AbstractSemicircular canal dehiscence remains one of the rare and least touched chronic vestibular diseases worldwide. Even though microbial, congenital, and genetically determined, as well as mixed characteristics are known to be involved in the pathogenesis of superior semicircular canal dehiscence syndrome (SSCDS), many facets of the pathogenesis of semicircular canal dehiscence syndrome still need to be simplified. Management plan related to pathogenesis has not yet been established. The primary objective of this review is to present and evaluate the etiopathogenesis behind SSCDS. This study is a systematic narrative review. A PubMed search (1970–2022) was performed for studies on epidemiology and pathogenesis of SSCDS. All included articles were categorized according to level of evidence. Five hundred and sixty papers were identified, of which 25 were found to be relevant for this review. SSCDS is a multifactorial disease. There is still no consensus as to what the specific etiology is behind the syndrome. No convincing evidence is available for most associated factors and pathogenesis. Important objectives in research of SSCDS should be achieving consensus about the definition of SSCDS and gaining more in-depth knowledge of the pathogenesis of SSCDS, especially the role of congenital and acquired causes. There is still a need for further well-designed studies on the various etiopathogenesis and management of SSCDS.
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