Search results for "Inner ear"
showing 10 items of 58 documents
Evaluation of Planar-Cell-Polarity Phenotypes in Ciliopathy Mouse Mutant Cochlea
2016
In recent years, primary cilia have emerged as key regulators in development and disease by influencing numerous signaling pathways. One of the earliest signaling pathways shown to be associated with ciliary function was the non-canonical Wnt signaling pathway, also referred to as planar cell polarity (PCP) signaling. One of the best places in which to study the effects of planar cell polarity (PCP) signaling during vertebrate development is the mammalian cochlea. PCP signaling disruption in the mouse cochlea disrupts cochlear outgrowth, cellular patterning and hair cell orientation, all of which are affected by cilia dysfunction. The goal of this protocol is to describe the analysis of PCP…
Thalamic infarctions cause side-specific suppression of vestibular cortex activations.
2005
H2O15-PET was performed during caloric vestibular stimulation of the right and left external ears in eight right-handed patients with acute unilateral infarctions or haemorrhages of the posterolateral thalamus (four right, four left). The posterolateral thalamus is the relay station for ipsi- and contralateral ascending vestibular input to the multiple multisensory vestibular cortex areas. The aim of this study was to evaluate the differential effects of unilateral vestibular thalamic lesions on thalamo-cortical projections, right hemispheric dominance and reciprocal inhibitory visual-vestibular interaction, as well as perceptual and ocular motor consequences during caloric irrigation. The …
Virtual endoscopy of the inner ear and the auditory canal.
2000
To assess the role of virtual endoscopy (VE) in the examination of intracisternal structures and of the inner ear, we studied the anatomy of the labyrinth and internal auditory canal using the original CT slices and VE on the unaffected side in three female and three male patients, age range 3–46 years, with contralateral retrocochlear hearing loss. We also examined seven patients with different pathological findings. VE was performed using an advanced postprocessing program with high- resolution 3D data sets of CT (1–1.5 mm thickness, pitch 1.25) and MRI-CISS-3D (constructive interference in steady state) images of the basal cisterns (1.5 T, slice thickness 0.7–1 mm). VE provides an endosc…
Localization of Congenital Tegmen Tympani Defects
2007
Objective: This study sets out to demonstrate the normal developmental steps of the tegmen tympani and thus explains the typical localization of congenital tegmental defects. Specimens: For this study, 79 macerated and formalin-fixed human temporal bones from 14th fetal week to adults were observed and prepared. Intervention: Macroscopic and microscopic examination of the prenatal and postnatal changes of the tegmen tympani during its development. Main Outcome Measure: Temporal bones from 14th fetal week to adults underwent descriptive anatomic studies to understand the normal development of the tegmen tympani and to find a possible cause of its congenital defects. Results: The medial part …
Management of intralabyrinthine schwannomas
2006
Abstract Objectives Our protocol to manage the intralabyrinthine schwannoma (ILS). Methods Retrospective chart review of 7 consecutive patients managed for ILS. Results Five patients underwent surgical removal of the lesions and none experienced significant complications or recurrent disease. One patient refused surgical treatment and was closely followed by serial MRI scans with no signs of tumor growth. One patient is presently managed conservatively due to a good hearing. Conclusions Diagnosis of ILS is based on high resolution MRI scans and should be included in the differential diagnosis of patients investigated for cochleovestibular symptoms. Treatment modality of ILS is controversial…
Immunosuppressive treatment in bilateral vestibulopathy with inner ear antibodies.
2005
Although vestibular recovery was observed after steroid treatment, it remains uncertain whether this improvement was spontaneous or due to medication. These data do not allow us to generally recommend corticosteroid treatment in patients with BVF and inner ear antibodies.A retrospective study was performed based on the observation of two patients with suspected autoimmune bilateral vestibular failure (BVF) with normal hearing and antilabyrinthine or nervous tissue-specific serum antibodies who showed vestibular recovery after corticosteroid treatment.Twelve patients with BVF and serum inner ear antibodies who had received imuunosuppressive treatment with corticosteroids were evaluated in te…
Otosclerosis associated with type B-1 inner ear malformation
2010
Malformations of bony inner ear are rare anomalies occurring in approximately 20% of patients with congenital sensorineural hearing loss. Conductive hearing loss is usually associated with abnormalities of the external and middle ear. Recent reports of patients with lateral semicircular canal malformations indicate inner ear malformations to be associated with sensorineural or conductive hearing loss. Differential diagnosis of conductive hearing loss should include otosclerosis, isolated ossicular deformities, inner ear anomalies or a combination of these. In this report, a case is described with right vestibule-lateral semicircular canal dysplasia presenting at our centre with bilateral ot…
Use of electron spectroscopic imaging to determine element composition of the melanin granules in the stria vascularis of the guinea pig.
1998
Electron spectroscopic imaging (ESI) was used to analyze the element content of melanin granules in the stria vascularis seen in ultrathin sections of Spurr-embedded cochleae of the guinea pig. To determine element composition, ESI images were taken at different ionization edges, and non-specific background signals were subtracted digitally by an image processing system. The presence of calcium and nitrogen in the melanin granules could be demonstrated clearly. The calcium identified in the melanin granules was then compared with the spatial distributions of calcium binding sites after the application of an antimonate precipitation method, which was used to localize loosely bound calcium. D…
Autoimmune ear disease: clinical and diagnostic relevance in Cogan’s sydrome
2017
The autoimmune inner ear disease is a clinical syndrome with uncertain pathogenesis that is often associated to rapidly progressive hearing loss that, especially at the early stages of disease, may be at monoaural localization, although more often it is at binaural localization. It usually occurs as a sudden deafness, or a rapidly progressive sensorineural hearing loss. In this study a particular form of autoimmune inner ear disease is described, Cogan’s syndrome. Cogan’s syndrome is a chronic inflammatory disorder that most commonly affects young adults. Clinical hallmarks are interstitial keratitis, vestibular and auditory dysfunction. Associations between Cogan’s syndrome and systemic va…
Sudden sensorineural hearing loss as prodromal symptom of anterior inferior cerebellar artery infarction.
2011
Sudden sensorineural hearing loss is a clinical condition characterized by a sudden onset of unilateral or bilateral hearing loss. In recent years sudden deafness has been frequently described in association with anterior inferior cerebellar artery (AICA) infarction generally presenting along with other brainstem and cerebellar signs such as ataxia, dysmetria and peripheral facial palsy. The authors report a rare clinical case of a 53-year-old man who suddenly developed hearing loss and tinnitus without any brainstem or cerebellar signs. Computed tomography of his brain was normal, and the audiological results localized the lesion causing deafness to the inner ear. Surprisingly, magnetic re…