Endolymphatic hydrops in the unaffected ear of patients with unilateral Ménière's disease.

PURPOSE: Current studies show that frequency tuning modification is a good marker for the detection of endolymphatic hydrops (EH) employing magnetic resonance imaging (MRI) in patients with Ménière's disease (MD). The purpose of the present study is to analyze the auditory and vestibular function with audiometric and vestibular-evoked myogenic potentials (VEMP) responses, respectively, in both the affected and unaffected ears of patients with unilateral MD using MRI as diagnostic support for the degree of EH. METHODS: We retrospectively reviewed the medical records of 76 consecutive patients with unilateral definite MD (age 55 (28-75); 39 women, 37 men). MRI was used through intravenous gadolinium administration, audiometry, and VEMPs. Functional tests were performed up to a week after the MRI. All were followed up one year after imaging utilizing clinical, auditory, and vestibular testing to rule out bilateral involvement. RESULTS: In the unaffected ear, the mean pure-tone average is normal even in cases with hydrops and, for a similar severity of hydrops is significantly lower than in the affected ear. Significant differences for the amplitude of the response at 0.5 kHz, at 1 kHz between the affected and unaffected ears were found to be lower in the affected ears. The relative amplitude ratio (1 Kz-0.5 kHz) was significantly lower in the affected ear and in the case of the oVEMP response depends on the degree of EH. The response in the unaffected ear was not modified by the presence or the degree of hydrops. CONCLUSION: In the unaffected ear, hydrops is not associated with hearing deterioration. For a similar degree of hydrops, hearing loss is significantly greater in the affected ear. The endolymphatic hydrops in the vestibule induces a frequency bias in the VEMP response only in the affected ear and not in the unaffected ear. Because of these findings we consider that hydrops does not represent an active disorder in the unaffected ear.

Magnetic Resonance Volumetric Quantification of Vestibular Endolymphatic Hydrops in Patients with Unilateral Definite Meniere's Disease Using 3D Inversion Recovery with Real Reconstruction (3D-REAL-IR) Sequence.

BACKGROUND: The 3D-REAL-IR MRI sequence allows for an in vivo visualization of endolymphatic hydrops. Qualitative assessment methods of the severity of vestibular and cochlear hydrops are the most commonly used. METHODS: A quantitative volumetric measurement of vestibular EH in patients with definite unilateral Ménière's disease using the 3D-REAL-IR sequence and the calculation of the endolymphatic ratio (ELR) was intended. RESULTS: Volumetric calculations of the vestibules, vestibular endolymph and vestibular ELR are performed in 96 patients with unilateral Ménière's disease and correlated with classic qualitative grading scales. CONCLUSIONS: Quantitative volumetric measurement of vestibular hydrops using the 3D-REAL-IR sequence is feasible and reproducible in daily clinical practice. Vestibular ELR values exceeding 60% defined radiologically significant vestibular hydrops, while values below 30% defined radiologically non-significant vestibular hydrops.

Congruence and incongruence on the radiological and functional examination of inner ear hemorrhage.

BACKGROUND: Inner ear hemorrhage (IEH) is an increasingly recognized cochlear lesion that can cause sensorineural hearing loss (SNHL). Magnetic resonance imaging (MRI) is known to be the best imaging modality for clarifying the causes of SNHL and providing images that point to those causes. AIMS: Evaluate the lesional patterns in patients with presumed Inner ear hemorrhage (IEH) from radiological and functional aspects. MATERIAL AND METHODS: We retrospectively reviewed 10 patients performed in our institution from 2014 to 2020, with suspected labyrinthine hemorrhage based on radiological and functional examination. RESULTS: We included 8 patients with IEH and sensorineural hearing loss (SNHL). The median age was 55 years (range: 3 months - 78 years). The results from the MRI and functional tests were compared for each end-organ. Only three cases (37.5%) showed a correlation between signal abnormalities and dysfunction in the labyrinthine apparatus. CONCLUSIONS: In patients with SNHL inner ear hemorrhage needs to be ruled out in the differential diagnosis, so specific MRI sequences should be requested. It represents a way to a better understanding of the disorder and the variety of findings claim for a complete auditory and vestibular testing.

Skull Vibration-Induced Nystagmus and High Frequency Ocular Vestibular-Evoked Myogenic Potentials in Superior Canal Dehiscence.

BACKGROUND: Although diagnostic criteria have been established for superior canal dehiscence syndrome, cases in which the diagnosis is not easy are frequent. On those occasions, some tests such as vibration-induced nystagmus or vestibular-evoked myogenic potentials can offer invaluable help due to their high sensitivity and specificity. METHODS: We studied 30 patients showing superior canal dehiscence or "near-dehiscence" in a CT scan. Skull vibration-induced nystagmus and high frequency ocular vestibular-evoked myogenic potentials are performed in each patient. The aim of the study is to determine how useful both tests are for detection of superior canal dehiscence or near-dehiscence. RESULTS: Of the 60 temporal bones studied, no dehiscence was the result in 22, near-dehiscence in 17 and a definite finding in 21. In 10/30 patients, there was no SVIN (Skull vibration induced nystagmus) during otoneurological testing, while in 6/30, induced nystagmus was mainly horizontal, and in 14/30 there was vertical up-beating. All patients had a positive oVEMP (Ocular vestibular evoked myiogenic potentials) at 0.5 kHz in both ears and the HFoVEMP (High frequency ocular vestibular evoked myiogenic potentials) response was positive in 25/60 (41.6%) of the ears studied and in 19/30 of the patients evaluated (in 6 it was positive in both ears). Up-beat SVIN will point to a SCD (Superior Canal Dehiscence) mainly when HFoVEMP are present, and when this is negative there is a high probability that it is not a SCD. CONCLUSIONS: When SVIN and HFoVEMP results are added (or combined), they not only improve the possibilities of detecting SCD, but also the affected side.

Endolymphatic Hydrops in Fluctuating Hearing Loss and Recurrent Vertigo.

Background: Endolymphatic hydrops (EH) is the histopathological hallmark of Ménière's disease (MD) and has been found by in vivo magnetic resonance imaging (MRI) in patients with several inner ear syndromes without definite MD criteria. The incidence and relevance of this finding is under debate. Purpose: The purpose of the study is to evaluate the prevalence and characteristics of EH and audiovestibular test results in groups of patients with fluctuating audiovestibular symptoms not fulfilling the actual criteria for definite MD and compare them with a similar group of patients with definite MD and a group of patients with recent idiopathic sudden neurosensory hearing loss (ISSNHL). Material and Methods: 170 patients were included, 83 with definite MD, 38 with fluctuating sensorineural hearing loss, 34 with recurrent vertigo, and 15 with ISSNHL. The clinical variables, audiovestibular tests, and EH were evaluated and compared. Logistic proportional hazard models were used to obtain the odds ratio for hydrops development, including a multivariable adjusted model for potential confounders. Results: No statistical differences between groups were found regarding disease duration, episodes, Tumarkin spells, migraine, vascular risk factors, or vestibular tests; only hearing loss showed differences. Regarding EH, we found significant differences between groups, with odds ratio (OR) for EH presence in definite MD group vs. all other patients of 11.43 (4.5-29.02; p < 0.001). If the ISSNHL group was used as reference, OR was 55.2 (11.9-253.9; p < 0.001) for the definite MD group, 9.9 (2.1-38.9; p = 0.003) for the recurrent vertigo group, and 5.1 (1.2-21.7; p = 0.03) for the group with fluctuating sensorineural hearing loss. Conclusion: The percentage of patients with EH varies between groups. It is minimal in the ISSNHL group and increases in groups with increasing fluctuating audiovestibular symptoms, with a rate of severe EH similar to the known rate of progression to definite MD in those groups, suggesting that presence of EH by MRI could be related to the risk of progression to definite MD. Thus, EH imaging in these patients is recommended.

Trapped on the "whirl": diagnostic sign on emergency CT.

The "whirl sign" is an uncommon finding on emergency CT. However, it is easy to overlook if not kept in mind. Its recognition is of capital importance, being most of its causes potentially lethal. Surgical treatment is also mandatory when signs of complication are found. The whirl sign is usually found associated to midgut, cecal and sigmoid volvulus, small-bowel volvulus and closed-loop obstructions, and post-surgical mesenteric windows (including retroanastomotic hernias). CT is an optimal imaging technique to depict the so-called sign and associated CT features suggesting complication (circumferential wall thickening, pneumatosis intestinalis, pneumoperitoneum, mesenteric fat stranding, free intraperitoneal fluid, mesenteric haziness). Radiologists must be able to recognize the whirl sign and seek associated findings that strongly support the diagnosis of a spectrum of entities, some of them lethal if no treatment is established.

Comparison between high-resolution 3D-IR with real reconstruction and 3D-flair sequences in the assessment of endolymphatic hydrops in 3 tesla.

BACKGROUND: The main sequences used to assess degree of endolymphatic hydrops (EH) are the 3D- inversion-recovery (IR) with real reconstruction and the 3D- fluid-attenuated inversion recovery (FLAIR). OBJECTIVES: The purpose of this study is to describe and compare both sequences. MATERIAL AND METHODS: Forty-two consecutive patients diagnosed with probable or definite Ménièr´s disease were referred for hydrops MR examination. Vestibular and cochlear EH in both sequences were read by two independent radiologists, unaware of the patient's clinical status. The primary study endpoint was the concordance in the hydrops detection and severity with both imaging sequences. This was assessed using the Cohen's kappa Κ statistic for disease grading and Pearson Χ2 test to test the difference in detection rates of hydrops. Statistical significance was defined as two-sided p < .05. RESULTS: We obtained an excellent overall concordance, with a kappa of 0.821, (p < .001) when comparing hydrops detection. The degree of concordance is higher in vestibular hydrops than in cochlear hydrops. The 3D-IR sequence detected more hydrops than the 3D FLAIR, (62 vs. 39.5%, p < .03). CONCLUSION: The 3D-IR sequence seems to be superior to the 3D-FLAIR for the assessment of EH. Significance: The 3D-IR sequence should be considered as a standalone parameter for a shorter and optimized EH magnetic resonance imaging protocol.

CT and MR Unilateral Brain Features Secondary to Nonketotic Hyperglycemia Presenting as Hemichorea-Hemiballism.

Hemichorea-hemiballism is an unusual hyperkinetic movement disorder characterized by continuous involuntary movements of an entire limb or both limbs on one side of the body. The acute onset of this disorder occurs with an insult in contralateral basal ganglia. Ischemic events represent the most common cause. Nonketotic hyperglycemia comes in second place. Nonketotic hyperglycemic hemichorea-hemiballism (NHH) is a rare cause of unilateral brain abnormalities on imaging studies confined to basal ganglia (mainly putaminal region as well as caudate nucleus). Subtle hyperdensity in striatal region can be found on CT studies whereas brain MR imaging typically shows T1 hyperintensity and T2 hypointensity in the basal ganglia contralateral to the movements. Diagnosis is based on both glucose levels and neuroimaging findings. Elevated blood glucose and hemoglobin A1c levels occur with poorly controlled diabetes. In this case report, our aim is to present neuroimaging CT and MR unilateral findings in an elderly woman secondary to nonketotic hyperglycemia presenting as hemichorea-hemiballism.