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Abstract：
Susac Syndrome (SS) is a rare unique autoimmune underdiagnosed disorder characterized by the clinical triad of central nervous system (CNS) dysfunction, sensorineural hearing impairment, and branch retinal artery occlusion (BRAO). Objectives The purpose of reporting the case is to succinctly highlight this enigmatic condition with focus on otological and audiological aspect of SS and also to augment awareness of this disease among otolaryngologists neurologists, psychiatrists and ophthalmologists. Additionally we reviewed the otological menifestations and audiological findings related to SS. Methods A case of 38 year old female is presented with bilateral SNHL as the initial symptom of SS. The review of otological and audiological findings of previous 10 year case reports was executed using the PRISMA protocol on 20 cases. Results It was seen that inspite of the ear being one of the main organs involved in the SS very few had undergone detailed audiological assessment. Our review of literature showed that only 40% cases had done audiological assessment other than PTA, out of which only 5% had undergone complete audiological workup. 30% of the patients were having auditory symptoms as presenting chief complaint. Conclusion Otolyryngologists should keep SS in differentials when a case of SNHL of unknown etiology is encountered.We emphasize the use newer noninvasive Powered by Editorial Manager® and ProduXion Manager® from Aries Systems Corporation diagnostic tools like Optical coherence tomography (OCT), MRI and detailed audiological tests for timely diagnosis of SS. Further multidisciplinary assessment protocols should be used to prevent the development of severe sequelae.
摘要：
Susac综合征（SS）是一种罕见的独特的自身免疫性未诊断疾病，其特征是中枢神经系统（CNS）功能障碍的临床三联征，感觉神经性听力障碍,和视网膜分支动脉阻塞（BRAO）。目的报告病例的目的是简洁地强调这种神秘的状况，重点是SS的耳科和听力学方面，并提高耳鼻喉科神经科医师对这种疾病的认识。精神病医生和眼科医生。此外，我们还回顾了与SS相关的耳科月经和听力学发现。方法1例38岁女性患者以双侧SNHL为首发症状。使用PRISMA方案对20例病例进行了对以前10年病例报告的耳科和听力学发现的审查。结果可以看出，尽管耳朵是SS涉及的主要器官之一，但很少有经过详细的听力学评估。我们对文献的回顾表明，只有40％的病例进行了除PTA以外的听力学评估，其中只有5%接受了完整的听力学检查。30％的患者有听觉症状，表现为主诉。结论当遇到病因不明的SNHL病例时，耳鼻喉科医师应保持SS的差异。我们强调使用来自AriesSystemsCorporation的新型非侵入式诊断工具，如光学相干断层扫描（OCT），MRI和详细的听力学测试可及时诊断SS。应使用进一步的多学科评估方案来防止严重后遗症的发展。
