Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

acute symptoms

1st line – oral corticosteroid

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ACUTE
|
acute symptoms
1st line – 

oral corticosteroid

Give a high-dose oral corticosteroid early (i.e., <72 hours from symptom onset) to all patients with Ramsay Hunt syndrome.[1][26][27]

This recommendation is based on the efficacy of corticosteroid treatment shown in studies in patients with Bell's palsy, which is based on reduction of inflammatory oedema.[1][26][27]​ Expert consensus guidelines consider that corticosteroids are still the best treatment option for viral inflammation of the facial nerve; however, it is not clear how corticosteroids work on patients with Ramsay Hunt syndrome.[1] The recommendation to use antiviral treatment is based on its demonstrated efficacy in patients with herpes zoster. Antivirals are believed to reduce acute pain, improve herpes zoster lesions, and reduce the risk of post-herpetic neuralgia.[28]

Data in patients with Ramsay Hunt syndrome are of low quality and have shown mixed efficacy overall.

One retrospective case review (n=128) of patients with Ramsay Hunt syndrome with complete facial paralysis (House-Brackmann scale VI) showed highest rates of recovery in patients receiving early administration of high-dose corticosteroid and antiviral treatment, compared with normal-dose corticosteroid and antiviral treatment, or with high-dose corticosteroid alone (71%, 60%, and 57%, respectively). However, the results were not statistically significant.[29]

One small study (n=91) evaluating a combination of corticosteroids plus antiviral treatment showed higher rates of good nerve excitability (a promising sign of nerve function) in patients with Ramsay Hunt syndrome receiving combination treatment than in those receiving corticosteroids alone (75% and 53%, respectively).​[30]​

One Cochrane review found one randomised controlled trial (n=15) comparing intravenous aciclovir and corticosteroids with corticosteroids alone in patients with Ramsay Hunt syndrome and found no statistically significant difference between the two groups.[31]​ 

Despite the lack of randomised prospective studies, collective data from retrospective studies show that patients with Ramsay Hunt syndrome treated with corticosteroids and antivirals have better recovery rates than those receiving no medication.

General warnings and cautions concerning short-course corticosteroids should be followed. Temporary or permanent adverse effects can occur with high doses (e.g., blurry vision, weight gain, Cushingoid appearance, altered glucose metabolism, dyslipidaemia, hypertension, cataracts, glaucoma, osteoporosis, diabetes). Monitor patients closely for adverse effects. Consider adding a proton-pump inhibitor for gastric protection when prescribing high-dose corticosteroids.

Primary options

prednisolone: 1 mg/kg/day orally for at least 7 days, taper gradually over 5-7 days, maximum 60 mg/day

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Plus – antiviral

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ACUTE
|
acute symptoms
Plus – 

antiviral

Treatment recommended for ALL patients in selected patient group

Give a high-dose antiviral (e.g., aciclovir, valaciclovir), in combination with oral corticosteroids, early (i.e., <72 hours from symptom onset) to all patients with Ramsay Hunt syndrome.[1][26][27]

This recommendation is based on the efficacy of corticosteroid treatment shown in studies in patients with Bell's palsy, which is based on reduction of inflammatory oedema.[1][26][27]​ Expert consensus guidelines consider that corticosteroids are still the best treatment option for viral inflammation of the facial nerve; however, it is not clear how corticosteroids work on patients with Ramsay Hunt syndrome.[1] The recommendation to use antiviral treatment is based on its demonstrated efficacy in patients with herpes zoster. Antivirals are believed to reduce acute pain, improve herpes zoster lesions, and reduce the risk of post-herpetic neuralgia.[28]

Data in patients with Ramsay Hunt syndrome are of low quality and have shown mixed efficacy overall.

One retrospective case review (n=128) of patients with Ramsay Hunt syndrome with complete facial paralysis (House-Brackmann scale VI) showed highest rates of recovery in patients receiving early administration of high-dose corticosteroid and antiviral treatment, compared with normal-dose corticosteroid and antiviral treatment, or with high-dose corticosteroid alone (71%, 60%, and 57%, respectively). However, the results were not statistically significant.[29]

One small study (n=91)​ evaluating a combination of corticosteroids plus antiviral treatment showed higher rates of good nerve excitability (a promising sign of nerve function) in patients with Ramsay Hunt syndrome receiving combination treatment than in those receiving corticosteroids alone (75% and 53%, respectively).[30]

One Cochrane review found one randomised controlled trial (n=15) comparing intravenous aciclovir and corticosteroids with corticosteroids alone in patients with Ramsay Hunt syndrome and found no statistically significant difference between the two groups.[31]

Despite the lack of randomised prospective studies, collective data from retrospective studies show that patients with Ramsay Hunt syndrome treated with corticosteroids and antivirals have better recovery rates than those patients receiving no medication.

Assess kidney function in patients on high-dose antiviral therapy. Use caution in patients with renal impairment; a dose adjustment may be necessary.

Primary options

valaciclovir: 1000 mg orally three times daily for 7 days

OR

aciclovir: 800 mg orally five times daily for 7 days

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Plus – eye protection

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ACUTE
|
acute symptoms
Plus – 

eye protection

Treatment recommended for ALL patients in selected patient group

Keratoconjunctivitis sicca (dry eye) is common and may lead to exposure keratopathy. For patients with facial paralysis and symptomatic eye irritation, give moisture-based therapy (e.g., preservative-free methylcellulose ophthalmic drops) and a lubricant eye ointment.

Advise patients with eye irritation when waking up in the morning to tape the eye shut at night after ointment has been applied to prevent corneal damage.[32]​ Eye patches are contraindicated because the eye may easily open under the patch, leading to corneal abrasion.​

ONGOING

chronic symptoms

1st line – referral to specialist

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ONGOING
|
chronic symptoms
1st line – 

referral to specialist

Many patients will develop chronic Ramsay Hunt syndrome, despite prompt treatment, with chronic facial paralysis manifested by facial asymmetry, facial tightness, and facial synkinesis (i.e., non-flaccid facial paralysis, post-paralytic facial paralysis).[36] Long-term treatment for these patients is individualised and highly specialised.

Refer the patient to a specialist in facial reanimation to discuss both surgical and non-surgical options. Chronic facial paralysis can be a significant source of depression, anxiety, and social withdrawal for patients, and close monitoring is warranted.

Post-herpetic neuralgia (defined as pain that persists for >3 months after the cutaneous herpes zoster lesions have resolved) is rare in patients with Ramsay Hunt syndrome. Refer affected patients to a pain specialist.

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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