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

Ansley Unterberger; Jessa Miller; Quinton Gopen; Isaac Yang

doi:10.7759/cureus.19943

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

Cureus. 2021 Nov 27;13(11):e19943. doi: 10.7759/cureus.19943. eCollection 2021 Nov.

Authors

Ansley Unterberger¹, Jessa Miller², Quinton Gopen³, Isaac Yang¹

Affiliations

¹ Neurosurgery, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, USA.
² Otolaryngology - Head and Neck Surgery, University of California, Los Angeles, Los Angeles, USA.
³ Otolaryngology - Head and Neck Surgery, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, USA.

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.

Keywords: case series; ehlers-danlos syndrome; hypermobility; middle fossa craniotomy; superior semicircular canal dehiscence.

Publication types

Case Reports

Grants and funding

Isaac Yang is supported by the UCLA Visionary Ball Fund Grant, Eli and Edythe Broad UCLA Center of Regenerative Medicine and Stem Cell Research Scholars in Translational Medicine Program Award, Jason Dessel Memorial Seed Grant, UCLA Honberger Endowment Brain Tumor Research Seed Grant, and Stop Cancer (US) Research Career Development Award.