This paper presents the second case in the literature of large vestibular aqueduct syndrome without associated cochlear anomalies in 2 members of the same family. The syndrome is frequently associated with sensorineural hearing loss presenting in childhood. The onset is commonly sudden, following an event causing increased intracranial pressure. On the basis of an emerging pattern of inheritance, we recommend screening siblings of an affected child. We also discuss the importance of characterizing the extent of disease of the inner ear.
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