What is the best course of action for a patient with normal pressure hydrocephalus (NPH) experiencing brief, transient pain behind the ear?

Brief Retroauricular Pain in Normal Pressure Hydrocephalus

This transient, 1-second pain behind the ear is unlikely to be directly related to the normal pressure hydrocephalus (NPH) itself and warrants evaluation for other common causes of brief retroauricular pain, including benign paroxysmal positional vertigo (BPPV), sudden sensorineural hearing loss (SSNHL), or local musculoskeletal issues.

Clinical Context and NPH Symptoms

NPH classically presents with the triad of gait impairment, urinary incontinence, and cognitive decline 1, 2. The characteristic symptoms do not include brief, transient retroauricular pain 1, 3. While NPH patients may develop hydrocephalus-related complications such as headache, altered mental status, or cranial neuropathies, these typically present as persistent symptoms rather than 1-second pain episodes 4.

Differential Diagnosis Considerations

BPPV Assessment

• Brief positional symptoms lasting seconds are characteristic of BPPV, which can present with retroauricular discomfort 4
• BPPV does not cause constant severe dizziness and does not affect hearing 4
• Perform bedside Dix-Hallpike or supine roll testing to evaluate for characteristic nystagmus 4

Sudden Hearing Loss Evaluation

• Ear fullness or pain accompanied by hearing changes, tinnitus, or vertigo requires urgent audiometric evaluation 4
• SSNHL should be evaluated as quickly as possible, particularly if accompanied by pressure sensation in the ear 4
• A comprehensive hearing test (audiogram) should be performed if any auditory symptoms are present 4

NPH-Specific Complications

• Shunt-related issues in NPH patients with existing ventriculoperitoneal shunts can cause new symptoms 4
• However, shunt malfunction typically presents with headache, altered mental status, gait changes, or urinary symptoms rather than brief retroauricular pain 4
• Inner ear biomechanics can be affected by CSF pressure changes in hydrocephalus patients, particularly those with shunts 5

Recommended Evaluation Algorithm

Immediate Assessment

• Otoscopic examination to exclude external or middle ear pathology
• Neurological examination focusing on cranial nerves, particularly CN VII and VIII
• Gait assessment to establish baseline NPH symptoms and detect any acute changes 1, 2

Targeted Testing Based on Associated Symptoms

• If accompanied by hearing changes or tinnitus: Obtain audiogram urgently 4
• If accompanied by positional vertigo: Perform BPPV testing maneuvers 4
• If accompanied by NPH symptom worsening (gait, cognition, urinary function): Consider brain MRI to evaluate ventricular size and assess for shunt malfunction if applicable 2, 6

Clinical Pitfalls to Avoid

Do not attribute all new symptoms to the underlying NPH diagnosis. Brief, transient pain is not a recognized manifestation of NPH 1, 3. Three-quarters of NPH patients have coexisting neurodegenerative disorders, making differential diagnosis essential 1.

Do not delay evaluation of sudden hearing symptoms. Ear fullness or pain with auditory symptoms requires prompt audiometric testing, as SSNHL has time-sensitive treatment implications 4.

Consider local causes first before attributing symptoms to central pathology. Musculoskeletal causes (cervicogenic pain, temporomandibular joint dysfunction) or peripheral nerve irritation are more likely explanations for 1-second retroauricular pain episodes.