耳鼻咽喉科: オープンアクセス

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Case Study: An Uncommon Case of Bilateral Superior Semicircular Canal Dehiscence Associated with Sudden Sensoryneural Hearing Loss

Gabriela Cornelia Musat, Alina Georgiana Anghel and Ovidiu Musat

Superior Canal Dehiscence (SCD) is a rare condition described for the first time by Minor in 1998. Bilateral superior canal dehiscence is even less frequent (0.6% of all SCD).

We present the case of a patient with bilateral superior canal dehiscence associated with sudden sensorineural hearing loss on one ear. The diagnosis of SCD was based on the symptoms of the patient (intolerance to loud noises, disequilibrium, hearing loss), based on the CT scan that revealed the bilateral dehiscence and based on the audiogram and the VEMP (Vestibular Evoked Myogenic Potential) testing with low cVEMP threshold bilaterally. All the data gathered were consistent with the diagnosis we made.

The particularity of the case consists in the fact that although the patient diagnosis was made according to all diagnostic criteria available and the CT scan and VEMP testing showed bilateral dehiscence, the symptoms were installed acutely and were evident only for one ear. In our opinion this can be explained by the fact that the patient had a more extended dehiscence in the left ear and also suffered a concomitant Sudden Sensorineural Hearing Loss (SSHL) that required additional testing making possible the correct diagnosis of the condition.