Congenital hypoplasia of the nodulus and ventral uvula of the cerebellum in a Norfolk Terrier

Abstract This case report describes a congenital hypoplasia of the nodulus and uvula of the cerebellum in an 8‐month‐old Norfolk Terrier with an episodic, positioning head tilt and mild vestibular ataxia. Neurological examination revealed a head tilt that changed direction with head movement. Magnetic resonance imaging identified a defect in the caudoventral cerebellar vermis. VLDLR‐Gene testing for Dandy‐Walker‐like malformation/cerebellar hypoplasia was negative. Nodulus and uvula hypoplasia, a rare condition in dogs, impairs the nodulus and uvula's role in vestibular coordination, causing postural imbalances. The presenting signs are consistent with previously reported cases in the literature. While the specific cause of the nodulus and uvula hypoplasia remains unknown, this report emphasises the importance of the positioning head tilt in neuroanatomical localisation. No progression of neurological symptoms was noted 3.5 months post‐presentation.