Fluctuating Hearing Loss Research Articles

Endolymphatic hydrops and cochlear synaptopathy after noise exposure are distinct sequelae of hair cell stereociliary bundle trauma

Endolymphatic hydrops, increased endolymphatic fluid within the cochlea, is the key pathologic finding in patients with Meniere’s disease, a disease of episodic vertigo, fluctuating hearing loss, tinnitus, and aural fullness. Endolymphatic hydrops also can occur after noise trauma and its presence correlates with cochlear synaptopathy, a form of hearing loss caused by reduced numbers of synapses between hair cells and auditory nerve fibers. Here we tested whether there is a mechanistic link between these two phenomena by using multimodal imaging techniques to analyze the cochleae of transgenic mice exposed to blast and osmotic challenge. In vivo cochlear imaging after blast exposure revealed dynamic increases in endolymph that involved hair cell mechanoelectrical transduction channel block but not the synaptic release of glutamate at the hair cell–auditory nerve synapse. In contrast, ex vivo and in vivo auditory nerve imaging revealed that synaptopathy requires glutamate release from hair cells but not endolymphatic hydrops. Thus, although endolymphatic hydrops and cochlear synaptopathy are both observed after noise exposure, one does not cause the other. They are simply co-existent sequelae that derive from the traumatic stimulation of hair cell stereociliary bundles. Importantly, these data argue that Meniere’s disease derives from hair cell transduction channel blockade.

Epidemiological Evidence for Upper Respiratory Infections as a Potential Risk Factor for Meniere's Disease: A Korean National Health Sample Cohort Study.

Meniere's disease (MD) is a chronic inner ear disorder characterized by tinnitus, ear fullness, episodic vertigo, and fluctuating hearing loss, which significantly impacts quality of life and poses management challenges. Recent evidence suggests that upper respiratory infections (URIs) may contribute to MD's onset. This study examines the potential link between URIs and MD using data from the Korean National Health Insurance Service-National Sample Cohort (2002-2019). We analyzed 19,721 individuals with MD and 78,884 matched controls, adjusting for demographic factors and comorbidities using propensity score matching. Our results showed that individuals with a URI within one year prior to the index date exhibited a 2.01-fold greater likelihood of developing MD (95% confidence interval [CI] = 1.91-2.11, p < 0.001), while those with URIs within two years demonstrated a 1.54-fold higher probability (95% CI = 1.50-1.59, p < 0.001). Furthermore, we found that even remote URIs occurring up to two years before the index date significantly increased the risk of developing MD, underscoring the need for long-term patient follow-up. Overall, our study suggests that individuals with a history of URI may have an elevated risk of developing MD over multiple time frames, regardless of demographic or health profiles.

A review of Meniere’s disease – reflection of published MRI, ECochG and newer otoconial theory from Aotearoa New Zealand

ABSTRACT Vertigo, or the symptom of an hallucination of motion, is usually caused by disorders of the inner ear. Buried in the bone of the otic capsule, the inner ear is the most difficult human sensory organ to image and investigate. As an inner ear disorder, Meniere’s disease (MD) is characterised as recurrent vertiginous attacks, fluctuating hearing loss which can be progressive, tinnitus and aural fullness. Post-mortem studies, and now MRI inner ear imaging, show an excess of endolymph or endolymphatic hydrops (ELH) which has been assumed to usually commence in the cochlea. However, the cause of the initial hydrops is still debated. This paper reflects on the research published in Ōtautahi Christchurch, Aotearoa New Zealand. There is evidence from post-mortem studies, electrophysiological test, and some inner ear imaging that endolymphatic obstruction from clumps of detached saccular otoconia may be a cause of MD.

The genomic landscape of Ménière's disease: a path to endolymphatic hydrops

BackgroundMénière's disease (MD) is a disorder of the inner ear that causes episodic bouts of severe dizziness, roaring tinnitus, and fluctuating hearing loss. To date, no targeted therapy exists. As such, we have undertaken a large whole genome sequencing study on carefully phenotyped unilateral MD patients with the goal of gene/pathway discovery and a move towards targeted intervention. This study was a retrospective review of patients with a history of Ménière's disease. Genomic DNA, acquired from saliva samples, was purified and subjected to whole genome sequencing.ResultsStringent variant calling, performed on 511 samples passing quality checks, followed by gene-based filtering by recurrence and proximity in molecular interaction networks, led to 481 high priority MD genes. These high priority genes, including MPHOSPH8, MYO18A, TRIOBP, OTOGL, TNC, and MYO6, were previously implicated in hearing loss, balance, and cochlear function, and were significantly enriched in common variant studies of hearing loss. Validation in an independent MD cohort confirmed 82 recurrent genes. Pathway analysis pointed to cell–cell adhesion, extracellular matrix, and cellular energy maintenance as key mediators of MD. Furthermore, the MD-prioritized genes were highly expressed in human inner ear hair cells and dark/vestibular cells, and were differentially expressed in a mouse model of hearing loss.ConclusionBy enabling the development of model systems that may lead to targeted therapies and MD screening panels, the genes and variants identified in this study will inform diagnosis and treatment of MD.

Effective management of Meniere’s disease through Ayurveda- A case report

Meneirs disease is inner ear disease characterized by episodic vertigo, fluctuating hearing loss and tinnitus. The pathophysiology of the disease is obscure but theories postulated and stated that, due to decreased absorption or increased secretion of endolymphatic fluid in membranous labyrinth of the inner ear leads to pressure increase thus manifests the disease. There is no promising cure to this disease in conventional science only with life style adjustments, low salt diet, diuretics and surgery are used to manage the condition. The present case study patient with meniers disease was successfully managed with ayurveda treatment. A 55yrs old male patient complained of giddiness, reduced hearing and tinnitus since 9months which was hampering his daily activities was treated with panchakarma procedures and internal medicines. The exact meniers disease is not mentioned in samhitas but on the basis of symptoms and dosha predominance it is considered as vata-pitta pradhana tridoshaja vyadhi. As per dosha predominance it is treated with virechana (Purgation), Pratimarsha nasya (nasal medication) and internal medicines with Dhanadhanyadi kashaya, Cap ksheerabala and cap palsineuron, Tab Kamaduga rasa was administered. On functional scale level the stage 5 was reduced to 1 which shows good improvement. Meniers disease may satisfactory be managed with Ayurveda treatment.

Efficacy of endolymphatic duct blockage in treating Ménière's disease.

Ménière's disease (MD) is an idiopathic inner ear disorder characterized by recurrent episodes of episodic rotational vertigo, fluctuating hearing loss, tinnitus, and a feeling of ear stuffiness. Endolymphatic sac (ES)-related surgery is used primarily in patients with MD who have failed to respond to pharmacologic therapy. Endolymphatic duct blockage (EDB) is a new procedure for the treatment of MD, and related clinical studies are still scarce. This study aims to investigate the dynamic changes in endolymphatic hydrops (EH) and the long-term surgical outcomes in MD patients undergoing EDB, and to evaluate the impact of different types of ES on the surgical efficacy. A retrospective analysis was conducted on 33 patients with refractory MD who underwent EDB. Based on the morphology of their endolymphatic sacs, patients were divided into a normal-type group (n=14) and an atrophic-type group (n=19). The frequency of vertigo symptoms, hearing, vestibular function, and the dynamic changes of gadolinium-enhanced MRI of the inner ear were compared were compared before and after surgery between the 2 groups. Compared with the atrophic-type group, the patients in the normal-type group had a higher rate of complete vertigo control, better cochlear and vestibular function, and a lower endolymph to vestibule volume ratio (all P<0.05). In addition, 7 patients in the normal-type group were found to have reversal of EH, while no reversal of EH was detected in the atrophic-type group after surgery. The response to EDB treatment varies between normal and atrophic MD patients, suggesting that the 2 pathological types of endolymphatic sacs may have different underlying mechanisms of disease.

Otic Capsule Dehiscences Simulating Other Inner Ear Diseases: Characterization, Clinical Profile, and Follow-Up-Is Ménière's Disease the Sole Cause of Vertigo and Fluctuating Hearing Loss?

We present a series of six cases whose clinical presentations exhibited audiovestibular manifestations of a third mobile window mechanism, bearing a reasonable resemblance to Ménière's disease and otosclerosis. The occurrence of these cases in such a short period has prompted a review of the underlying causes of its development. Understanding the pathophysiology of third mobile window syndrome and considering these entities in the differential diagnosis of conditions presenting with vertigo and hearing loss with slight air-bone gaps is essential for comprehending this group of pathologies. A descriptive retrospective cohort study of six cases diagnosed at a tertiary center. All of them went through auditive and vestibular examinations before and after a therapeutic strategy was performed. Out of 84 cases of dehiscences described in our center during the period from 2014 to 2024, 78 belonged to superior semicircular canal dehiscence, while 6 were other otic capsule dehiscences. Among these six patients with a mean age of 47.17 years (range: 18-73), all had some form of otic capsule dehiscence with auditory and/or vestibular repercussions, measured through hearing and vestibular tests, with abnormalities in the results in five out of six patients. Two of them were diagnosed with Ménière's disease (MD). Another two had cochleo-vestibular hydrops without meeting the diagnostic criteria for MD. In two cases, the otic capsule dehiscence diagnosis resulted from an intraoperative complication due to a gusher phenomenon, while in one case, it was an accidental radiological finding. All responded well to the proposed treatment, whether medical or surgical, if needed. Otic capsule dehiscences are relatively new and unfamiliar entities that should be considered when faced with cases clinically suggestive of Ménière's disease, with discrepancies in complementary tests or a poor response to treatment. While high-sensitivity and specificity audiovestibular tests exist, completing the study with imaging, especially petrous bone CT scans, is necessary to locate and characterize the otic capsule defect responsible for the clinical presentation.

Therapeutic Response of Meniere's Disease Utilizing Sorbus domestica.

Introduction Meniere's disease (MD) is a chronic condition characterized by episodic attacks of vertigo, fluctuating hearing loss, and tinnitus. MD can impart a significant socioeconomic impact with associated progressive hearing loss. First-line therapies consist of diuretics and antihistamines, with second-line therapies including intratympanic steroids and pulse therapy. Third-linetreatments include endolymphatic sac surgery (ELSS) followed by intratympanic gentamicin injection and/or vestibular neurectomy. The gemmotherapy Sorbus domestica's inherent properties to regulate venous circulation and lymphatic drainage have been utilized in the European literature for the treatment of MD and the patients in this study. Methods Patients presenting for rehabilitation at Pulaski Health and Rehabilitation Facility with a history of vertigo were examined and, through history and specific exam, to define MD. This resulted in six patients whose symptoms and exam were consistent with MD and interfered with their therapeutic progression. These patients were offered and accepted treatment with Sorbus domestica for their MD. Results All patients responded with either resolution or significant improvement in their symptoms and hearing loss. Treatment also resulted in an improved and probably shortened rehabilitative course. All patients had no adverse reactions and were supplied with resources for continual treatment upon discharge. Conclusion Sorbus domestica is a safe and viable treatment option for MD. It has been useful, especially in treatment-resistant diseases, without side effects and can be utilized in initial cases with improvement or resolution of hearing loss.

A Multicenter Validity Study of Four Smartphone Hearing Test Apps in Optimized and Home Environments.

Pure tone audiometry (PTA) is the gold standard for hearing assessment. However, it requires access to specialized equipment. Smartphone audiometry applications (apps) have been developed to perform automated threshold audiometry and could allow patients to perform self-administered screening or monitoring. This study aimed to assess the validity and feasibility of patients using apps to self-assess hearing thresholds at home, with comparison to PTA. A multi-center, prospective randomized study was conducted amongst patients undergoing PTA in clinics. Participants were randomly allocated to one of four publicly-available apps designed to measure pure tone thresholds. Participants used an app once in optimal sound-treated conditions and a further three times at home. Ear-specific frequency-specific thresholds and pure tone average were compared using Pearson correlation coefficient. The percentage of app hearing tests with results within ±10 dB of PTA was calculated. Patient acceptability was assessed via an online survey. One hundred thirty-nine participants submitted data. The results of two at-home automated smartphone apps correlated strongly/very strongly with PTA average and their frequency-specific median was within ±10 dB accuracy. Smartphone audiometry performed in sound-treated and home conditions were very strongly correlated. The apps were rated as easy/very easy to use by 90% of participants and 90% would be happy/very happy to use an app to monitor their hearing. Judicious use of self-performed smartphone audiometry was both valid and feasible for two of four apps. It could provide frequency-specific threshold estimates at home, potentially allowing assessments of patients remotely or monitoring of fluctuating hearing loss. 2 Laryngoscope, 134:2864-2870, 2024.

Otoprotective Mechanisms of Carvone As An Antioxidant Agent Against Ototoxic Damage Caused By Paclitaxel

Objective: Ototoxicity is cellular damage caused by the use of solid treatments as chemotherapeutics in critical illnesses like cancer. The generation of free radicals is linked to fluctuating hearing loss caused by chemotherapeutics. Antioxidants can help to prevent ototoxicity-related oxidative damage. Carvone (CVN) is a monoterpene with excellent antioxidant properties that protect against oxidative damage. This study investigates the biochemical and functional aspects of CVN’s putative otoprotective mechanisms against paclitaxel (PCX)-induced ototoxicity.&#x0D; Methods: 24 Wistar albino rats were assigned into four different groups: Control, CVN, PCX, and PCX+CVN. Once a week, the control group received saline. The PCX group received 5 mg/kg PCX intraperitoneally once a week (4 times). Once a week, the CVN group received 50 mg/kg intraperitoneally. The PCX+ CVN group received 5 mg/kg PCX followed by 5 mg/kg CVN once a week. All animals were subjected to deterioration product otoacoustic emission testing before (day 0) and after drug administration (day 23).&#x0D; Results: PCX showed an ototoxic effect by weakening otoacoustic emission values. PCX leads to significant otoacoustic emission value shifts ameliorated by CVN co-treatment (for 2000Hz p&lt; .001, for 4000 levels p&lt; .01, for 6000Hz p&lt; .001, and for 8000 Hz p&lt; .01 in PCX+CVN group). Furthermore, the PCX group had significantly greater malondialdehyde levels and significantly lower glutathione levels in the cochlear tissues, compared to the other groups. Co-administered CVN with PCX reversed these effects, making oxidative stress parameters close to those of the control group (for GSH levels p&lt; .001, for MDA levels p&lt; .01 in the PCX+CVN group).&#x0D; Conclusion: According to the findings, CVN appears to preserve cochlear function in rats against the disruptive effects of PCX.

Fluctuating hearing loss secondary to spontaneous intracranial hypotension: A case report and review of the literature.

Fluctuating sensorineural hearing loss (SNHL) has multiple etiologies, most commonly Ménière's disease (MD), recurrent sudden SNHL, and autoimmune inner ear disorders. Fluctuating SNHL has rarely been described as a symptom of spontaneous intracranial hypotension (SIH). A 39-year-old previously healthy female presented with "Ménière's like" symptoms responsive to steroid treatment, which worsened during the day and improved in the supine position. Conservative treatment for MD consisting of low salt and caffeine diet and betahistine medication yielded no improvement. Secondary revision of brain imaging scans showed signs indicative of SIH, and a spinal cerebrospinal fluid leak was ultimately found and treated by a novel technique of transvenous fistula embolization by means of Onyx® glue, leading to gradual clinical improvement and near-complete resolution of symptoms. SIH should be considered as part of the differential diagnosis of fluctuating SNHL. Clinical and radiological features should be known and sought. We suspect that early diagnosis and treatment can lead to cure and prevent permanent auditory damage.

Hearing characteristics and otoradiological abnormalities in three patients with novel pathogenic variants of KMT2D-related Kabuki syndrome.

Kabuki syndrome 1 (KS1; OMIM:147920), which is characterized by distinctive dysmorphic facial features (such as arched eyebrows, long palpebral fissures with eversion of the lower lid, and large protuberant ears), intellectual disability, short stature, and dermatoglyphic and skeletal abnormalities, is brought on by pathogenic variants in KMT2D (OMIM:602113). In this work, three individuals with novel pathogenic KMT2D gene variants had their longitudinal audiological manifestations and ear structural characteristics outlined. The longitudinal audiological data from neonatal hearing screening and a battery of several hearing tests were evaluated. The battery of hearing tests included tympanometry, distortion product otoacoustic emission (DPOAE), click-evoked air-conduction auditory brain-stem response (AC-ABR), click-evoked bone-conduction auditory brain-stem response (BC-ABR), narrow band CE-chirp auditory steady-state response (NB CE-chirp ASSR), and pure-tone audiometry (PTA). Phenotype identification and whole exome sequencing (WES) were performed on recruited individuals. All three patients (two females and on male; last evaluations at 14 months, 11 months, and 5.7 years, respectively) failed the newborn hearing screening, and the audiological follow-up data revealed mild to profound fluctuating hearing loss, which was directly influenced by the incidence and severity of otitis media with effusion (OME). When OME occurred, the AC-ABR thresholds increased from 30-75 dBnHL to 45-90 dBnHL. The threshold for the BC-ABR and BC-PTA was between 25 and 50 dBnHL, indicating mild to moderate sensorineural hearing loss (SNHL). The high-resolution computed tomography (HRCT) pictures indicated that all three patients had middle and inner ear abnormalities. Middle ear anomalies showed as diminished mastoid gasification and ossicle dysplasia. Cochlear dysplasia, a dilated vestibule, fusion of the vestibule with the horizontal semicircular canals, and a short and thick horizontal semicircular canal were visible on images of the inner ear. This study recruited three individuals with three novel pathogenic variants (c.5104C>T, c.10205delA, and c.12840delC) of KMT2D who were identified at ages 27 days, 2 months, and 5.5 years. Hearing characteristics of three individuals with three novel pathogenic variants of KMT2D range from mild to profound fluctuating hearing loss with mild to moderate SNHL. HRCT scans showed that all three individuals had anatomical middle and inner ear abnormalities. KS 1 patients must get clinical therapy for OME, frequent auditory monitoring, and prompt intervention.

A Case of Chronic Meniere's Disease Improved by Korean Medicine Treatment Including Pharmacoacupuncture of Cervical and Temporomandibular Area - A Case Report

Meniere’s disease is characterized by episodic vertigo, fluctuating hearing loss, tinnitus and ear fullness. The main pathological finding is endolymphatic hydrops, but the etiology of disease and effective treatment methods are still disputed. Recently, Cervical spine disorders(CSD) and Temporomandibular disorders(TMD) have been attracting attention as one of the causes of Meniere’s disease. A 65-year-old female Meniere patient with musculoskeletal problems in the cervical and mandible area was treated by Korean medical therapies including pharmacoacupuncture treatment. After 5 weeks of treatment, there was no meaningful change in hearing level evaluated with pure tone audiometry, but the subjective symptoms of Meniere improved significantly. Numerical rating scale (NRS) decreased from 10 to 0 for hearing loss, 10 to 3 for tinnitus and 8 to 3 for ear fullness. Also NRS of cervicalgia was reduced from 5 to 0 after treatment. The result suggests that the Korean medical therapy including pharmacoapuncture targeting CSD and TMD could be safe and effective method for patients with Meniere’s disease.

How differences in anatomy and physiology and other aetiology affect the way we label and describe speech in individuals with cleft lip and palate.

Speech in individuals with cleft lip and/or palate (CLP) is a complex myriad of presenting symptoms. It is uniquely associated with the structural difference of velopharyngeal insufficiency (VPI), together with a wide and heterogeneous range of other aetiologies which often co-occur. The nature of the speech sound disorder (SSD) including VPI may also change over the course of an individual's care pathway. Differences in terminology and approaches to analysis are currently used, resulting in confusion internationally. Additionally, current diagnostic labels and classification systems in SSD do not capture the complexity and full nature of speech characteristics in CLP. This paper aims to explore the different aetiologies of cleft palate/VPI speech and to relate aetiology with speech characteristic(s). In so doing, it attempts to unravel the different terminology used in the field, describing commonalities and differences, and identifying overlaps with the speech summary patterns used in the United Kingdom and elsewhere. The paper also aims to explore the applicability of current diagnostic labels and classification systems in the non-cleft SSD literature and illustrate certain implications for speech intervention in CLP. The different aetiologies were identified from the literature and mapped onto cleft palate/VPI speech characteristics. Different terminology and approaches to analysis are defined and overlaps described. The applicability of current classification systems in SSD is discussed including additional diagnostic labels proposed in the field. Aetiologies of cleft palate/VPI speech identified include developmental (cognitive-linguistic), middle ear disease and fluctuating hearing loss, altered oral structure, abnormal facial growth, VPI-structural (abnormal palate muscle) and VPI-iatrogenic (maxillary advancement surgery). There are four main terminologies used to describe cleft palate/VPI speech: active/passive and compensatory/obligatory, which overlap with the four categories used in the UK speech summary patterns: anterior oral cleft speech characteristics (CSCs), posterior oral CSCs, non-oral CSCs and passive CSCs, although not directly comparable. Current classification systems in non-cleft SSD do not sufficiently capture the full nature and complexity of cleft palate/VPI speech. Our attempt at identifying the heterogeneous range of aetiologies provides clinicians with a better understanding of cleft palate/VPI speech to inform the management pathway and the nature and type of speech intervention required. We hope that the unravelling of the different terminology in relation to the UK speech summary patterns, and those used elsewhere, reduces confusion and provides more clarity for clinicians in the field. Diagnostic labels and classification require international agreement. What is already known on the subject Speech associated with cleft palate/velopharyngeal insufficiency (VPI) is a complex myriad of speech characteristics with a wide and heterogeneous range of aetiologies. Different terminology and speech summary patterns are used to describe the speech characteristics. The traditional classification of cleft palate/VPI speech is Articulation Disorder, although evidence is building for Phonological Disorder and contrastive approaches in cleft speech intervention. What this paper adds to existing knowledge This paper explores the range of aetiologies of cleft palate/VPI speech (e.g., altered oral structure, abnormal facial growth, abnormal palate muscle and iatrogenic aetiologies) and attempts to relate aetiology with speech characteristic(s). An attempt is made at unravelling the different terminology used in relation to a well-known and validated approach to analysis, used in the United Kingdom and elsewhere. Complexities of current diagnostic labels and classifications in Speech Sound Disorder to describe cleft palate/VPI speech are discussed. What are the potential or actual clinical implications of this work? There needs to be a common language for describing and summarising cleft palate/VPI speech. Speech summary patterns based on narrow phonetic transcription and correct identification of aetiology are essential for the accurate classification of the speech disorder and identification of speech intervention approaches. There is an urgent need for research to identify the most appropriate type of contrastive (phonological) approach in cleft lip and/or palate.

Bilateral Sensorineural Hearing Loss Related to a Skull Base Spontaneous Cerebrospinal Fluid Leak: A Case Report and Literature Review.

Spontaneous intracranial hypotension (SIH) is a rare condition characterized by orthostatic headache, pulsatile tinnitus, vertigo, nausea, and fluctuating hearing loss; this latter seems to be due to the development of cochlear endolymphatic hydrops following negative cerebrospinal fluid (CSF) pressure transmitted to the perilymphatic space through a patent cochlear aqueduct. We here describe a case of bilateral progressive sensorineural hearing loss (SNHL) due to intracranial hypotension caused by an undiagnosed spontaneous CSF leak from a skull base defect in a middle-aged woman. To the best of our knowledge, this is the first report describing a SNHL in a patient affected by SIH secondary to sphenoidal CSF leak.

Diffusion tensor imaging and auditory tractography to evaluate cochlear implant candidacy: a pilot study

Background Conventional radiological evaluation does not evaluate the functional status of the auditory pathway in patients scheduled for cochlear implantation (CI). Objectives Determine the value of diffusion tensor imaging [DTI] in the preoperative evaluation of some patients scheduled for CI. Material and Methods Patients with profound SNHL and inner ear and/or cochlear nerve anomalies or long standing SNHL were selected. They underwent conventional MRI images of the brain in three orthogonal planes, MR arterial spin labelling (ASL), perfusion, and DTI auditory tractography to determine functional status of the auditory pathways. Results Ten patients were included. Seven with bilateral SNHL, one with fluctuating hearing loss and one with long standing single sided deafness. In 8 patients the auditory pathway could be traced and functional maps could determine the side of possible better function. In the patient with progressive hearing loss DTI revealed major central pathway problems and CI was discouraged. In the patient with SSD, DTI revealed a robust intact pathway and CI was advised. Conclusions DTI and auditory tractography can help in outlining the functional integrity of the 33auditory pathway and assist in decision making before CI.

메니에르병 유사증상을 동반한 내림프낭에 발생한 콜레스테롤 육아종 1예

The endolymphatic sac tumor is a very rare neurological tumor. This could be accompanied with symptoms of Meniere’s disease, such as sensorineural hearing loss, tinnitus, dizziness. A 48-year-old man presented with right side fluctuating hearing loss lasting more than 10 years and intermittent vertigo. Magnetic resonance imaging (MRI) revealed mild enlargement with abnormal high signal intensity on T1-weighted at endolymphatic sac. The patient underwent right labyrinthectomy and surgical tumor resection. During surgery, reddish mass of filling retrofacial air cell nearby common crus was observed and histologic examination was performed. The mass was connected to the endolymphatic sac. Histopathological diagnosis is cholesterol granuloma. After 10 weeks of the surgery, vertigo had disappeared. At 7 years of postoperative follow-up, there was no recurrence of previous symptoms.

Symptoms: Dizziness and Deafness

Symptoms: Dizziness and Deafness

The Diagnostic Value of Multifrequency Tympanometry in Patients with Ménière’s Disease: A Prospective Analysis

Introduction: Diagnosing Ménière’s disease (MD) by its characteristics such as episodes of vertigo, fluctuating hearing loss, and tinnitus with aural fullness remains challenging. Available tests evaluating the presence of endolymphatic hydrops (EH) are often expensive or time assuming. An in-office quick and simple non-invasive diagnostic test is multifrequency tympanometry (MFT). It can measure conductance at 2 kHz probe tones, which was demonstrated to reflect variations in cochlear pressure. Previous studies investigating MFT as a diagnostic test for MD showed conflicting outcomes possibly biased by their retrospective design. Methods: We prospectively collected MFT results (Y width) in patients with dizziness and compared MFT test results in affected (group 1) and unaffected (group 2) ears of 37 MD subjects and in control ears of 33 non-MD subjects (group 3). Results: The mean value of the Y width in affected ears was 315.6 ± 70.2 daPa compared to 292.3 ± 98.6 daPa in unaffected ears in MD subjects and 259.4. ± 60.6 daPa in the non-MD group. A positive test result (i.e., a Y width of 235 daPa or more) was found in 35 ears in the MD group, 21 times involving the affected ear and 14 times involving the unaffected ear, compared to 16 in the non-MD group. No significant differences between the three groups could be demonstrated (p > 0.05). We found a sensitivity of 58.3% and specificity of 66.3% for detecting EH in an affected ear in MD subjects. Conclusion: There is a trend towards increased conductance tympanometry in affected ears. However, we noticed a high false positive rate of MFT and do not support standardized use of MFT as an additional diagnostic tool for detecting EH in MD patients. A negative test result on the contrary is unlikely related to EH.

Symptoms: Sudden Hearing Loss and Aural Fullness

Symptoms: Sudden Hearing Loss and Aural Fullness