Giant Cell Arteritis Must Be Excluded Urgently
In a 78-year-old woman presenting with scalp paresthesias ("ants sensation") and hearing loss, giant cell arteritis (GCA) is the most urgent diagnosis to exclude, as delayed treatment can result in irreversible blindness and stroke. While the provided evidence focuses on otologic causes of hearing loss, this specific constellation of symptoms in an elderly patient—new scalp dysesthesias combined with hearing loss—raises immediate concern for GCA affecting the temporal artery and potentially the vertebrobasilar circulation.
Immediate Clinical Assessment
Critical Historical Features to Elicit
- Temporal pattern: Onset over days to weeks suggests GCA; onset within 72 hours would indicate sudden sensorineural hearing loss (SSNHL) requiring different management 1
- Associated symptoms of GCA: New-onset headache (especially temporal), jaw claudication, visual changes (amaurosis fugax, diplopia, vision loss), scalp tenderness, constitutional symptoms (fever, weight loss, malaise)
- Hearing loss characteristics: Unilateral versus bilateral, sudden versus gradual, associated tinnitus or vertigo 1
Physical Examination Priorities
- Temporal artery examination: Palpate for tenderness, nodularity, decreased pulsation, or thickening
- Visual assessment: Check visual acuity, visual fields, pupillary responses, and fundoscopy for signs of anterior ischemic optic neuropathy
- Complete otologic examination: Otoscopy to exclude middle ear pathology, assess for retrotympanic masses 1
- Neurologic examination: Cranial nerve function, particularly facial nerve (CN VII) and vestibulocochlear nerve (CN VIII)
Urgent Diagnostic Workup
First-Line Laboratory Studies (Stat)
- Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP): Markedly elevated inflammatory markers (ESR typically >50 mm/hr, often >100 mm/hr) support GCA diagnosis
- Complete blood count: Normocytic anemia and thrombocytosis are common in GCA
- Comprehensive metabolic panel: Baseline renal and hepatic function before initiating high-dose corticosteroids
Audiologic Testing
- Pure-tone audiometry with speech discrimination testing should be obtained urgently to characterize the hearing loss and establish baseline 1
- Determine if hearing loss is sensorineural, conductive, or mixed 1
- Assess for asymmetry: asymmetric sensorineural hearing loss (≥15 dB at 2+ frequencies or ≥15% speech recognition difference) mandates imaging for retrocochlear pathology 1
Imaging Strategy
If GCA is suspected clinically (scalp symptoms + elevated inflammatory markers):
- Temporal artery ultrasound or temporal artery biopsy should be arranged urgently (within 1-2 weeks of starting steroids)
- Do not delay corticosteroid initiation while awaiting biopsy if clinical suspicion is high
For hearing loss evaluation:
- MRI of the internal auditory canals and brain with gadolinium is the gold standard to exclude retrocochlear pathology (vestibular schwannoma, meningioma) in patients with asymmetric or unilateral hearing loss 1
- MRI should be obtained even if GCA is being treated, as 3-10% of patients with sudden hearing loss have vestibular schwannoma 1
- CT angiography of head and neck may be considered if vascular pathology (dissection, dural arteriovenous fistula) is suspected, particularly if tinnitus is pulsatile 2, 3
Immediate Management Algorithm
If GCA is Suspected (High Priority)
- Initiate high-dose corticosteroids immediately (prednisone 40-60 mg daily or equivalent) without waiting for biopsy confirmation to prevent vision loss
- Arrange temporal artery biopsy within 1-2 weeks (histology remains positive for 2-4 weeks after starting steroids)
- Ophthalmology consultation for baseline examination and monitoring
If SSNHL is Diagnosed (Within 72 Hours of Onset)
- Offer oral corticosteroids (e.g., prednisone 1 mg/kg/day, maximum 60 mg, for 7-14 days with taper) as initial therapy if presenting within 2 weeks of symptom onset 1
- Intratympanic steroid therapy should be offered for incomplete recovery after initial therapy or for patients presenting 2-6 weeks after onset 1
- Avoid routine laboratory testing panels (CBC, metabolic panel, lipid panel, thyroid function) as they do not change management and may lead to false-positive results requiring unnecessary workup 1
- Targeted serologic testing only if history suggests specific etiology (e.g., Lyme disease in endemic area with tick exposure, syphilis with risk factors) 1
If Age-Related or Asymmetric Hearing Loss
- Refer to otolaryngology for comprehensive evaluation of medically treatable causes 1
- MRI with gadolinium to exclude vestibular schwannoma or other retrocochlear pathology 1
Critical Pitfalls to Avoid
- Do not dismiss scalp paresthesias in an elderly patient as benign neuropathy without excluding GCA; this combination with hearing loss may represent vertebrobasilar insufficiency from large-vessel vasculitis
- Do not obtain routine head CT for isolated hearing loss evaluation, as it has low yield, exposes the patient to radiation, and will not detect retrocochlear lesions or early brainstem infarcts 1
- Do not delay corticosteroids if GCA is clinically suspected while awaiting confirmatory testing; vision loss can occur within days and is often irreversible
- Do not attribute unilateral hearing loss to age-related changes without imaging; age-related hearing loss should be symmetric 1
- Do not use ABR as the primary screening tool for retrocochlear pathology, as it misses 20% (range 8-42%) of intracanalicular vestibular schwannomas; MRI with gadolinium is the definitive test 1
Follow-Up Considerations
- If GCA is confirmed and treated, monitor inflammatory markers and clinical response; hearing may improve with adequate immunosuppression
- If SSNHL is diagnosed, obtain follow-up audiometry within 6 months to document recovery or progression 1
- If vestibular schwannoma is identified, management options include observation with serial imaging, stereotactic radiosurgery, or microsurgical resection depending on tumor size, growth rate, and patient factors 1
- Approximately one-third to two-thirds of SSNHL patients recover some hearing within 2 weeks; those with minimal improvement in the first 2 weeks are unlikely to show significant spontaneous recovery 1