Journal of Otolaryngology and Ophthalmology of Shandong University ›› 2020, Vol. 34 ›› Issue (5): 89-96.doi: 10.6040/j.issn.1673-3770.1.2020.080

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Superior semicircular canal dehiscence syndrome

  

  • Received:2020-08-10 Published:2020-11-17

Abstract: Superior semicircular canal dehiscence(SSCD)syndrome occurs as a result of a bony defect of the skull base involving the superior semicircular canal, particularly at the arcuate eminence. The bony labyrinthine defect creates a direct communication between the dura and the labyrinthine membranous structure and acts as a mobile third window which may result in various auditory and vestibular manifestations. Tinnitus and autophony are the most common audiological manifestations. Dizziness and disequilibrium are the most common vestibular manifestations. Audiometric findings vary based on the severity of the disease. Low-frequency conductive hearing loss is a common finding. Bone conduction thresholds may be negative. A patient with SSCD will typically have a lower Vestibular Evoked Myogenic Potentials(VEMP)threshold response in the affected ear and may also have a larger than normal VEMP amplitude. High-resolution computed tomography(CT)scan of temporal bone plays an important role in confirming the diagnosis of SSCD. Pöschl and Stenver reformatted views are often recommended. Surgical treatment is reserved for patients presenting with debilitating vestibular and auditory manifestations that substantially interfere with their quality of life. There are two main surgical approaches(middle fossa, trans-mastoid)and several techniques(plugging, capping, resurfacing and combination). Presently, there is insufficient evidence to clearly determine which surgical approach or technique is superior. Surgical repair of SSCD through either the middle cranial fossa approach or trans-mastoid approach is highly effective for auditory and vestibular symptom improvement and is associated with a low risk of complications.

Key words: Superior semicircular canal dehiscence, Clinical features, Diagnosis and treatment

CLC Number: 

  • R764
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