1/14. Atrophy of the left lobe of the liver and anomalous hepatic vessel in a patient with Yunis-Varon syndrome.Yunis-Varon syndrome is inherited as an autosomal recessive trait. It is characterized by facial and digit anomalies. This report describes a young woman with clinical features of this syndrome, atrophy of the left lobe of the liver, and a vascular anomaly. liver abnormalities have not been described as features of this rare syndrome.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/14. Mitochondrial maculopathy: geographic atrophy of the macula in the MELAS associated A to G 3243 mitochondrial dna point mutation.PURPOSE: To report ocular findings in the mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (melas syndrome) in a family with the A to G 3243 mitochondrial (mt) dna point mutation. methods: case reports. Ocular findings are described from four family members with the MELAS associated A to G 3243 mt dna point mutation. RESULTS: Findings included ophthalmoplegia, neurosensory deafness, reduction of photopic and scotopic electroretinogram b-wave amplitudes, and myopathy, as well as macular retinal pigment epithelial atrophy. No family members had nyctalopia, attenuation of retinal blood vessels, or retinal bone spicule pigmentation. CONCLUSION: The finding of slowly progressive macular retinal pigment epithelial atrophy expands the reported phenotypic diversity of patients with A3243G mt dna mutations.- - - - - - - - - - ranking = 0.25keywords = vessel (Clic here for more details about this article) |
3/14. Sudden bilateral deafness from hyperleukocytosis in chronic myeloid leukemia.Sudden-onset bilateral deafness as a clinical manifestation of hyperleukocytosis in chronic myeloid leukemia (CML) is a rare occurrence. We found only 27 clinical descriptions in 16 published papers. In this work, the authors present a review on deafness in CML and describe a new case with prominent hyperleukocytosis, where the neurological findings suggest slowing of the circulation through small blood vessels in the brainstem as the cause of deafness. The evolution was good after treatment. To our knowledge, this is the second case documented with electrical auditory brainstem-evoked potentials and the first with magnetic resonance imaging.- - - - - - - - - - ranking = 0.25keywords = vessel (Clic here for more details about this article) |
4/14. Pathologic features of the inner ear in congenital deafness.We present the morphologic findings of the temporal bones and brain of a patient with congenital deafness. We discuss these findings in relation to pathologic observations in other reported cases of congenital deafness. Morphologic abnormalities in the patient were mainly in the pars inferior of the membranous labyrinths. The osseous labyrinths were well developed. There was severe dilation of the cochlear duct with herniation of the Reissner membrane, extensive atrophy of the stria vascularis that was associated with calcified thrombi to the strial vessels, encasement of the tectorial membrane in a syncytium, and dyspiastic or regressive degeneration of the organ of corti. Absence of spiral ganglion cells and their fibers was a prominent feature. The extensive and varied pathologic changes that were present in our patient simultaneously suggest a congenital abnormality in endolymph production and raise the possibility of anomalous development of the labyrinthine vasculature.- - - - - - - - - - ranking = 0.25keywords = vessel (Clic here for more details about this article) |
5/14. cochlear implantation in a child with osteogenesis imperfecta.osteogenesis imperfecta (OI) is a hereditary disease of connective tissue and affects bone, dentine, sclera, joint, tendon, blood vessels, heart valves, and skin. Approximately 50% of the adult patients with OI have associated hearing impairment. To date, only three cases of cochlear implantation in adults with OI have been reported, but none in children. We present a case of cochlear implantation in a congenitally deaf 6-year-old boy with OI. The Nucleus 24 Contour device was successfully implanted using the suprameatal approach (SMA). At 6 months post-initial stimulation there was no evidence of non-acoustic nerve excitation (i.e. facial twitching) or discomfort, and significant progress in auditory abilities was manifested by open set word identification.- - - - - - - - - - ranking = 0.25keywords = vessel (Clic here for more details about this article) |
6/14. Cogan's syndrome with aortitis, aortic regurgitation, and aortic arch vessel stenoses.Cogan's syndrome of interstitial keratitis and vestibuloauditory dysfunction is rare. Systemic vasculitic manifestations occur, and 10% of patients with this syndrome have aortic valvular disease. A patient with Cogan's syndrome is presented who had aortitis of the ascending thoracic aorta, severe aortic valve regurgitation, orificial stenosis of the right coronary artery, and orificial stenoses of all three aortic arch vessels. Histopathology confirmed aortitis. aortic valve replacement combined with coronary and aortic arch vessel reconstruction was required for correction.- - - - - - - - - - ranking = 1.5keywords = vessel (Clic here for more details about this article) |
7/14. Late corneal opacities in the syndrome of interstitial keratitis and vestibulo-auditory symptoms.Progressive clouding of the cornea may be a delayed complication of the interstitial keratitis-vestibuloauditory syndrome. Two illustrative cases are presented. The first case illustrates progressive opacification about aberrant vessels in the deep stroma presenting a characteristic dendritiform pattern. The second case shows that the opacification is due, in this case at least, to formation of connective tissue with inclusion of lipid crystals and fat along with the blood vessels. Descemet's membrane is also thickened several fold.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
8/14. Encephalopathy, deafness and blindness in young women: a distinct retinocochleocerebral arteriolopathy?Three young women (aged 18 years, 19 years and 19 years) who developed progressive neuropsychic and neurologic disturbances with hearing loss and multifocal retinal artery branch occlusions are reported. This retinocochleocerebral syndrome has been reported previously only in 12 young North American women. Its pathogenesis is unknown, but an atypical viral infection of the vessel walls has been suggested. Abnormalities of T lymphocytes subsets in blood in one of the patients suggested an immunological dysfunction, but all other tests, including immunological reactions on brain and skin biopsies, were negative or non-specific. steroids and immunosuppressive agents have been advocated on an empirical basis, but the second patient showed a substantial recovery without any therapy and the third gradually deteriorated despite azathioprine, cyclophosphamide, prednisone and plasma exchanges. This retinocochleocerebral syndrome probably corresponds to an arteriolopathy of unknown nature.- - - - - - - - - - ranking = 0.25keywords = vessel (Clic here for more details about this article) |
9/14. Fluctuating hearing losses in children can be migraine equivalents.Fluctuation of hearing thresholds in an already severely to profoundly deaf child constitutes a stressing condition and a therapeutic challenge. Thorough medical inquiries revealed strong histories of migraine headaches in the parents of 13 severely deaf children (mean age: 7 years) and two of them also presented symptoms of migraine. This disease is viewed as a form of a relatively benign cerebral vasospasm causing an intense transitory vasodilatation of the small vessels of the brain and a subsequent sterile inflammatory reaction. Liberation of histamine, serotonin and plasma kinins appear to interfere with the metabolism of nerve cells. All children in our study had suffered from anoxia at birth, a condition related to a depopulation of cochlear brainstem nuclei. Migraines may therefore produce obvious hearing symptoms when vasomotor disturbances occur in already damaged nervous structures. Treatment with propranolol hydrochloride (HCl), a potent beta-blocker, resulted in cessation of hearing fluctuations in all patients and in an improvement of thresholds in two of them. We presented our results, as well as preliminary studies on asphyxiated rats shortly after birth, with transitory artificially induced cerebral vasodilatation.- - - - - - - - - - ranking = 0.25keywords = vessel (Clic here for more details about this article) |
10/14. retinal telangiectasis in facioscapulohumeral muscular dystrophy with deafness.A 22-year-old patient with newly diagnosed facioscapulohumeral (FSH) muscular dystrophy had a macular lesion in her right eye and poor central vision, which had been present since early childhood. Fluorescein angiographic examination revealed bilateral peripheral vessel closure, peripheral retinal telangiectasis, and hyperfluorescence in both foveae. This widespread vascular abnormality was deemed responsible for her macular disease. Her mother, brother, and sister, all of whom are affected by varying degrees of FSH muscular dystrophy and clinical deafness, also have abnormal retinal vasculature, as determined by fluorescein angiography. However, none had related visual symptoms and two showed no ophthalmoscopic evidence of vascular abnormalities. In young patients with unexplained retinal vascular lesions, the diagnosis of FSH muscular dystrophy should be considered. Similarly, young patients with FSH muscular dystrophy should be examined for sight-threatening and potentially treatable vascular retinopathy.- - - - - - - - - - ranking = 0.25keywords = vessel (Clic here for more details about this article) |
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