摘要：Introduction. Although the association between inner ear abnormalities and progressive sensorineural hearing loss is well known, vestibular signs or loss of vestibular function in these patients are often unrecognised by medical practitioners. Case report. We report on two siblings with identical inner ear malformations, with special reference to the vestibular symptoms they displayed, as well as the rapid decline in hearing they both experienced in early childhood. We provide a brief overview of the latest classification of these inner ear defects as well as a review of the literature pertaining to children with inner ear malformations presenting with vestibular symptoms. Conclusion. Gross anatomical defects of the inner ear are present in approximately 20% of cases of congenital hearing loss. These defects may result in a rapidly progressive hearing loss. Any child presenting with vestibular symptoms should be referred for an audiological assessment.
