An overview of congenital/developmental sensorineural hearing loss with emphasis on the vestibular aqueduct syndrome.

The evaluation of children with congenital/developmental hearing loss has undergone substantial transformation in recent years. This communication focuses on the most common cause of this type of hearing loss for which there are imaging findings: the vestibular aqueduct syndrome. The normal anatomy of the vestibular aqueduct is discussed in detail. The cochlear aqueduct with which it is commonly confused is also discussed. An overview of other congenital defects and abnormal middle/inner ear communications is also included. A brief discussion of cochlear implants is also included for completeness.