Augmented ocular vestibular evoked myogenic potentials to air-conducted sound in large vestibular aqueduct syndrome

Objective: To demonstrate the value of recording air-conducted ocular Vestibular Evoked Myogenic Potentials (oVEMP) in a patient with bilaterally enlarged vestibular aqueducts. Design: Cervical VEMP and oVEMP were recorded from a patient presenting with bilateral hearing loss and imbalance, attributable to large vestibular aqueduct syndrome. The stimuli were air-conducted tone bursts at octave frequencies from 250 to 2000 Hz. Amplitudes and thresholds were measured and compared with the normal response range of 32 healthy control subjects. Results: oVEMP reflexes demonstrated pathologically increased amplitudes and reduced thresholds for low-frequency tone bursts. Cervical VEMP amplitudes and thresholds were within normal limits for both ears across all frequencies of stimulation. Conclusions: This study is the first to describe the augmentation of AC oVEMPs in an adult with large vestibular aqueduct syndrome.