CONCLUSIONS: Enlarged vestibular aqueduct (EVA) may precipitate secondary benign paroxysmal positional vertigo (BPPV), especially in pediatric populations, as well as paretic dysfunction of the vestibular end organ. OBJECTIVES: EVA is characterized by the early onset of sudden or progressive sensorineural hearing loss with or without vestibular dysfunction. However, vestibular dysfunction in patients with EVA has not been described as frequently as hearing loss, and an association with BPPV has not been previously reported. By reviewing the cochleo-vestibular status of three children and two young adults with EVA who had been diagnosed with BPPV, characteristic features of BPPV in EVA patients were investigated and possible pathogenetic mechanisms are speculated. METHODS: A retrospective review of medical records was conducted for five patients with EVA who presented with BPPV from January 2004 to July 2009. Clinical courses, characteristics of vertigo and audiovestibular laboratory findings were reviewed. RESULTS: Among 26 patients with radiologically confirmed EVA, 5 (19.2%) exhibited positional nystagmus compatible with BPPV through Dix-Hallpike and head-roll tests. Hearing loss usually accompanied BPPV attacks, and BPPV was recurrent in three patients. Multiple semicircular canals were frequently involved in each episode, and different canals were also involved in recurrent cases. Canalith repositioning procedures were usually successful without difficulty.
CONCLUSIONS:Enlarged vestibular aqueduct (EVA) may precipitate secondary benign paroxysmal positional vertigo (BPPV), especially in pediatric populations, as well as paretic dysfunction of the vestibular end organ. OBJECTIVES: EVA is characterized by the early onset of sudden or progressive sensorineural hearing loss with or without vestibular dysfunction. However, vestibular dysfunction in patients with EVA has not been described as frequently as hearing loss, and an association with BPPV has not been previously reported. By reviewing the cochleo-vestibular status of three children and two young adults with EVA who had been diagnosed with BPPV, characteristic features of BPPV in EVA patients were investigated and possible pathogenetic mechanisms are speculated. METHODS: A retrospective review of medical records was conducted for five patients with EVA who presented with BPPV from January 2004 to July 2009. Clinical courses, characteristics of vertigo and audiovestibular laboratory findings were reviewed. RESULTS: Among 26 patients with radiologically confirmed EVA, 5 (19.2%) exhibited positional nystagmus compatible with BPPV through Dix-Hallpike and head-roll tests. Hearing loss usually accompanied BPPV attacks, and BPPV was recurrent in three patients. Multiple semicircular canals were frequently involved in each episode, and different canals were also involved in recurrent cases. Canalith repositioning procedures were usually successful without difficulty.