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Superior Semi-Circular Canal Dehiscence Syndrome

 

SSCDS is a recently identified disorder affecting the bone of the skull base and the enclosed inner ear structures. For unknown reasons, bone of the skull base slowly dissolves exposing the superior balance canal. This allows sound to “escape” from the inner ear resulting in hearing loss. With loud noises, an extreme and sudden sensation of movement (called vertigo) may result for the patient. Active research at CEI is defining the effects and remedies available to patients with this disorder.

 

WHAT IS SUPERIOR SEMI-CIRCULAR CANAL DEHISCENCE SYMDROME?

Superior Semi-circular Canal Dehiscence Syndrome (SSCD) is a very rare medical condition where a thinning or complete absence of a portion of the temporal bone overlying the superior semicircular canal of the inner ear causes hypersensitivity to sound and balance disorders. One unique characteristic of SSCD is that audiologically, the hearing loss appears to be conductive, though it is actually sensorineural. Computed Tomographic scanning (CT) and Magnetic Resonance Imaging (MRI) may be used to diagnose SSCD, and distinguish it from more common diseases with similar symptoms such as Meniere’s disease.

WHAT ARE THE SYMPTOMS OF SUPERIOR SEMI-CIRCULAR CANAL DEHISCENCE SYNDROME?

Symptoms of SSCD include dizziness and balance problems which increases with activity and which is relieved by rest. A cardinal symptom of this disorder is vertigo produced with a loud sound. Other symptoms may include hearing loss, tinnitus and a fullness of the ear. The symptoms of SSCD can get worse with extended episodes of coughing, sneezing, or blowing of the nose. Sometimes the use of ones own voice or a musical instrument can also aggravate SSCD.

WHAT IS THE TREATMENT FOR SUPERIOR SEMI-CIRCULAR CANAL DEHISCENCE SYNDROME?

Treatment for SSCD can only be determined after a comprehensive medical examination of an otologist and a full audiological workup. A specialized CT scan of the ear is needed to accurately diagnose the condition. The California Ear Institute has such a scanner in its offices in order to allow demonstration of this rare condition in affected patients. Once the condition has been diagnosed, surgery may be an option in order to repair the defect in the superior semi-circular canal. Otologists at the California Ear Institute are perfecting a less invasive means of repairing the defect compared to standard craniotomy procedures which have previously required brain retraction and entrance into the cranial cavity.

CONCLUSION:

Balance disorders and hearing losses, including those caused by SSCD require immediate comprehensive medical examination and specialty care from an Otologist, who is an otolaryngologist (ENT) who has undergone two additional years of training and specializes in issues related hearing only. With appropriate medical treatment and therapy, individuals with SSCD can make a partial or even full recovery.

Click here to make an appointment with the California Ear Institute to consult with one of our board certified otologists regarding your condition.

Watch a related video on SSCD Vein surgery.

 
California Ear Institute