Susac's syndrome: a rare cause of fluctuating sensorineural hearing loss.
ABSTRACT Susac's syndrome is extremely rare and is characterized by the clinical triad of encephalopathy, retinal artery occlusion and deafness. A case of this rare syndrome is presented and for the first time the fluctuating nature of the hearing loss is demonstrated. The site and nature of the hearing loss has been investigated. The value of neural hearing loss as a method of monitoring the clinical severity is discussed.
Article: Disorders of cochlear blood flow.[Show abstract] [Hide abstract]
ABSTRACT: The cochlea is principally supplied from the inner ear artery (labyrinthine artery), which is usually a branch of the anterior inferior cerebellar artery. Cochlear blood flow is a function of cochlear perfusion pressure, which is calculated as the difference between mean arterial blood pressure and inner ear fluid pressure. Many otologic disorders such as noise-induced hearing loss, endolymphatic hydrops and presbycusis are suspected of being related to alterations in cochlear blood flow. However, the human cochlea is not easily accessible for investigation because this delicate sensory organ is hidden deep in the temporal bone. In patients with sensorineural hearing loss, magnetic resonance imaging, laser-Doppler flowmetry and ultrasonography have been used to investigate the status of cochlear blood flow. There have been many reports of hearing loss that were considered to be caused by blood flow disturbance in the cochlea. However, direct evidence of blood flow disturbance in the cochlea is still lacking in most of the cases.Brain Research Reviews 10/2003; 43(1):17-28. · 7.82 Impact Factor
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ABSTRACT: Susac syndrome is characterized by the triad of branch retinal arterial occlusions, encephalopathy and cochlear microangiopathy. The underlying process is believed to be a small vessel vasculitis causing microinfarcts in the retina, brain and cochlea. Analysis of two male and two female cases of Susac syndrome recognized in Australia. In this series the epidemiology, mode of presentation, ophthalmologic features, neurologic and cochleo-vestibular features, radiologic characteristics, cerebrospinal fluid findings, therapeutic interventions, clinical course and outcome of Susac syndrome is examined. Key ophthalmologic differential diagnoses include systemic lupus erythematosis (SLE), Behçet's syndrome and other vasculitides such as sarcoidosis, tuberculosis, syphilis and lymphoma. Neuro-otologic features are most frequently misdiagnosed as multiple sclerosis. Susac syndrome, first described in 1979, is becoming an increasingly recognized condition. Early recognition of the syndrome is important because treatment with systemic immunosuppression may minimize permanent cognitive, audiologic and visual sequelae.Clinical and Experimental Ophthalmology 11/2000; 28(5):373-81. · 1.96 Impact Factor
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ABSTRACT: Patients with hearing loss depend more on their vision on their communication and vice versa. ENT physician faces difficult situation when he or she consults a patient complaining of hearing loss with a problem of vision. The prevalence of ophthalmic abnormalities in deaf population is also higher compared to their hearing peers. This paper is to summarize the common syndromes associated with visual defect according to the site of affection in the eye. Study design: Review article. Syndromes which result in deaf blind person are different among adults compared to among children. Rubella syndrome and Down syndrome are the main causes of deaf blind people above 18 years of age. While in children, CHARGE syndrome is the main cause of deaf blind children. ENT physician should be aware of these syndromes for earlier detection of deaf blind persons. Deaf blind people need a great deal of help. Communication and education are crucial for them and need to be properly supported.Archives of Oto-Rhino-Laryngology 04/2013; · 1.29 Impact Factor