Three patients presented with Machado-Joseph disease. We documented the vestibular abnormalities characteristic of Machado-Joseph disease during clinical examination. Electronystagmographic (ENG) abnormalities included bilateral gaze nystagmus, saccadic pursuit and failure of fixation suppression suggestive of cerebellar-brainstem pathology. Machado-Joseph disease is a degenerative neurological condition, inherited in an autosomal dominant fashion. It occurs in patients of Portuguese Azorean extraction. We suggest that otolaryngologists with a referral base including a Portuguese community be familiar with this disease as it may present in its early stages with symptoms of non-specific imbalance.