Superficial Siderosis is the Most Likely Diagnosis
In a 60-year-old man presenting with progressive cerebellar ataxia and sensorineural hearing loss, superficial siderosis of the central nervous system is the most likely diagnosis. This classic combination of slowly progressive ataxia with hearing loss is pathognomonic for this condition 1, 2, 3.
Clinical Reasoning
Why Superficial Siderosis Fits Best
The combination of progressive cerebellar ataxia and sensorineural hearing loss is the hallmark presentation of superficial siderosis, resulting from chronic hemosiderin deposition in the subpial layers of the brain and spinal cord from recurrent subarachnoid hemorrhage 1, 4, 5.
Progressive ataxia and hearing impairment are the most common manifestations of this disorder, with the slowly progressive nature over months to years being characteristic 6, 4, 7.
The age of presentation (60 years) fits the typical demographic for superficial siderosis, which usually manifests in middle to older adulthood 6, 5.
Why Other Options Are Less Likely
Acute cerebellar hemorrhage presents with sudden onset symptoms including severe headache, altered consciousness, and rapid neurological deterioration—not the progressive course described here 1.
Hypoxic encephalopathy would require a clear history of hypoxic event (cardiac arrest, respiratory failure, carbon monoxide poisoning) and typically causes diffuse brain injury with cognitive impairment and motor deficits, not the selective pattern of cerebellar ataxia with hearing loss 1.
Cerebellar infarction presents acutely with sudden onset ataxia, often with vertigo, nausea, and vomiting, rather than progressive symptoms over time 1.
Congenital cerebellar ataxia would manifest in childhood or early adulthood, not with new onset at age 60 1, 2.
Diagnostic Approach
Essential Imaging
Order MRI brain with gradient-echo T2-weighted sequences immediately, as hypointensity of the marginal zones of the CNS on T2-weighted images indicates iron-induced susceptibility effect and is pathognomonic for superficial siderosis 1, 5.
MRI head without IV contrast is the preferred initial imaging modality for evaluating cerebellar ataxia 2, 3.
If superficial siderosis is confirmed on brain MRI, obtain complete spine MRI to identify the bleeding source, as spinal tumors (particularly ependymomas and paragangliomas) are important treatable causes even in asymptomatic patients 6, 4.
Clinical Examination Findings to Confirm
Look for wide-based gait, dysmetria, truncal instability, and dysarthria that persist regardless of visual input (negative Romberg test), distinguishing cerebellar from sensory ataxia 2, 3.
Document bilateral sensorineural hearing loss on audiometry 1, 6, 4.
Assess for pyramidal tract signs (spasticity, hyperreflexia), which are commonly associated with superficial siderosis 5, 8.
Critical Management Considerations
The only proven treatment to prevent further deterioration is identifying and stopping the source of chronic bleeding into the subarachnoid space 6, 5. This makes early diagnosis paramount.
Common Pitfalls to Avoid
Do not dismiss the diagnosis if there is no clear history of subarachnoid hemorrhage—a definite history of SAH is often lacking, as bleeding may be chronic and silent 4, 5.
Do not delay spinal imaging—any tumoral lesion on spinal MRI should be considered for resection even without documented signs of bleeding, given the severity of potential deterioration 6.
Recognize that the disease may progress secondarily even after successful treatment of the bleeding source, potentially mimicking other neurodegenerative conditions 8.