Susac's syndrome: A rare cause of fluctuating sensorineural hearing loss
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.
[Show abstract] [Hide abstract] 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.0Comments 1Citation
- "Loss of hearing in an audiometric test is usually observed within low and middle frequencies. Vestibular dysfunction is evaluated with a caloric test . Susac syndrome is a rare disease characterized by microangiopathy involving the arterioles of the brain, retina and cochlea. "
- [Show abstract] [Hide abstract] ABSTRACT: Susac syndrome is a rare vasculopathy affecting small vessels of the brain, the retina and the cochlea. The pathogenesis of this syndrome is unknown. It is characterized by a triad of clinical symptoms, i.e. encephalopathy, visual disorders and hearing disorders. The aim of this work was to review available data about the clinical course, diagnosis and treatment of Susac syndrome.0Comments 3Citations
- [Show abstract] [Hide abstract] 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.0Comments 29Citations