Objective: To describe Tullio phenomenon in a patient with CANVAS (cerebellar ataxia with neuropathy and vestibular areflexia syndrome).

Background: CANVAS is a progressive ataxia syndrome involving cerebellar degeneration, neuropathy, and vestibular disease. Tullio phenomenon is the sensation of disequilibrium evoked by loud sounds that signifies impaired vestibular function.

Method: Case report and review of literature.

Results: A 67-year-old man with Type 2 diabetes presented for evaluation of progressive imbalance. He endorsed an increased sense of motion and dizziness with loud noises citing worsening at loud sporting events. Initial examination showed normal eye movements with ataxic dysarthria, dysmetria, and ataxia of heel-to-shin testing with lower extremity length dependent sensory loss. His gait was wide based and ataxic with a positive Romberg sign. Two years after presentation, his pancerebellar ataxia worsened with examination showing impaired vestibular ocular reflex, square wave jerks, and a positive head impulse test. Serial MRI brain scans showed slowly progressive cerebellar atrophy.  EMG showed mild length dependent peripheral neuropathy. Autonomic reflex screen showed findings consistent with mild autonomic neuropathy.  Serum and CSF investigations for alternative causes of ataxia were negative. While he was initially diagnosed with sporadic adult onset ataxia, the later emergence of bilateral vestibulopathy with sensory neuropathy led to the diagnosis of CANVAS.

Conclusion: The presentation with Tullio phenomenon highlights a rare initial presentation of CANVAS.  Tullio phenomenon denotes vestibular involvement which in the setting of cerebellar ataxia may be a clue to evaluate for CANVAS.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/tullio-phenomenon-in-canvas-syndrome/