Dataset Information

Cochlin expression in vestibular endorgans obtained from patients with Meniere's disease.

ABSTRACT: The distribution of cochlin and its associated basement membrane proteins (collagen IV, collagen II, laminin-β2, and nidogen-1) were evaluated in the vestibular endorgans of subjects with Meniere's disease and compared with normal specimens. Cochlin mRNA expression in vestibular endorgans from Meniere's disease specimens was also investigated. Specimens were obtained from patients who had Meniere's disease and who were undergoing ablative labyrinthectomy. Control specimens were obtained both from autopsy specimens with documented normal audiovestibular function and from patients undergoing labyrinthectomy for acoustic neuroma excision. In the normal control specimens, cochlin immunoreactivity was found evenly distributed in the stroma of the cristae ampullaris and maculae of the utricle. In Meniere's specimens, cochlin immunoreactivity was markedly increased; this was associated with an increase in cochlin mRNA expression as shown by real-time reverse transcription with the polymerase chain reaction. Collagen IV and laminin-β2 immunoreactivity was significantly decreased in Meniere's specimens. Nidogen-1 and collagen II immunoreactivity was unchanged in Meniere's specimens when compared with normal samples. Cochlin upregulation has been implicated in the hereditary audiovestibulopathy, DFNA9. The increased expression of cochlin and decreased expression of collagen IV and laminin in Meniere's disease are suggestive that the overexpression of cochlin contributes to the dysfunctional inner ear homeostasis seen in this disease.

SUBMITTER: Calzada AP

PROVIDER: S-EPMC4420027 | biostudies-literature | 2012 Nov

REPOSITORIES: biostudies-literature

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Cochlin expression in vestibular endorgans obtained from patients with Meniere's disease.

Calzada Audrey P AP Lopez Ivan A IA Beltran Parrazal Luis L Ishiyama Akira A Ishiyama Gail G

Cell and tissue research 20120920 2

The distribution of cochlin and its associated basement membrane proteins (collagen IV, collagen II, laminin-β2, and nidogen-1) were evaluated in the vestibular endorgans of subjects with Meniere's disease and compared with normal specimens. Cochlin mRNA expression in vestibular endorgans from Meniere's disease specimens was also investigated. Specimens were obtained from patients who had Meniere's disease and who were undergoing ablative labyrinthectomy. Control specimens were obtained both fro ...[more]

PMID: 22992960

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Project description:Objective: Intravenous contrast agent enhanced, high-resolution magnetic resonance imaging of the inner ear (iMRI) confirmed that patients with Menière's disease (MD) and vestibular migraine (VM) could present with endolymphatic hydrops (EH). The present study aimed to investigate EH characteristics and their interrelation to neurotologic testing in patients with VM, MD, or VM with concurrent MD (VM-MD). Methods: Sixty-two patients (45 females, aged 23-81 years) with definite or probable VM (n = 25, 19 definite), MD (n = 29, 17 definite), or showing characteristics of both diseases (n = 8) were included in this study. Diagnostic workup included neurotologic assessments including video-oculography (VOG) during caloric stimulation and head-impulse test (HIT), ocular and cervical vestibular evoked myogenic potentials (o/cVEMP), pure tone audiometry (PTA), as well as iMRI. EH's degree was assessed visually and via volumetric quantification using a probabilistic atlas-based segmentation of the bony labyrinth and volumetric local thresholding (VOLT). Results: Although a relevant number of VM patients reported varying auditory symptoms (13 of 25, 52.0%), EH in VM was only observed twice. In contrast, EH in VM-MD was prevalent (2/8, 25%) and in MD frequent [23/29, 79.3%; χ2(2) = 29.1, p < 0.001, φ = 0.7]. Location and laterality of EH and neurophysiological testing classifications were highly associated (Fisher exact test, p < 0.005). In MD, visual semi-quantitative grading and volumetric quantification correlated highly to each other (r S = 0.8, p < 0.005, two-sided) and to side differences in VOG during caloric irrigation (vestibular EH ipsilateral: r S = 0.6, p < 0.05, two-sided). In VM, correlations were less pronounced. VM-MD assumed an intermediate position between VM and MD. Conclusion: Cochlear and vestibular hydrops can occur in MD and VM patients with auditory symptoms; this suggests inner ear damage irrespective of the diagnosis of MD or VM. The EH grades often correlated with auditory symptoms such as hearing impairment and tinnitus. Further research is required to uncover whether migraine is one causative factor of EH or whether EH in VM patients with auditory symptoms suggests an additional pathology due to MD.

Project description:Despite the huge progress in the definition and classification of vestibular disorders within the last decade, there are still patients whose recurrent vestibular symptoms cannot be attributed to any of the recognized episodic vestibular syndromes, such as Menière's disease (MD), vestibular migraine (VM), benign paroxysmal positional vertigo (BPPV), vestibular paroxysmia, orthostatic vertigo or transient ischemic attack (TIA). The aim of the present international, multi-center, cross-sectional study was to systematically characterize the clinical picture of recurrent vestibular symptoms not otherwise specified (RVS-NOS) and to compare it to MD and VM. Thirty-five patients with RVS-NOS, 150 patients with VM or probable VM and 119 patients with MD were included in the study. The symptoms of RVS-NOS had been present for 5.4 years on average before inclusion, similar to VM and MD in this study, suggesting that RVS-NOS is not a transitory state before converting into another diagnosis. Overall, the profile of RVS-NOS vestibular symptoms was more similar to VM than MD. In particular, the spectrum of vestibular symptom types was larger in VM and RVS-NOS than in MD, both at group comparison and the individual level. However, in contrast to VM, no female preponderance was observed for RVS-NOS. Positional, head-motion and orthostatic vertigo were reported more frequently by patients with RVS-NOS than MD, while external vertigo was more prevalent in the MD group. At group level, the spectrum of attack durations from minutes to 3 days was evenly distributed for VM, while a small peak for short and long attacks in RVS-NOS and a big single peak of hours in MD were discernible. In general, vertigo attacks and associated vegetative symptoms (nausea and vomiting) were milder in RVS-NOS than in the other two disorders. Some patients with RVS-NOS described accompanying auditory symptoms (tinnitus: 2.9%, aural fullness and hearing loss: 5.7% each), migrainous symptoms (photophobia, phonophobia or visual aura in 5.7% each) or non-migrainous headaches (14%), but did not fulfill the diagnostic criteria for MD or VM. Absence of a life time diagnosis of migraine headache and attack duration of <5 min were further reasons not to qualify for VM. In some RVS-NOS patients with accompanying ear symptoms, attack durations of <20 min excluded them from being diagnosed with MD. These findings suggest that RVS-NOS is a stable diagnosis over time whose overall clinical presentation is more similar to VM than to MD. It is more likely to be composed of several disorders including a spectrum of mild or incomplete variants of known vestibular disorders, such as VM and MD, rather than a single disease entity with distinct pathognomonic features.

Dataset Information

Cochlin expression in vestibular endorgans obtained from patients with Meniere's disease.

Publications

Cochlin expression in vestibular endorgans obtained from patients with Meniere's disease.

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