The remediation of hearing deterioration in children with large vestibular aqueduct syndrome

Based on imaging findings, large vestibular aqueduct syndrome (LVAS) in early childhood is the most common cause of sensorineural hearing loss. Children with LVAS are at a high risk of suffering sudden deteriorations in hearing. This study was to review treatment of sudden hearing deterioration in children with LVAS who underwent corticosteroid therapy. We conducted a retrospective study of patients presenting to an academic tertiary medical center. Sixteen children of LVAS were evaluated. Corticosteroid therapy (prednisolone 1-2 mg/(kg day) or equal titer's dexamethasone) was administered as soon as sudden hearing loss developed. The pure tone audiometric result improved more than 10 dB at two or more consecutive frequencies and was regarded as a significant response to corticosteroid therapy. Sixteen cases comprising 12 boys and 4 girls were retrospectively analyzed in this study. The mean age at which LVAS was diagnosed was 2.3 years. Mean follow-up for the 16 cases from the first clinic visit to November 2003 was 4.2 years. The initial audiograms varied from down-sloping, valve or rising patterns. In addition, bilateral enlargement of the vestibular aqueduct was found to all children and the mean diameter of right and left ears were 7.23 and 6.83 mm, respectively. Seven children had totally experienced 13 episodes of sudden hearing deterioration. After receiving corticosteroid therapy in time, 11 of 13 episodes had indicated significant responses to treatment, a response rate of 85%. Early detection of LVAS and the timing of treatment are crucial for preventing the residual hearing from deteriorating. As soon as the hearing deterioration of a child with LVAS is recognized, aggressive intervention such as corticosteroid therapy should be performed in no time.