[Clinical manifestations of 20 cases of the superior semicircular canal dehiscence syndrome and the intervention strategies].

OBJECTIVE: The objective of this study is to investigate the clinical manifestations of the superior semicircular canal dehiscence syndrome(SSCDS) and the treatment strategies.

METHODS: Data from 20 cases diagnosed with SSCDS from September 2004 to December 2014 were retrospectively analyzed in this study. The clinical presentations including symptoms, signs, audiological and vestibular function examination, and their imaging characteristics and treatment strategies were reviewed.

RESULTS: All of the patients demonstrated variable degree of vertigo. Four patients could not tolerate the environmental noise. Autophony was noted in nine cases, among whom, two patients could feel their eye movements and heart beat, one patient could feel his footstep, one patient couldn't endure the singing by himself. Slow component vertical tortional eye movement away from the effected eye were observed in twelve patients when loud noise was given or middle ear or intracranial pressure increased. Head movement was induced by loud noise in one case. Ten patients presented with low frequency hearing loss. One case was concomitance with chronic otitis media and demonstrated severe sensorineural hearing loss. Nine patients demonstrated normal hearing. Decreased thresholds were showed by VEMP examination in six cases. Variable bone defect overlying in the SSCDS was confirmed by CT scans in all of the cases. The surgical repair of the superior semicircular canal dehiscence was performed through the middle cranial fossa approach in three cases and mastoid approach in two cases. The dizziness and the autophony were significantly alleviated after surgery.

CONCLUSIONS: The clinical manifestations of SSCDS mainly demonstrate dizziness, autophony and loss of low frequency hearing. Treatment strategies are mainly composed of avoidance of environmental noise and shouting voice of the patients themselves. Surgical repair of the superior semicircular canal dehiscence was proposed to conduct, either through the middle cranial fossa approach or mastoid approach, when the patient couldn't tolerate the sypmtoms.