Vision Lagging Behind Upward Head Movement
This symptom most likely represents a vestibulo-ocular reflex (VOR) deficit, but you must urgently rule out skew deviation—a vertical strabismus caused by posterior fossa pathology including stroke, demyelination, or mass lesions that demands immediate neuroimaging. 1, 2
Critical Differential Diagnosis
The primary concern is distinguishing between benign VOR dysfunction and life-threatening central pathology:
Skew Deviation (Requires Urgent Action)
- Skew deviation is a vertical strabismus resulting from disruption of vestibular pathways in the inner ear, brainstem, or cerebellum and presents with vertical diplopia, ocular torsion, and head tilt (the ocular tilt reaction). 1, 2
- The hypertropic eye demonstrates fundus incyclotorsion while the hypotropic eye shows fundus excyclotorsion—this pattern distinguishes it from fourth nerve palsy where the hypertropic eye is excyclotorted. 1, 2
- Urgent MRI of brain and brainstem with and without contrast is mandatory to evaluate for stroke, demyelination, or mass lesions. 1, 2
Bilateral VOR Deficit (More Benign)
- Bilateral vestibular loss causes oscillopsia (illusion of visual oscillation) specifically during head movements, as the VOR fails to stabilize gaze. 3
- Common causes include post-meningitic damage, gentamicin ototoxicity, or bilateral idiopathic vestibular failure. 3
Examination Priorities
Perform a focused neuro-ophthalmic examination looking for red flags:
- Complete sensorimotor evaluation including the three-step test for vertical misalignment and fundus examination for torsion patterns. 1
- Check for associated neurologic signs: Horner's syndrome, other cranial nerve palsies, internuclear ophthalmoplegia (INO), nystagmus (especially direction-changing nystagmus), ataxia, hemiparesis, or sensory loss. 1, 2
- Perform the upright-supine test: In chronic skew deviation (>2 months), hypertropia reduces by ≥50% when supine (sensitivity 80%, specificity 100%), though this is unreliable in acute presentations. 1, 2
- Assess for papilledema or optic atrophy on fundus examination and consider visual field testing. 1
- Head impulse test: A normal horizontal head impulse test with vertical misalignment suggests central pathology rather than peripheral vestibular disease. 4
When to Image Immediately
Obtain urgent MRI if ANY of the following are present:
- Any additional neurological signs or symptoms beyond isolated vertical diplopia. 1, 2
- Small vertical fusional amplitudes in an isolated case without trauma history (suggests acquired central cause). 1, 2
- Direction-changing nystagmus in eccentric gaze. 4
- Symptoms of acute vestibular neuronitis (severe vertigo, nausea, vomiting) combined with vertical diplopia—11% of skew deviation cases have no other neurologic signs. 1, 2
Localization Based on Examination
The anatomic location determines the pattern of misalignment:
- Rostral pons/midbrain lesions (e.g., with INO): Contralateral hypotropia and head tilt. 1
- Vestibular periphery/medulla/caudal pons lesions: Ipsilateral hypotropia and head tilt. 1
Mandatory Specialist Referrals
- Neuro-ophthalmology and neurology consultation for all cases of suspected skew deviation to evaluate vestibular-ocular coordination, perform specialized testing for ocular torsion, and assess the ocular tilt reaction. 2, 5
- Otolaryngology consultation if peripheral vestibular pathology (vestibular neuritis, labyrinthitis) is suspected based on clinical features. 1, 5
Management Approach
Acute Phase
- Initial treatment with prisms may help manage diplopia while awaiting possible recovery or definitive diagnosis. 1
- Treatment is symptom-directed, targeting improved binocular vision, diplopia control, and reduced tilt of subjective visual vertical. 1
Chronic Phase (if no resolution after 6 months)
- Consider botulinum toxin, prism therapy, or strabismus surgery for persistent cases. 1
- Surgical goals should address the patient's specific complaints: diplopia, head tilt, and/or perceived visual vertical tilt. 1
Critical Pitfalls to Avoid
- Do not assume benign etiology even with isolated vertical diplopia—serious central pathology can present without other neurologic signs. 2
- Do not rely solely on the three-step test to exclude skew deviation, as it can mimic fourth nerve palsy. 1, 2
- Do not delay imaging in patients with poor fusional amplitudes, atypical features, or any associated neurologic symptoms. 2
- Do not miss giant cell arteritis in elderly patients with temporal tenderness or jaw claudication—this requires immediate treatment to prevent permanent vision loss. 1