遅発性内リンパ水腫

OBJECTIVE: Endolymphatic hydrops (EH), Menière's disease (MD) pathological hallmark, is visualized via 3 T delayed phase MRI. This study examines MRI correlations between EH severity and migraine brain biomarkers. METHODS: Retrospective review of 144 inner ear MRI scans yielded 108 patients with confirmed EH. Two blinded radiologists graded EH, assessed migraine markers (white matter lesions (WML), frontal predominance of WML, enlarged perivascular spaces), and measured cranial nerve volumes. Binary logistic regression tested associations. RESULTS: Mean age 53.6 ± 15.8 years (56.5% female). Grade 2 vestibular EH predominated bilaterally; grade 2 cochlear EH affected 34-43%. No EH associations with migraine biomarkers or cranial nerve volumes were found (all p > 0.05). Left vestibular EH grade 1 linked to sudden hearing loss (OR 5.205, p = 0.018); left cochlear EH grade 1 to aural fullness (OR 5.019, p = 0.016). CONCLUSION: EH severity was not associated with established migraine MRI biomarkers in this cohort. These findings support the concept that EH and migraine-related structural brain changes may represent distinct imaging phenomena.

BACKGROUND: Unilateral Ménière's disease (MD) is characterized by severe episodic attacks of rotational vertigo and audiovestibular signs that include fluctuating sensorineural hearing loss, aural fullness, tinnitus, and vestibular loss. PURPOSE: To investigate the relationship between the degree of audiovestibular loss, symptoms, and the number of vertigo attacks during the previous 6 months and 1 month of patients with MD. STUDY SAMPLE: Sixty patients with active MD refractory to oral treatment. DATA COLLECTION AND ANALYSIS: Hearing (pure-tone audiometry and speech discrimination) and vestibular (caloric test, vestibular evoked myogenic potential [VEMP]) tests were performed. Symptoms were scored using the following validated questionnaires: Vertigo Symptom Scale-short form, Dizziness Handicap Inventory, Tinnitus Handicap Inventory, Aural Fullness Scale, and Functional Level Scale. Linear regression, a factor analysis, and a correlation analysis were conducted. RESULTS: Patients experienced a mean number of 16 attacks (range, 2-65) during the previous 6 months and five attacks (range, 0-30) during the previous 1 month. Hearing and vestibular functions were reduced in the affected ear. Linear regression, the factor analysis, and the correlation analysis showed no association between the degree of audiovestibular loss and the number of vertigo attacks during the previous 6 months or 1 month or symptoms of disease activity. An exploratory analysis showed evidence of some associations between the degree of hearing loss, caloric paresis, and VEMP asymmetry, indicating a consistent pattern of loss across the cochlear semi-circular canals and saccules. CONCLUSION: Although audiovestibular tests help establish diagnoses, they cannot determine current disease activity of unilateral refractory MD. CLINICAL RELEVANCE STATEMENT: It may be prudent to assume that patients with MD and high remaining vestibular function in the affected ear would experience more frequent episodes of vertigo and intense dizziness and experience more troublesome symptoms. Conversely, it may be prudent to assume that patients with MD with low remaining vestibular function in the affected ear would experience fewer episodes of vertigo; however, as shown by these results, this does not seem to be the case. No obvious audiovestibular markers suggest that intratympanic therapy for vertigo treatment should be delayed.

BACKGROUND: To evaluate the clinical outcomes and safety profile of round window reinforcement (RWR) as a minimally invasive surgical treatment for patients with semicircular canal dehiscence syndrome (SCDS). METHODS: This retrospective case series analyzed 7 patients (mean age: 59 years) diagnosed with SCDS who underwent transcanal RWR between June 2024 and June 2025 at the Kopfzentrum Bielefeld. Inclusion criteria followed the Bárány Society consensus diagnostic standards. Clinical symptoms, audiometric findings, and vestibular test results-including Dizziness Handicap Inventory (DHI) scores-were assessed pre and postoperatively. In 2 patients, endolymphatic hydrops was diagnosed via delayed contrast-enhanced magnetic resonance imaging and monitored postoperatively. RESULTS: All patients completed follow-up (mean: 35 months). Improvement in auditory symptoms was observed in 5 of 7 patients (71.4%), including tinnitus relief in 83.3% and hyperacusis relief in 75%. Vertigo improved in 50% of symptomatic cases. No postoperative deterioration in symptoms, DHI score, or hearing was observed. Audiometric outcomes showed a non-significant mean change in air-bone gap (±2.5 dB). No intra- or postoperative complications occurred. In patients with concomitant hydrops, auditory improvement was noted, though vertigo persisted. CONCLUSION: Round window reinforcement appears to be a safe and effective therapeutic option for selected patients with SCDS, particularly those presenting predominantly auditory symptoms or contraindications to more invasive procedures. Further prospective studies are needed to validate these findings and define the role of RWR in the broader surgical management of third window syndromes.

OBJECTIVE(S): Endolymphatic hydrops is associated with factors such as autoimmune responses and vascular issues. Vitamin D plays a vital role in immune regulation and is associated with various inflammatory conditions. However, evidence regarding the correlation between endolymphatic hydrops and vitamin D is lacking. We clarified the correlation between vitamin D and endolymphatic hydrops to explain clinical symptoms and intervene in endolymphatic hydrops formation and progression. METHODS: Intravenous gadolinium-enhanced delayed magnetic resonance imaging three-dimensional-fluid-attenuated inversion recovery technique was used to screen unilateral endolymphatic hydrops patients. Vestibular and cochlear hydrops were categorized as follows: 0 = normal, 1 = mild hydrops, and 2 = severe hydrops. Values of low, middle, and high-frequency bands of pure tone audiometry (low-frequency-, middle-frequency-, and high-frequency-pure tone audiometry); serum 25(OH)D3 level; slow phase velocity of bilateral ears in the caloric test; and summating potential to action potential ratio of affected ears in electrocochleography were documented. Correlations between vestibular-cochlear hydrops grade and these examinations were further analyzed. RESULTS: Overall, 82 endolymphatic hydrops patients were included. The grade of vestibular-cochlear hydrops was significantly correlated with the summating potential to action potential ratio and slow phase velocity of the affected ear. A significant linear correlation was observed between the vitamin D level and contralateral ear slow phase velocity and low-frequency-pure tone audiometry value (r = 0.236 and -0.323, respectively). High-frequency-pure tone audiometry and vitamin D levels exhibited correlations with cochlear hydrops (U = 15.06, p = 0.001) and vestibular hydrops (F = 4.394, p = 0.016), respectively. CONCLUSIONS: Vitamin D is directly associated with contralateral slow phase velocity and low-frequency-pure tone audiometry, suggesting its correlation with episodic vertigo and fluctuating hearing loss. Early vitamin D supplementation may improve fluctuating hearing loss and vertigo in Meniere's disease patients with endolymphatic hydrops.