Predictive nomograms and an algorithm for managing patients with probable Meniere's disease.

PURPOSE: To explore the efficacy of diagnostic tests in accurately reclassifying patients initially diagnosed with probable Meniere's disease (MD) into either definite or non-MD categories. MATERIALS AND METHODS: A retrospective cohort study was conducted at a neurotology clinic between 1/2016 and 5/2022. Patients underwent a battery of tests, from which sensitivity, specificity, positive and negative predictive values, as well as positive and negative likelihood ratios, were calculated. Additionally, prediction nomograms were developed. RESULTS: Of the 69 patients, 25 (36.2 %) were initially classified as definite MD, 21 (30.4 %), probable MD, and 23 (33.4 %) non-MD. The mean follow-up was 3.5 years. The sensitivity of electrocochleography (ECochG) was the highest (92 %), with a negative likelihood ratio of 15 %. Magnetic resonance imaging (MRI) with MD-protocol had the highest specificity (100 %), with a positive likelihood ratio of 100 %. Videonystagmography, video head impulse test, and cervical vestibular-evoked myogenic potentials, had lower sensitivity and specificity. We were able to reclassify 18 (86 %) patients with probable MD: 12 (57 %) were diagnosed with definite MD, and 6 (29 %) were diagnosed with non-MD, consistent with their clinical course. CONCLUSIONS: The combination of ECochG and MRI with MD-protocol provides the most reliable approach to reclassify patients with Probable MD, ensuring a precise and accurate diagnosis. Vestibular tests express the functional status of the labyrinth and may not be reliable. Our findings provide valuable insights into clinical decision-making for patients with Probable MD and raise the consideration of additional diagnostic tests as supplementary to the existing clinical-only diagnosis criteria.

New non-contrast MRI of endolymphatic hydrops in Ménière's disease considering inversion time.

Objectives: Three-tesla MRI with gadolinium-based contrast agents is important in diagnosing Ménière's disease. However, contrast agents cannot be used in some patients. By using the compositional difference between the inner ear endolymph and perilymph, we performed basic and clinical research focused on potassium ions and protein to find the optimal parameters for visualizing endolymphatic hydrops on MRI without contrast. We then examined the relationship between severity stage and visualization rate of endolymphatic hydrops. Methods: In phantom experiments simulating the endolymph and perilymph, we explored MRI parameters that could be used to separate endolymph from perilymph by gradually changing the inversion time. We then used these parameters to perform both new non-contrast MRI and contrast MRI on the same day in Ménière's disease patients, and we compared the visualization rates of endolymphatic hydrops under the two modalities. Fifty patients were selected from 478 patients with Ménière's disease of different severity stages; 12 patients had asthma and allergy to contrast agents. Results: The higher the disease stage, the higher the endolymphatic hydrops visualization rate. The new non-contrast MRI gave significantly higher (p < .01) visualization rates of endolymphatic hydrops on the affected side in patients at Stage 3 or above than in Stages 1 and 2 combined. Conclusion: New non-contrast MRI with parameters focusing on the endolymph-perilymph difference in the density of protons surrounding the potassium ions and protein can produce images consistent with endolymphatic hydrops. We believe that this groundbreaking method will be useful for diagnosing Ménière's disease in patients. Evidence Level: Clinical studies are at evidence level 3 in non-randomized controlled trials.

Outcomes of deep anterior lamellar keratoplasty in eyes with healed hydrops - A retrospective interventional analysis.

AIM: The aim of the study was to analyze the techniques and outcomes of deep anterior lamellar keratoplasty (DALK) done in eyes with healed hydrops. METHODS: Thirty-one eyes with paracentral scars following acute hydrops underwent DALK and were included in the study. Manual layer-by-layer dissection with stromal air injection was done in all cases, keeping dissection of the site of preexisting perforation to the end. RESULTS: Out of the 31 eyes, in 28 eyes, DALK could be completed, and in 3 eyes, procedure had to be converted to penetrating keratoplasty. Visual outcomes in all cases were good, with 26 out of 28 eyes (92.8%) achieving a best-corrected visual acuity (BCVA) of 6/12 or better. Preoperative mean BCVA of LogMAR 1.19 ± 0.27 improved to a postoperative mean BCVA of LogMAR 0.23 ± 0.08 (P < 0.00001). Postoperative mean refractive astigmatism at the last follow-up was -2.69 ± 0.82 D with postoperative a spherical equivalent of -2.31 ± 0.7 D. Complications included double anterior chamber in one eye and secondary angle closure in two eyes. Stromal rejection in one eye was managed with steroids and one eye with suture infiltrate needed selective suture removal. CONCLUSION: Layer-by-layer dissection with limited air injection into the stroma has shown good visual outcomes in patients undergoing DALK following healed hydrops, and DALK can very well be the primary treatment option even in such cases with a paracentral posthydrops scar.

Clinical Management of Hydrops Fetalis in a Premature Neonate in India: A Case Report.

Hydrops fetalis has classically been defined as the presence of extracellular fluid in at least two fetal body compartments. This fluid collection includes skin edema (> 5 mm thickness), pericardial effusion, pleural effusion, and ascites. Here we present a case of a 29-year-old female with antenatally diagnosed severe hydrops fetalis which was postnatally successfully managed. Despite recent advances, immune hydrops are still a challenge for healthcare workers in third-world nations.

Magnetic resonance imaging evaluation of cochlear and vestibular nerve calibre: a case-control study in Ménière's disease and endolymphatic hydrops.

PURPOSE: To compare the calibre of the cochlear (CN), superior vestibular (SVN) and inferior vestibular (IVN) nerves on magnetic resonance imaging (MRI), both between Ménière's Disease (MD) ears and clinical controls, and between inner ears with and without endolymphatic hydrops (EH) on MRI. METHODS: A retrospective case-control study evaluated patients undergoing MRI for suspected hydropic ear disease from 9/2017 to 8/2022. The CN, SVN, IVN and facial nerve (FN) diameters and cross-sectional areas (CSA) were measured on T2-weighted sequences whilst EH was evaluated on delayed post-gadolinium MRI. Absolute nerve calibre (and that relative to the FN) in unilateral definite MD ears (2015 Barany criteria) was compared to that in both asymptomatic contralateral ears and clinical control ears. Nerve calibre in ears with severe cochlear and vestibular EH was compared to ears without EH. t tests or Wilcoxon signed-rank test/Mann-Whitney U test were applied (p < 0.001). RESULTS: 173 patients (mean age 51.3 ± 15.1, 65 men) with 84 MD (62 unilateral) and 62 clinical control ears were studied. Absolute and relative CN dimensions were decreased in both MD ears (CSA and diameter) and the contralateral asymptomatic ears (CSA) when compared to clinical controls (p < 0.001). Absolute nerve dimensions were reduced in both severe vestibular EH (CN, IVN and SVN) and severe cochlear EH (CN) (p < 0.001), however this was not evident when adjusted according to facial nerve calibre. CONCLUSION: There is decreased absolute CN calibre in both symptomatic and asymptomatic MD ears as well as ears with severe cochlear and vestibular EH on MRI.

Large placental chorioangioma with maternal and perinatal morbidity.

INTRODUCTION: Placental chorioangioma is a benign placenta tumour. Majority of cases, the placental chorioangioma are small and no maternal and fetal complications. We highlight a case diagnosed with large placental chorioangioma with an intrapartum event associated with significant maternal and perinatal morbidity. METHOD: A 38-year-old woman, Gravida 3 Para 1, with one previous miscarriage, presented with preterm labour at 33 weeks gestation. Antenatally, she was referred to a feto-maternal specialist for finding a placental tumour size 12 × 10 cm. Features are consistent with placental chorioangioma with polyhydramnios. The anomaly scan was normal. Antenatal fetal surveillance with Doppler studies were normal. RESULTS: During this admission, corticosteroid was given together with a tocolytic agent and opioid analgesia. Unfortunately, the labour progressed, and the patient felt reduced in fetal movement. The cardiotograph showed suspicious tracing. We proceed with emergency caesarean delivery. The placenta was sent for histopathology assessment which confirmed a large placental chorioangioma. The baby was born with Apgar's score of 9 at 1 min, pH of 7.28 and lactate of 7.28 with anaemia and thrombocytopenia. The uterus developed intermittent uterine atony, and the uterotonic agent was given. She recovered well post-delivery. The baby was admitted to the neonatal intensive care unit (NICU) and received a blood product transfusion and discharged from NICU on day 15 of life. DISCUSSION: Large placental chorioangioma is associated with polyhydramnios, preterm labour, postpartum haemorrhage, fetal anaemia, fetal distress, fetal hydrops and possible perinatal death. Multidisciplinary team involvement with feto-maternal specialists, anaesthetic and neonatologists would improve the outcome of both mother and fetus.

NT >99th centile and cystic hygroma: Which diagnosis, which management and outcome.

OBJECTIVES: To evaluate the prevalence of chromosomal anomalies in fetuses affected by increased nuchal translucency(iNT >99Th centile), cystic hygroma (CH) and cases progressing to fetal hydrops (NIHF) in order to correlate this result to prognosis and perinatal fetal outcomes, improving patient's counseling. METHODS: From January 2020 to December 2023, first trimester screening according to FMF criteria were performed in "Maternal fetal medicine Unit" of Foggia's hospital. We studied and collected clinical data of fetuses affected by nuchal translucency >99th centile (iNT), CH and cases progressing to Fetal Hydrops (non-immune fetal hydrop, NIFH). In these selected cases, we evaluated fetal Karyotype to demonstrate the presence of chromosomal abnormalities, associated to fetal structural anomalies and different pregnancy outcome. RESULTS: We have evaluated 45 cases of iNT, CH, and NIFH, identified by ultrasound during first trimester screening. Of these 45 pregnant women, four were lost to follow-up. Of the 41 pregnancies, 20 cases (48.8%) delivered at our division with newborns discharged in good health. Of the 21 patients (51.2%) who miscarried, only two miscarried spontaneously; the other 19 decided on a medical termination of pregnancy (MTP). The 19 patients who decided for MTP showed genetic abnormalities and/or malformation. The 20 cases of fetuses discharged as healthy newborns were enrolled with the following diagnoses: 5 had a diagnosis of CH (20% of cases), 1 case of NIFH (5% of cases), and 14 of iNT (75% of cases). All these fetuses had a normal karyotype and no major malformations. CONCLUSIONS: Isolated CH diagnosis is more frequent than described in Literature (5 cases out of 12: 41.7%) and their prognosis are better than previously described, with the same outcomes of fetuses with iNT without associated anomalies. The possibility of early diagnosis of chromosomal anomalies, associated malformations or the evolution into hydrops is essential for a complete consultation.

Intrauterine transfusion in hydropic fetuses: An outcome analysis.

The objective of this study is to determine the perinatal outcome in pregnancies with hydropic fetuses. The study was a retrospective evaluation of data on intrauterine transfusion (IUT) done in hydropic fetuses for correction of severe anemia from December 2017 to August 2021 in AIIMS Jodhpur. The retrospective case series involves five cases that underwent IUT for severe fetal anemia. All had a sign of hydrops at the time of presentation. Out of five cases, four were of alloimmunized pregnancies while one was of hydrops fetalis secondary to parvovirus infection. The presence of severe hydrops at the time of presentation is a poor prognostic factor affecting fetal survival post-IUT therapy.

Maternal isoimmunization associated fetal anemia: A case report.

Maternal isoimmunization occurs when a pregnant woman develops an immune reaction due to the inheritance of a red-cell antigen, which is paternally derived and can result in fetal anemia, hemolysis, fetal death, and hydrops fetalis as the antibodies might travel through the placenta and get adhered to the antigens present in the erythrocytes of the fetus. This report highlights a rare case of Rh isoimmunization leading to fetal anemia in a 26-year-old female and evaluates the impact of intrauterine transfusion (IUT) in terms of the gestational age at delivery along with the mode of delivery, procedural complications, and overall survival rate of the fetus. In conclusion, the most frequent cause of fetal anemia is Rh alloimmunization, which should be taken into consideration while making a differential diagnosis throughout the assessment. Improvements in IUT procedures and earlier detection of the MCA-PSV by Doppler ultrasonographic examination have also contributed to better results.

Glucose-6-phosphate dehydrogenase deficiency as a cause for nonimmune hydrops fetalis and severe fetal anemia: A systematic review.

BACKGROUND: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an X-linked recessive disorder that predisposes individuals to hemolysis due to an inborn error of metabolism. We performed a systematic literature review to evaluate G6PD deficiency as a possible etiology of nonimmune hydrops fetalis (NIHF) and severe fetal anemia. METHODS: PubMed, OVID Medline, Scopus, and clinicaltrials.gov were queried from inception until 31 April 2023 for all published cases of NIHF and severe fetal anemia caused by G6PD deficiency. Keywords included "fetal edema," "hydrops fetalis," "glucose 6 phosphate dehydrogenase deficiency," and "fetal anemia." Cases with workup presuming G6PD deficiency as an etiology for NIHF and severe fetal anemia were included. PRISMA guidelines were followed. RESULTS: Five cases of G6PD-related NIHF and one case of severe fetal anemia were identified. Four fetuses (4/6, 66.7%) were male and two fetuses (2/6, 33.3%) were female. Mean gestational age at diagnosis of NIHF/anemia and delivery was 32.2 ± 4.9 and 35.7 ± 2.4 weeks, respectively. Four cases (66.7%) required a cordocentesis for fetal transfusion, and two cases (33.3%) received blood transfusions immediately following delivery. Among the four multigravida cases, two (50%) noted previous pregnancies complicated by neonatal anemia. When reported, the maternal cases included two G6PD deficiency carrier patients and two G6PD-deficient patients. Exposures to substances known to cause G6PD deficiency-related hemolysis occurred in 3/6 (50%) cases. CONCLUSION: Six cases of NIHF/severe fetal anemia were associated with G6PD deficiency. While G6PD deficiency is an X-linked recessive condition, female fetuses can be affected. Fetal G6PD deficiency testing can be considered if parental history indicates, particularly if the standard workup for NIHF is negative.

Prenatal parvovirus B19 infection.

Parvovirus B19 (B19V) causes erythema infectiosum, a.k.a., fifth disease. This disease primarily affects children. It is generally self-limiting and subsides after 1-2 weeks. In pregnancy, the virus can cross the placenta and result in a fetal infection. This may lead to severe fetal anemia, hydrops fetalis, a miscarriage, or intrauterine fetal death. The risk of long-term sequelae also appears to be increased. About one-third of pregnant women are not immune to B19V and, therefore, are at risk to contract a primary infection. The seroconversion rate during pregnancy is generally around 1-2%. During a primary infection, maternal-fetal transplacental transmission of B19V occurs in about 30-50% of the cases and the risk of fetal infection increases with advancing gestational age. The risk of severe fetal anemia or hydrops is around 3-4% overall and is around 6-7% if the primary infection occurs before 20 weeks' gestation. Fetal monitoring in women with a primary B19V infection includes regular ultrasound examinations looking for evidence of hydrops fetalis and Doppler measurements of the middle cerebral artery peak velocity. Fetal blood sampling is performed if a significant anemia is suspected and, if such is found, an intrauterine blood transfusion is needed. This article provides an overview of the epidemiology, pathogenesis, clinical manifestations, diagnostic methods, and management of B19V infection during pregnancy.

An unenhanced 3D-FLAIR sequence using long repetition time and constant flip angle to image endolymphatic hydrops.

OBJECTIVES: To evaluate a three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) sequence using a long repetition time (TR) and constant flip angle (CFA) in differentiating between perilymph and endolymph in a phantom study, and unenhanced endolymphatic hydrops (EH) imaging in a patient study. METHODS: Three solutions in similar ion and protein concentrations with endolymph, perilymph, and cerebrospinal fluid were prepared for variable flip angle (VFA) 3D-FLAIR (TR 10,000 ms) and CFA (120°) 3D-FLAIR using different TR (10,000, 16,000, and 20,000 ms). Fifty-two patients with probable or definite Meniere's disease received unenhanced CFA (120°) 3D-FLAIR using a long TR (20,000 ms) and 4-h-delay enhanced CFA (120°) 3D-FLAIR (TR 16,000 ms). Image quality, signal-to-noise ratio (SNR), and contrast-to-noise ratio (CNR) of them were compared. Agreement in the evaluation of the EH degree between them was analyzed. RESULTS: In the phantom study, CNRs between perilymphatic and endolymphatic samples of VFA 3D-FLAIR (TR 10,000 ms) and CFA 3D-FLAIR (TR 10,000, 16,000, and 20,000 ms) were 6.66 ± 1.30, 17.90 ± 2.76, 23.87 ± 3.09, and 28.22 ± 3.15 (p < 0.001). In patient study, average score (3.65 ± 0.48 vs. 4.19 ± 0.40), SNR (34.56 ± 9.80 vs. 51.40 ± 11.27), and CNR (30.66 ± 10.55 vs. 45.08 ± 12.27) of unenhanced 3D-FLAIR were lower than enhanced 3D-FLAIR (p < 0.001). Evaluations of the two sequences showed excellent agreement in the cochlear and vestibule (Kappa value: 0.898 and 0.909). CONCLUSIONS: The CFA 3D-FLAIR sequence using a long TR could be used in unenhanced EH imaging with high accuracy. CLINICAL RELEVANCE STATEMENT: Unenhanced imaging of endolymphatic hydrops is valuable in the diagnosis and follow-up of patients, especially those who cannot receive contrast-enhanced MRI. KEY POINTS: Ion and protein concentration differences can be utilized in differentiating endolymph and perilymph on MRI. Endolymphatic and perilymphatic samples could be differentiated in vitro on this 3D-FLAIR sequence. This unenhanced 3D-FLAIR sequence is in excellent agreement with the enhanced constant flip angle 3D-FLAIR sequence.

Simultaneous Muraine Sutures and Excimer Laser-Assisted Penetrating Keratoplasty for Acute Keratoconus.

Background: Acute keratoconus (acute KC), which affects approximately 1.6-2.8% of keratoconus (KC) patients, is a pathological condition of the cornea characterized by stromal edema due to entry of aqueous humor through a tear in Descemet's membrane. Methods: We present a novel combination of surgical procedures that allows swifter visual recovery in a consecutive, retrospective case series. The new surgical procedure for acute KC consists of a combination of Muraine corneal sutures to smooth the corneal curvature and Excimer laser-assisted penetrating keratoplasty and was performed in six acute KC patients from 2019 to 2022 at the Department of Ophthalmology, University Hospital of Martin-Luther-University Halle-Wittenberg (UMH), Germany. We monitored data on preoperative status, operative details, intraoperative and postoperative complications and visual outcomes were analyzed. Results: The mean age was 41.5 ± 13.5 years (3 OD, 3 OS). Neurodermatitis was present in 3 patients (50%). All patients received significant visual benefits from the procedure. Preoperative BCVA was hand motion (logMAR 3.0) in all patients; postoperatively, BCVA improved significantly logMAR 0.03 ± 0.09 [range: 0.2-0.4; p < 0.001, FUP 20+/-10 months). Visual acuity remained stable throughout the roughly biannual follow-ups. One patient developed endothelial graft rejection after 2 years. During the last examination, all eyes had clear grafts and stable curvatures, K1 and K2 being 42.43 ± 4.17 D and 44.95 ± 4.07 D, respectively, and mean corneal astigmatism was 2.61 ± 1.74 D. The thinnest corneal thickness was 519 ± 31 µm. A graft size of 8.0 × 8.1 mm was the most beneficial. Conclusions: in patients with acute KC and hydrops, a penetrating keratoplasty with Muraine corneal sutures is successful in terms of graft clarity and visual outcome. Combining the procedures allows quicker visual recovery. Patients with a history of neurodermatitis should have preoperative and postoperative dermatologic treatment and close follow-up for possible complications.

Temporal Bone Histopathology of Atypical Cogan Syndrome.

Cogan's syndrome is a rare disorder first clinically defined in 1945, characterized by nonsyphilitic interstitial keratitis and progressive audiovestibular symptoms. Later, patients with audiovestibular dysfunction and various types of inflammatory eye disease were classified as having atypical Cogan's syndrome. The etiology and pathogenesis of Cogan's syndrome remain largely unknown. Here, we report a case of atypical Cogan's syndrome with a histological assessment of the temporal bone during the acute disease period. Temporal bone histology was compared to age- and gender-matched normal control, and our findings revealed endolymphatic hydrops and degenerative changes in various parts of the inner ear. Laryngoscope, 2024.

Hydrops regression after vestibular denervation - longitudinal magnetic resonance study in patients with severe Meniere's disease treated with vestibular neurectomy.

OBJECTIVES: The aim was to evaluate endolymphatic hydrops in patients with severe Ménière's disease (MD) before and after vestibular neurectomy to verify if vestibular denervation results in hydrops regression. METHODS: Magnetic resonance imaging was performed after intravenous gadolinium injection in twenty patients with unilateral definite MD before and after the vestibular neurectomy. Clinical symptoms and audiovestibular tests were evaluated. Follow-up intervals ranged from 18 to 35 months after the surgery. RESULTS: Endolymphatic hydrops were visualized in all patients in the preoperative scans. After the vestibular neurectomy, all patients presented a complete resolution of vertigo episodes. Regression of the endolymphatic hydrops was observed in 35% and 15% of cases analyzing cochlea and vestibule, respectively. In 71.43% of patients with utricular herniation into the lateral semicircular canal, withdrawal of the hernia was visualized. Asymmetrical contrast enhancement in the cochlea regressed in 17.64% of cases. Analyzing all the parameters collectively, in 60% of patients, partial regression of at least one of the radiological signs was confirmed in the follow-up examination. No progression of the endolymphatic hydrops was visualized after the surgery in either the cochlea or the vestibule. CONCLUSIONS: Vestibular neurectomy is an effective treatment, eliminating vertigo attacks and improving the quality of life in patients with MD. Magnetic resonance imaging of the inner ear allows visualization of changes in endolymphatic hydrops degree after treatment. Regression of the endolymphatic hydrops after vestibular neurectomy suggests that vestibular denervation may effectively halt the progression of the endolymphatic space dilatation and result in hydrops regression.

Relationship Between Cochlear Lateral Wall Changes and Endolymphatic Hydrops in Otitis Media.

OBJECTIVES: Despite otitis media and various disease processes being associated with endolymphatic hydrops (EH), an exact explanation of the pathophysiology has yet to be reported. This study aimed to investigate the changes in the cochlear lateral wall structures and their potential correlation with the presence and severity of cochlear EH in acute and chronic otitis media cases. The investigations were conducted in both chinchilla animal model and human temporal bone specimens. METHODS: We studied a total of 15 chinchilla and 25 human temporal bones from our collection, which were categorized into acute otitis media, chronic otitis media (COM), and control groups. Through quantitative analysis, we measured the area of cochlear lateral wall structures and observed the presence and the degree of EH using light microscopy. RESULTS: No significant changes were determined in the area of the spiral ligament (p > 0.05) across the species. However, a significant (p < 0.05) decrease in the mean area of the stria vascularis in the basal turn was identified in COM groups compared to controls of both species. Chinchilla model additionally exhibited pathology extending to the lower mid turn. A negative correlation was found between the mean strial area and the severity of EH in both the animal model and human samples. CONCLUSIONS: COM associated with significant changes in the stria vascularis that may lead to significant increase in the degree of EH. The presented animal model exhibited parallel findings with human samples, suggesting its viability as a valuable model for future studies. LEVEL OF EVIDENCE: N/A Laryngoscope, 2024.

Fetal Hydrops Associated With 47,XXX: A Case Report and Literature Review.

This report aims to investigate the association between 47,XXX and fetal hydrops by examining a clinical case and performing a comprehensive review of the relevant literature. A 34-year-old Japanese woman, gravida 2, para 1, was diagnosed with fetal hydrops at 27 weeks' gestation. Prenatal testing revealed a 47,XXX karyotype. Interventions included thoracocentesis and a thoracoamniotic shunt. A cesarean delivery was performed at 34 weeks and the female neonate initially had respiratory challenges. After 69 days in the neonatal intensive care unit, the infant was discharged in stable condition, and the 47,XXX karyotype was confirmed. This case may add evidence suggesting an association between 47,XXX and fetal hydrops. Chromosomal abnormalities are causes of fetal hydrops, but its association with 47,XXX remains unclear. Providing comprehensive information on this condition to couples is crucial, and considering the inclusion of fetal hydrops in the list of associated conditions might be advisable.

Sudden Sensorineural Hearing Loss in Patients Aged from 15 to 40 Years.

Objectives: The purpose of this study was to investigate the hearing characteristics and causes of sudden sensorineural hearing loss (SSNHL) in patients aged from 15 to 40 years, focusing on audiological outcomes one year after the diagnosis. Methods: The medical records of individuals with SSNHL who were referred to our tertiary-level audiologic center were reviewed. All patients had undergone comprehensive diagnostic evaluations, including high-resolution 3D-FLAIR delayed magnetic resonance imaging (MRI), cone beam computed tomography (CBCT), and screening for coagulation, infectious, and autoimmune diseases. Results: Overall, 56 patients (mean age 28.1 ± 7.6 years) were included in the study. The hearing threshold in the affected ear improved significantly from 56.0 ± 18.0 dB at the diagnosis to 46.9 ± 22.3 dB after one year (p = 0.02). The degree of hearing loss, audiometric configurations, hearing improvements, and adherence to hearing treatments showed considerable variability among patients. Aural fullness, tinnitus, and hyperacusis were the predominant symptoms associated with SSNHL, and their prevalence decreased significantly over time. The diagnostic protocol led to the identification of the specific cause of SSNHL in 75% (42/56) of patients. The known etiology was found to be otological (39.3%), infectious (21.4%), autoimmune (7.1%), vascular (5.4%), or neoplastic (1.8%). In particular, Menière's disease (n = 12), isolated cochlear endolymphatic hydrops (n = 6), HSV-1 (n = 5), and EBV (n = 4) infections were the most frequent causes of SSNHL. Conclusions: The identification of the specific etiology of SSNHL may facilitate a more personalized approach to management and treatment.

Potential Application of Hydrops MR Imaging: A Systematic Review.

BACKGROUND: Diagnostic dilemma between clinical Meniere's disease and radiological endolymphatic hydrops (EH) has emerged since the introduction of hydrops magnetic resonance imaging (MRI). The aim of this study is to explore the potential application of hydrops MRI on diagnosing the EH. METHODS: This review was developed from peer-reviewed articles published in those journals listed on journal of citation reports. The MEDLINE database of the US National Library of Medicine, Scopus, and Google Scholar were used to collect articles based on the guidelines (PRISMA 2020 statement) for reporting reviews. RESULTS: Initially, 470 articles were retrieved from 1983 to 2023, and 80 relevant articles were ultimately selected. The sensitivity (69%-92%) and specificity (78%-96%) values varied from each laboratory for detecting EH via hydrops MRI, probably due to candidate selection and the grading system employed. CONCLUSION: The application of hydrops MRI allows (1) differentiation between EH and sudden sensorineural hearing loss; (2) determination of the affected side of EH; and (3) confirmation of the diagnosis of EH concomitant with other disorders. Notably, not all differentials for EH can be visualized on MR images. One of the existing gaps to be filled is that updated hydrops MRI fails to identify distortion, that is, rupture, collapse, fistula, or fibrosis of the inner ear compartments, akin to what histopathological evidence can demonstrate. Hence, enhanced ultrahigh resolution of hydrops MRI is required for demonstrating fine structures of the inner ear compartments in the future.

Efficacy of 100 hz sound stimulation on saccular dysfunction in meniere's disease.

BACKGROUND: Although various medical remedies have been attempted to alleviate the symptoms of Meniere's disease (MD), the risk-benefit ratios of these various treatments remain debatable. OBJECTIVE: We investigated the efficacy of sound stimulation of 100 Hz for treating vestibular dysfunction in patients with Meniere's disease (MD). MATERIALS AND METHODS: Patients with definitive MD with intractable vestibular symptoms and endolymphatic hydrops (EH) in the inner ear were evaluated. The experimental group received sound stimulation of 75 dB at a frequency of 100 Hz for 5 min, and the control group received sound stimulation of 75 dB at a frequency of 250 Hz for 5 min. Cervical vestibular-evoked myogenic potentials (cVEMPs) were measured before and after each sound stimulation, and the results of the clinical tests were compared between the two patient groups. RESULTS: Significant increases in cVEMP amplitudes were observed after sound stimulation of 100 Hz in ears with vestibular endolymphatic hydrops, although no such improvement was observed in the control group. CONCLUSION: Sound stimulation of 75 dB at a frequency of 100 Hz leads to improvement in cVEMP amplitude in patients with definitive MD. Adequate sound stimulation might be a new method for treating vestibular dysfunction associated with MD.