defect in the bone overlying the superior semicircular canal, creating a third mobile window into the inner ear. Patients can experience disabling symptoms and opt for surgical management. Limited data are available on the impact of SCDS on health-related quality of life (HRQoL) and disease-specific HRQoL more specifically. Objective: To perform a prospective analysis on generic HRQoL in SCDS patients compared to healthy age-matched controls. Methods: A prospective study was performed on patients diagnosed with SCDS and who did not undergo reconstructive surgery yet. Patients were recruited between November 2017 and January 2020 and asked to complete the Health Utility Index (HUI) Mark 2 (HUI2)/Mark 3 (HUI3) questionnaire. For the control group, age-matched participants without otovestibular pathology or other chronic pathology were recruited. The multi-attribute utility function (MAUF) score was calculated for the HUI2 and HUI3. Results of both groups were compared using the Mann-Whitney U test. Results: A total of 20 patients completed the questionnaire. Age ranged from 37 to 79 years with a mean age of 56 years (45% males and 55% females). The control group consisted of 20 participants with a mean age of 56.4 years and ranged from 37 to 82 years (35% males and 65% females). For the case group, median HUI2 MAUF score was 0.75 and median HUI3 MAUF score was 0.65. For the control group, the median scores were 0.88 and 0.86 respectively. There was a statistically significant difference for both HUI2 ( p = 0.024) and HUI3 ( p = 0.011). SCDS patients had a worse generic HRQoL than age-matched healthy controls. One patient with unilateral SCDS had a negative HUI3 MAUF score (-0.07), indicating a health-state worse than death. Conclusion: SCDS patients have significantly lower health utility values than an age-matched control group. This confirms the negative impact of SCDS on generic HRQoL, even when using an instrument that is not designed to be disease-specific but to assess health state in general. These data can be useful to compare impact on HRQoL among diseases., (Copyright © 2020 Ocak, Topsakal, Van de Heyning, Van Haesendonck, Jorissen, van de Berg, Vanderveken and Van Rompaey.)

Background: Betahistine is used worldwide to treat patients with Menière's disease. However, despite it being used for decades, diverging opinions on the effect of betahistine on Menière's symptomatology still exist. Aims: The objective of this systematic review was to provide an overview and rate the certainty of the current evidence base regarding the use of betahistine to treat patients with Menière's disease. Materials and methods: A systematic literature search was conducted in October 2019. The search strategy was subdivided into searches for existing guidelines, systematic reviews and individual randomized controlled trials (RCT) investigating the usage of betahistine as compared to placebo, in patients with Ménière's disease. The primary outcome was the frequency of vertigo attack(s) and occurrence of serious adverse events. Results: We identified three relevant guidelines and three systematic reviews: however, neither included any relevant trials matching our inclusion criteria. An individual search for RCTs identified one trial. The results from this particular trial showed no difference in effects on symptoms following treatment with betahistine. Conclusions and Significance: There is a need for further well-conducted placebo RCTs. Currently, there is still a lack of substantial evidence supporting betahistine as a significant and adequate treatment for patients diagnosed with Menière's disease. Trial registration number: The protocol is registered in PROSPERO. Registration number: CRD42018110127 Accepted 11.10.2018.

Objective: To improve the level of clinical diagnosis and treatment of idiopathic bilateral vestibulopathy（IBV） by investigating the clinical characteristics of this disease. Method: Retrospective analysis was performed on patients with IBV diagnosed, excluding patients with clear primary factors, and including IBV patients with unknown causes, to analyze their clinical symptoms and vestibular function examination results. Result: In this group of cases, three cases were in line with the diagnosis of IBV. The clinical manifestations included imbalance, oscillopsia, and normal hearing level, which cannot be explained by other diseases. The vestibular function testing of the patients with IBV showed bilateral reduced response in caloric tests, positive response in video head impulse test, and decreased score in sensory organization test. Vestibular rehabilitation therapy were carried for these patients, and could improve the patients' symptoms. Conclusion: IBV has typical clinical manifestations. Vestibular function testing indicates bilateral vestibular dysfunction. Other diseases with bilateral vestibulopathy should be excluded for diagnoses, and vestibular rehabilitation therapy is one of the appropriate treatments., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Background and Objectives: We sought to evaluate the diagnostic and prognostic value of measurable parameters of internal auditory canal (IAC) magnetic resonance imaging (MRI) in patients with idiopathic sudden sensorineural hearing loss (ISSNHL)., Subjects and Methods: We retrospectively reviewed the patients with ISSNHL who underwent IAC MRI from January 2008 to March 2019. Measurable parameters of IAC MRI, such as the diameter of the IAC, bony cochlear nerve canal, and cross-sectional area of the cochlear nerve, were measured by a single examiner. These parameters were then compared between the affected and healthy sides. Inner-ear abnormalities such as intralabyrinthine hemorrhage or labyrinthitis were also evaluated. The relationship between the surveyed parameters and the diagnosis of ISSNHL was assessed., Results: A total of 208 patients with ISSNHL were included. The measured parameters of IAC MRI were not different between the affected and healthy sides and were also not associated with the diagnosis of ISSNHL. However, inner-ear abnormalities of IAC MRI in ISSNHL displayed a significant association with worse hearing before and after treatment. An age that was older than 40 years also correlated with poorer outcomes. Further, inner-ear abnormalities were more frequently detected when IAC MRI was performed early after ISSNHL onset., Conclusions: Patients with ISSNHL and inner ear abnormalities such as intralabyrinthine hemorrhage or labyrinthitis identified via IAC MRI may experience poorer hearing outcomes. To detect such abnormal findings, it is recommended to perform IAC MRI early after the onset of ISSNHL.

Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht.

Autoimmune sensorineural hearing loss is a rare clinical entity which accounting for less than 1% in all cases with hearing loss. The prevalence of hearing loss in immune-mediated inner ear diseases, as shown in case reports or single-center statistics, varies widely. We reviewed the current literatures on the association between sensorineural hearing loss and autoimmune diseases, focused on the prevalence of hearing loss in different autoimmune diseases, treatments and challenges., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Vestibular diseases often have no or only nonspecific biomarkers. It is therefore necessary to define these disorders using operational criteria based on patterns of symptoms, i.e., the presence of inclusion and exclusion criteria, similar to the situation with headaches or psychiatric diseases. Ten years ago, the Bárány Society embarked upon development of the International Classification of Vestibular Disorders (ICVD). This entails producing the different definitions iteratively according to a structured procedure with an internal review process, resulting in an open-access publication on the diagnostic criteria in each case. It is a multidisciplinary effort, and depending on the topic, cooperation with other scientific societies is sought. The classification encompasses primary vestibular disorders and non-vestibular disorders that may manifest with prominent vestibular symptoms. The following paper describes the procedure and briefly presents definitions which have already been published as well as those presently in elaboration.

Objective: To describe a case of traumatic pneumolabyrinth and subsequent hearing recovery after ejection of air with transcanal endoscopic surgical exploration., Patients: A 38-year-old man was struck by his child while cleaning his ear with an ear pick made of bamboo, which penetrated deep into the left ear canal. Severe vertigo with vomiting and left hearing impairment ensued. In addition, high-resolution computed tomography demonstrated an air density within the vestibule., Interventions: Exploratory tympanotomy was performed endoscopically a day after the injury and air was ejected from the oval window surgically., Main Outcome Measures: High-resolution computed tomography, audiologic testing., Results: Several hours after surgery, the patient's subjective vestibular symptoms lessened and 7 days after surgery, the patient felt slight dizziness when moving his head and no apparent spontaneous nystagmus was observed with an infrared charge-coupled device camera and was discharged from the hospital. Two years later, there are no subjective vestibular symptoms at all and the pure-tone average of his left ear improved to 16.7 dB., Conclusion: We presented a case of traumatic pneumolabyrinth and the subsequent hearing recovery after ejection of air following endoscopic exploratory tympanotomy. We propose that initial management for traumatic pneumolabyrinth should be ejection of the air bubble if it is located solely in the vestibule and sparing the cochlea.

Objectives: To assess for any association between the presence of superior semicircular canal dehiscence (SSCD) and temporal bone pneumatization and the height of middle fossa in symptomatic patients., Study Design: Retrospective case-control study., Setting: Tertiary, university referral center., Patients: Symptomatic patients with SSCD and a matched group of non-SSCD patients., Intervention: High-resolution computed tomography of the temporal bones., Main Outcome Measures: We graded the temporal bone pneumatization using the Virapongse classification and measured the distance between the middle fossa and the lateral semicircular canal on coronal plane at the most anterior aspect of the lateral and superior semicircular canals. We statistically compared all parameters (level of significance 0.05)., Results: We enrolled 30 patients with SSCD, (males = 13, females = 17) with mean age of 47 years (SD: 26). The matched control group had similar baseline characteristics (p > 0.05). Grade 4 pneumatization was noted in 83.3% (n = 25) of SSCD patients with the remainder having grade 3. In the control group, 46.6% (n = 14) had a grade 4 pneumatization, 36.6% (n = 11) grade 3 and 16.6 5 (n = 5) grade 2. This difference was statistically significant (p = 0.003). The difference in the height of the middle cranial fossa between the two groups was also statistically significant (mean 3.80 [SD: 0.84] in SSCD group and 4.68 mm [SD: 0.85] in the control group; p < 0.0001)., Conclusions: We found better-pneumatized mastoids in patients with SSCD, indicative of a direct association between temporal bone pneumatization and SSCD. Additionally, the middle fossa dura is lying significantly lower in patients with SSCD, factor of surgical importance.

Objective: The aim of the study is to compare clinical symptoms and treatment of posttraumatic benign paroxysmal positional vertigo (BPPV), BPPV associated with middle and inner ear diseases and idiopathic BPPV (iBPPV)., Patients and Methods: 640 patients with BPPV of various canals were enrolled in the study. 455 (71.1%) patients had iBPPV, 185 (28.9%) had secondary BPPV. Among patients with secondary BPPV in 16 (2.5%) patients it was associated with Meniere's disease (MD), in 34 (5.3%) patients - with sudden sensorineural hearing loss (SSHL), in 14 (2.2%) patients - with vestibular neuritis (VN), in 19 (2.9%) patients - with acute and chronic otitis media (OM) and in 75 (11.7%) patients - with chronic sensorineural hearing loss (CSHL). Posttraumatic BPPV was observed in 27 (4.2%) patients. All patients were treated with repositioning maneuvers and observed for recurrences the following 3 years., Results: IBPPV is the most frequent type in population and has the most favorable prognosis. Combined involvement of posterior and horizontal canals is more frequent in BPPV associated with MD, SSHL and OM in comparison with iBPPV ( p <0.01). BPPV associated with BM, SSHL and VN is more resistant to treatment with repositioning maneuvers in comparison with iBPPV: successful treatment with one performed maneuver is more rarely and more follow-up visits to the doctor are needed ( p <0.01). BPPV associated with MD has higher recurrence rate ( p =0.04). The clinical course of BPPV associated with CSHL and posttraumatic BPPV is most similar to iBPPV.

Introduction: Labyrinthine fistulae (LF) are the common complications of chronic otitis media (COM) of squamosal variety. The final therapeutic outcome of this condition is to preserve the cochlear and vestibular functions. Herein, we present the data of the cases managed at our institute with respect to their presenting complaints, adopted therapeutic approaches and outcomes., Materials and Methods: A retrospective chart review was conducted on all cases with COM squamosal type in adult patients. A total of 275 patients were reviewed, out of whom 30 cases had LF. The results were mainly studied with respect to the postoperative improvement of hearing and vertigo., Results: The incidence rate of LF in the present study was obtained at 10.9%. Only 50% of the cases had the symptoms of vertigo. Furthermore, positive fistula test was elicited in 3.3% of the cases. All cases undergoing preoperative imaging were diagnosed successfully. In addition, 42.85% of the cases had profound hearing loss preoperatively, which sustained after the operation. However, 47.61% of the cases showed an improvement of at least ≥ 10 dB in the air-bone gap. Out of the 15 LF cases with vertigo as the main complaint, only 11 cases referred for follow-up. In this regard, 63.63% of the cases had no postoperative vertigo symptoms., Conclusion: Patients with LF may not have complaints of vertigo and a positive fistula sign upon admission. Pre-operative imaging facilitates the diagnosis of this condition. The removal of the matrix under constant irrigation, followed by repair with bone wax and/or autologous tissue, is sufficient to preserve the cochlear and vestibular symptoms postoperatively.

Objectives:: The development of pneumolabyrinth without previous head trauma is a rare event; the associated symptoms may be nonspecific, and they can simulate various cochleo-vestibular pathological entities. The aim of the present study is to describe one of these rare occurrences, characterized by a peculiar onset., Methods:: We report a case of stapes fracture secondary to ear pick penetration into the middle ear with a pneumolabyrinth that caused a recurrent paroxysmal positional vertigo (PPV) mimicking a canalolithiasis., Results:: The patient developed a profound left sensorineural hearing loss and an intractable PPV with "migrant" features. A pneumolabyrinth was visualized with high-resolution computed tomography. A perilymphatic fistula (PLF) with stapes fracture was found while performing an explorative tympanotomy. After the surgical treatment of the PLF, the patient no longer complained of vestibular symptoms., Conclusions:: To our knowledge, this is the fifth case of traumatic pneumolabyrinth simulating a canalolithiasis without previous history of temporal bone trauma and/or middle ear surgery. A pneumolabyrinth should be suspected in case of patients presenting recurrent intractable PPV after ear trauma.

Purpose: To analyze the effectiveness of various methods of treating dizziness with fistulas of the labyrinth (FL) of inflammatory genesis. An analysis of the surgical treatment of 216 patients with chronic suppurative otitis media (CSOM) with cholesteatoma complicated by FL using a variety of FL treatment methods is presented. All patients underwent a debridement operation on the middle ear or revision of the postoperative cavity: in 48 patients with a matrix of cholesteatoma in situ on the fistula, in 109 patients with matrix removal followed by plastic repair or FL filling with various autotissues, in 59 patients with matrix conservation, plastic removal or filling FL and SLD labyrinth. In the last two groups, filling of a semicircular canal (SC) defect was performed in 33 patients, and mastoidoplasty - in 47., Conclusion: All used FL treatment techniques are effective in the treatment of peripheral vestibular disorders. Careful treatment of SC fistula after removal of all pathological conditions in the absence of active inflammation and the appointment of hormone therapy in the postoperative period are the main factors in preserving the functions of the labyrinth in any surgical technique. Filling of the lumen of the SC and mastoidoplasty in patients with CSOM with FL can eliminate peripheral dizziness.

Objective: To evaluate the association between signal changes during intraoperative audiologic monitoring and postoperative audiometric outcome in patients undergoing vestibular schwannoma (VS) resection via middle cranial fossa (MCF) approach., Study Design: Retrospective chart review., Setting: Academic tertiary referral center., Patients: One hundred twenty-six consecutive patients (mean age 48.6 yrs, range 16-67; mean tumor size 9.9 mm, range 1.8-18.9 mm) who underwent MCF VS resection., Interventions: Intraoperative audiologic monitoring using auditory brainstem response (ABR) and direct cochlear nerve action potential (CNAP)., Main Outcome Measures: Intraoperative ABR wave V and CNAP N1 amplitudes and postoperative pure-tone average (PTA) and word recognition score (WRS)., Results: On ABR, absent wave V amplitude was associated with an 81.1% increase and 82.3% decrease in postoperative PTA and WRS, respectively. On CNAP, decreased or absent N1 amplitude was associated with 47.3 and 100% increase, respectively, in postoperative PTA, and 45.3% and 100% decrease, respectively, in postoperative WRS. Receiver-operating curve analysis showed that ABR combined with CNAP using a Cueva electrode achieved the highest diagnostic accuracy in predicting postoperative hearing decline when measured by WRS class (sensitivity 83.3%, specificity 100%), and performed better compared with each modality alone., Conclusion: Intraoperative ABR wave V and CNAP amplitude changes are associated with changes in postoperative hearing, and dual modality monitoring was more diagnostic of postoperative hearing decline compared with each modality alone during MCF VS resection. Overall, intraoperative ABR and CNAP were more specific than sensitive for postoperative hearing decline.

Objectives/hypothesis: To aggregate symptoms reported by patients with superior canal dehiscence syndrome (SCDS) and to develop an evidence-based symptom set by performing a systematic review of the literature., Study Design: Database search and critical assessment of research studies., Methods: Medline and PubMed databases were searched for articles that reported the preoperative symptoms of adult and pediatric patients with unilateral and bilateral SCDS. Articles were excluded if they reported on associated diseases or did not report symptoms., Results: Of the 397 articles retrieved, 66 were retained for quantitative analysis. Among 431 patients with SCDS, 91 symptom terms were reported. After combining synonymous terms, 22 symptoms were derived by consensus. Of the raw total number of reported symptoms, 92.5% can be attributed to five common symptoms: spontaneous dizziness (51%), autophony (42.5%), pressure-induced vertigo (37.4%), hearing loss (39.9%), and sound-induced vertigo (42.7%)., Conclusions: This systematic review of symptoms reported by patients with SCDS identified a 22-item common symptom set. These items can be used to create an evidence-based patient-reported outcome measure to evaluate health-related quality of life in SCDS. Laryngoscope, 1932-1938, 2018., (© 2017 The American Laryngological, Rhinological and Otological Society, Inc.)