Bilateral Superior Semicircular Canal Dehiscence Concurrent With Ehlers-Danlos Syndrome: A Case Report

Abstract

Superior semicircular canal dehiscence (SSCD) is characterized by temporal bone thinning, which creates an opening between the inner ear and middle cranial fossa. Ehlers-Danlos syndrome, hypermobility type (EDS-HT) is a genetic collagen synthesis disorder, often resulting in bony abnormalities. We present the case of a 39-year-old female with EDS-HT who exhibited the otological symptoms characteristic of bilateral SSCD. High-resolution computed tomography (CT) scans confirmed the diagnosis. The patient elected for middle fossa craniotomy and noted symptomatic improvement. Due to its potential to confer bone fragility, EDS-HT may predispose SSCD development. Further examination of the relationship between these disorders is necessary.