Assessment and Management of Facial Nerve Palsy

BMJ 2015;351:h3725 doi: 10.1136/bmj.h3725 (Published 16 September 2015) Page 1 of 5

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Assessment and management of facial nerve palsy

1 Liam Masterson specialty registrar, ear, nose, and throat surgery , Martin Vallis general practitioner 2 3 principal , Ros Quinlivan consultant, neuromuscular disease , Peter Prinsley consultant, ear, nose, and throat surgeon 4

1Ear, Nose, and Throat Department, Cambridge University Hospitals NHS Trust, Cambridge CB2 0QQ, UK; 2Rosedale Surgery, NHS Great Yarmouth and Waveney Clinical Commissioning Group, Lowestoft, UK; 3MRC Centre for Neuromuscular Disease, National Hospital for Neurology and Neurosurgery, London, UK; 4Ear, Nose, and Throat Department, Norfolk and Norwich University Hospital NHS Trust, Norwich, UK

The facial nerve is important for both communication and When an upper motor neurone lesion is suspected, it may help expression, and impairment of its function can severely affect to determine whether this is localised to the brainstem or cerebral quality of life.1 The main concern at first presentation of a facial cortex. Brainstem disease may present with vertigo, ataxia, or nerve lesion is to exclude the possibility of a stroke or other crossed neurology signs (ipsilateral cranial nerve involvement serious cause.2 The figure⇓ outlines possible causes. Correct and contralateral hemiplegia). A cortical lesion often affects the management within the first few days may prevent long term contralateral limbs and involuntary movements of the face, such complications. as spontaneous smiling, may be spared.6 Urgent referral to How is it assessed? secondary care (neurology or acute medical unit) is needed at this stage to assess the need for thrombolysis. The facial nerve is responsible for motor supply to the muscles of facial expression (frontalis, orbicularis oculi, buccinators, Lower motor neurone and orbicularis oris) and stapedius, parasympathetic supply to Once a central cause for facial palsy has been excluded, perform the lacrimal and submandibular glands, and sensory input from a focused examination of the ears, mastoid region, oral cavity, the anterior two thirds of the tongue. Thus, as well as a facial eyes, scalp, and parotid glands to look for the specific signs in droop, patients may present with a dry eye, reduced corneal the table⇓. Bell’s palsy is an idiopathic lower motor neurone reflex, drooling, hyperacusis, altered taste, otalgia, and speech 5 (LMN) facial nerve paralysis that accounts for most new cases articulation problems. 3 7 (incidence 10-40/100 000 population each year). However, 30-41% of patients with LMN facial nerve weakness will have Upper motor neurone another cause that requires specific management and is often After identifying the affected side, it is important to establish associated with a poorer prognosis.2-4 whether an upper motor neurone lesion is responsible for the 3 Bell’s palsy is a diagnosis of exclusion, made only after facial weakness. Although not an infallible sign, classic excluding features in the table. Epidemiological studies suggest neurology describes a bilateral innervation of that part of the that it normally develops fully within 24-48 hours.3 4 Most cases facial nuclei supplying the forehead, and thus preserving (60%) are associated with mild post-auricular pain. Incidence forehead movement in upper motor lesions. Lower motor does not differ significantly with ethnicity or sex, but diabetes neurone disorders of the main nerve trunk result in a weakness is associated with as many as 10% of cases, and it occurs more of the entire side of the face. often in the last trimester of pregnancy (three times baseline Patients may have risk factors for stroke, which include older risk).8 Incomplete facial nerve paralysis at presentation (some age (>60 years), hypertension, previous stroke or transient residual muscle movement and partial closure of the eyelid) is ischaemic attack, diabetes, high cholesterol, smoking, and atrial a good prognostic sign, indicating a 94% chance of full recovery, fibrillation.6 Corroborative evidence may also be found by as opposed to 61% in those with complete paralysis.3 examining for abnormalities in other cranial nerves and the The next most common cause of facial nerve paralysis is trauma peripheral nervous system—increased tone, limb weakness, 4 (accidental or surgical). Accidental trauma includes any sharp hyper-reflexia, upgoing plantars, and sensory loss. or blunt mechanism of injury, such as facial laceration, stab injury, or temporal bone fracture. Detection of a temporal bone

Correspondence to: L Masterson [email protected]

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The bottom line

• In patients presenting with facial weakness, the first priority is to exclude an upper motor neurone lesion; important associated signs may include concurrent limb weakness, hyper-reflexia, upgoing plantars, or ataxia • Check for causes of a lower motor neurone lesion by examining the ears, mastoid region, oral cavity, eyes, scalp, and parotid glands • Bell’s palsy is a diagnosis of exclusion, and oral steroids are needed within 72 hours to increase the chance of complete recovery. Prognosis is usually good compared with other causes of lower motor neurone weakness, such as tumours and Ramsay Hunt syndrome • Eye protection is crucial if lid closure is impaired

How patients were involved in the creation of this article

We sought feedback on the paper from patient and medical representatives of the charity Facial Palsy UK. We incorporated their comments into the paper and developed a patient consultation guide for management and prognosis of Bell’s palsy (see box below) fracture is essential because of the 90% risk of associated movement of one part of the face due to aberrant re-innervation), intracranial disease.9 A postmortem correlation study indicates and autonomic dysfunction (crocodile tears or hemi-facial that periorbital bruising (racoon sign), mastoid bruising (Battle’s spasm). In Bell’s palsy, routine investigations in the primary sign), and blood in the ear canal have a positive predictive value care setting are no longer recommended.2 of 85%, 66%, and 46%, respectively, for detecting a temporal bone fracture.10 Cerebellopontine angle and middle ear surgical Red flags for urgent referral procedures are the main causes of iatrogenic injury, which may These include potential upper motor neurone causes (such as also be seen after procedures carried out on the parotid gland limb paresis, paraesthesia of the face or limbs, involvement of or any other region along the facial nerve.4 other cranial nerves, postural imbalance), trauma, features After infection by herpes zoster virus, a geniculate ganglionitis suggesting cancer (such as gradual onset, persistent facial causes a prodrome of otalgia and vesicular eruption within the paralysis >6/12, pain within the facial nerve distribution, ear canal, with or without spread to the oral cavity. Facial ipsilateral hearing loss, suspicious head or neck lesion, previous paralysis (Ramsay Hunt syndrome) normally follows this and regional cancer), and acute systemic or severe local infection. is associated with sensorineural hearing loss and vertigo in 40% Urgent paediatric referral is warranted in children, for whom 6 of cases owing to involvement of cranial nerve VIII. Patients Bell’s palsy is less likely to be a cause of facial weakness (<50% with Ramsay Hunt syndrome generally have a poorer prognosis of cases).5 than those with Bell’s palsy, with only 21% showing full 3 recovery at 12 months. Treatments applicable to all patients A slowly progressive onset of facial weakness is suggestive of 3 5 Eye care is paramount for those with corneal exposure. To cancer. In addition, cancer may be associated with pain or prevent ulceration or dehydration of the cornea, apply artificial paralysis of select branches of the nerve, such as zygomatic 7 tears (such as hypromellose drops) every one or two hours (eyelid) or marginal mandibular (angle of mouth) branches. during the day. At night, keep the eye moist by using a thin strip There may be a history of regional cancer. A thorough of paraffin based ointment (such as Lacrilube) and secure the examination of the head and neck region will be needed to look upper eyelid in the closed position by applying of permeable for cervical lymphadenopathy, a parotid mass, or a scalp lesion synthetic tape (http://www.facialpalsy.org.uk/advice/guides/ in particular. Acoustic neuromas account for about 80% of how-to-tape-eyes-shut/433). All cases of incomplete eyelid cerebellopontine angle lesions and most cases of tumour related closure require urgent ophthalmology consultation at LMN facial nerve paralysis; they can be differentiated from presentation. other causes by ipsilateral sensorineural hearing loss (95% of cases) and absence of the corneal reflex (60%).4 11 Some cases of LMN facial palsy will need to be referred to the ear, nose, and throat department or another hospital specialty. Bacterial infections are responsible for 1-4% of new cases of 3 4 Such cases include all patients with atypical symptoms (see LMN facial palsy. Acute otitis media accounts for most and table) and those with suspected Bell’s palsy who do not respond is associated with systemic sepsis, a bulging tympanic to a trial of oral prednisolone (observe for maximum of two to membrane, conductive hearing loss, and pinna lateralisation. three weeks). Post referral tests may include computed Malignant otitis externa (or, more accurately, skull base tomography or magnetic resonance imaging to visualise the osteomyelitis) is characterised by lack of sleep due to otalgia. skull base, stylomastoid foramen, and parotid gland; blood tests More than 95% of cases are seen in older people (>65 years), (full blood count, urea, and electrolytes); pure tone audiography; immunocompromised people, and those with poorly controlled and topographical studies (such as Schirmer’s test, taste diabetes.12. The condition s associated with Pseudomonas 12 sensation, and stapedial reflex). Electroneuronography can guide aeruginosa infection. Lyme disease is a bacterial infection prognosis in cases of complete paralysis, but this test is caused by a tick bite that results in facial nerve paralysis in one 13 expensive, time consuming, and it has a short window of in 10 seropositive patients. opportunity after onset of symptoms (less than three weeks). In the case of longstanding facial palsy, impairment of eye closure How is it managed? may require insertion of a gold weight into the upper eyelid or lateral tarsorrhaphy.14 Management is influenced strongly by the initial clinical review and provisional working diagnosis. With an LMN lesion, the Alternative rehabilitation methods include physical therapy main priority is to treat the underlying cause, to improve (facial retraining exercises, transcutaneous electrical stimulation, symptoms, and to reduce associated morbidity, such as acupuncture), botulinum toxin injections (to reduce facial muscle contracture of the facial muscles, synkinesis (involuntary contractures, synkinesis, and hemifacial spasm), dynamic facial reanimation surgery, or counselling. Of these interventions, For personal use only: See rights and reprints http://www.bmj.com/permissions Subscribe: http://www.bmj.com/subscribe BMJ 2015;351:h3725 doi: 10.1136/bmj.h3725 (Published 16 September 2015) Page 3 of 5

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only physical therapy has been subjected to controlled trials,15 Thanks to Fiona Walter (general practitioner principal and senior lecturer, and no overall benefit over placebo was found. Tailored facial primary care unit, University of Cambridge, UK), Chaudhary Riaz (GP retraining exercises show limited evidence of earlier recovery specialty registrar, The Pennine Acute Hospitals NHS Trust, UK), of nerve function but this result needs to be confirmed by future Anthony Males (GP principal and senior partner, York Street Medical trials that are adequately powered with low risk of bias. Practice, Cambridge), and Martin Roland (primary care unit, University of Cambridge) for their expert review and constructive feedback. The Treatments in primary care for Bell’s palsy patient consultation guide (box) for Bell’s palsy was produced in and Ramsay Hunt syndrome conjunction with the charity Facial Palsy UK. Corticosteroids in Bell’s palsy Competing interests: We have read and understood BMJ policy on declaration of interests and declare the following interests: none. A Cochrane review (1569 patients, eight randomised trials) Provenance and peer review: Not commissioned; externally peer found that significantly more patients taking oral steroids reviewed. recovered complete motor function, compared with those taking placebo, if started less than 72 hours after symptom onset (77% 1 Coulson SE, O’Dwyer NJ, Adams RD, et al. Expression of emotion and quality of life after v 67%; relative risk 0.71, 95% confidence interval 0.61 to facial nerve paralysis. Otol Neurotol 2004;25:1014-9. 0.83).16 They also had significantly fewer motor synkinesis 2 Baugh RF, Basura GJ, Ishii LE, et al. Clinical practice guideline: Bell’s palsy executive 16 summary. Otolaryngol Head Neck Surg 2013;149:656-63. symptoms (0.60, 0.44 to 0.81). The results of one randomised 3 Peitersen E. Bell’s palsy: the spontaneous course of 2500 peripheral facial nerve palsies trial that recruited patients to oral steroids versus placebo up to of different etiologies. Acta Otolaryngol Suppl 2002;549:4-30. 4 May M, Schaitkin B, Shapiro A. The facial nerve. Thieme, 2001. one week after symptoms began were significantly inferior to 5 Holland NJ, Bernstein JM. Bell’s palsy. BMJ Clin Evid 2014;2014. pii:1204. 17 18 those of trials that recruited within 48 hours. Randomised 6 Donnan GA, Fisher M, Macleod M, et al. Stroke. Lancet Neurol 2008;371:1612-23. 7 De Diego-Sastre JI, Prim-Espada MP, Fernández-García F. The epidemiology of Bell’s trials showed two steroid regimens to be of similar, significant palsy. Rev Neurol 2005;41:287-90. benefit—prednisolone 25 mg twice daily for 10 days or 60 mg 8 Kosins AM, Hurvitz KA, Evans GR, et al. Facial paralysis for the plastic surgeon. Can J once daily for five days (the last dose should be tapered by 10 Plast Surg 2007;15:77-82. 18 19 9 Kamerer DB, Thompson SW. Middle ear and temporal bone trauma. In: Bailey BJ, ed. mg/day over the subsequent five days). A systematic review Head and neck surgery—otolaryngology. 3rd ed. Lippincott Williams & Wilkins, of 10 randomised controlled trials found no significant difference 2001:1108-14. 20 10 Pretto Flores L, De Almeida CS, Casulari LA. Positive predictive values of selected clinical in adverse event rates between oral steroids and placebo, signs associated with skull base fractures. J Neurosurg Sci 2000;44:77-82. although most studies excluded patients with specific 11 Sharp MC, MacfArlane R, Hardy DG et al. Team working to improve outcome in vestibular schwannoma surgery. Br J Neurosurg 2005;19:122-7. contraindications (such as poorly controlled diabetes, immune 12 Hobson CE, Moy JD, Byers KE, et al. Malignant otitis externa: evolving pathogens and compromise, hypertension, peptic ulcer disease, glaucoma, implications for diagnosis and treatment. Otolaryngol Head Neck Surg 2014;151:112-6. 13 Smith RP, Schoen RT, Rahn DW, et al. Clinical characteristics and treatment outcome active tuberculosis, first and second trimester of pregnancy, of early Lyme disease in patients with microbiologically confirmed erythema migrans. Ann 2 5 21 sepsis, renal or hepatic impairment, and psychosis). It is Intern Med 2002;136:421-8. important to discuss specific risks versus benefits of treatment 14 Buchanan M, Lyons M. Clinical review: Bell’s palsy GPonline 2010. www.gponline.com/ clinical-review-bells-palsy/neurology/article/1000464. with patients (box). 15 Teixeira LJ, Valbuza JS, Prado GF. Physical therapy for Bell’s palsy (idiopathic facial paralysis). Cochrane Database Syst Rev 2011;12:CD006283. 16 Salinas RA, Alvarez G, Daly F, et al. Corticosteroids for Bell’s palsy (idiopathic facial Antivirals paralysis). Cochrane Database Syst Rev 2010:CD001942. 17 Taverner D. Cortisone treatment of Bell’s palsy. Lancet Neurol 1954;267:1052-4. A meta-analysis of combination therapy (anti-viral (aciclovir 18 Engström M, Berg T, Stjernquist-Desatnik A. Prednisolone and valaciclovir in Bell’s palsy: or valaciclovir) plus oral steroid (prednisolone)) for Bell’s palsy, a randomised, double-blind, placebo-controlled, multicentre trial. Lancet Neurol 2008;7:993-1000. suggested a marginal benefit only when small poorer quality 19 Sullivan FM, Swan IR, Donnan PT. Early treatment with prednisolone or acyclovir in Bell’s 24 trials are included. The conclusion suggested that combined palsy. N Engl J Med 2007;357:1598-607. 20 De Almeida JR, Al Khabori M, Guyatt GH. Combined corticosteroid and antiviral treatment therapy should be reserved for patients with suspected Ramsay for bell palsy: a systematic review and meta analysis. JAMA 2009;302:985-93. Hunt syndrome (herpes zoster virus infection). In these patients, 21 Rafii B, Sridharan S, Taliercio S. Glucocorticoids in laryngology: a review. Laryngoscope primary care physicians may wish to start a trial of oral steroids 2014;124:1668-73. 22 Kenny T, Tidy C. Bell’s palsy. Cox J, series ed. 2013. www.patient.co.uk/health/bells- (dose as described above) if there are no contraindications, palsy. together with an antiviral agent (such as 1 g valaciclovir three 23 Walker D, Hallam M, Ni Mhurchadha S, et al. Our experience: the psychosocial impact 5 25 of facial palsy. Clin Otolaryngol 2012;37:474-7. times daily for one week. Because of the relatively poor 24 Browning GG. Bell’s palsy: a review of three systematic reviews of steroid and anti-viral functional outcome in this group and the high rate of coexistent therapy. Clin Otolaryngol 2010;35:56-8. 25 Lockhart P, Daly F, Pitkethly M, et al. Antiviral treatment for Bell’s palsy (idiopathic facial sensorineural hearing loss, it is advisable (where appropriate) paralysis). Cochrane Database Syst Rev 2009;4:CD001869. to consider referral to ear, nose, and throat specialists for repeat Accepted: 15 February 2015 assessment and imaging of the cerebellopontine angle.

Cite this as: BMJ 2015;351:h3725 © BMJ Publishing Group Ltd 2015

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Patient consultation guide for management and prognosis of Bell’s palsy What is Bell’s palsy? This condition involves swelling adjacent to the facial nerve as it passes through the skull base into the ear. Compression of this nerve can stop the muscles that it supplies from working. The cause of the swelling is currently unknown.22 How is it managed? Steroid tablets (usually prednisolone) help to reduce inflammation and are normally taken for 10 days.18 19 This short course of drugs is unlikely to have notable side effects if you have no history of high blood sugar levels (diabetes), hypertension, gastric ulcer, or glaucoma.22 To ensure maximum benefit, the steroid tablets should be started within three days of the facial weakness appearing.5 If the eyelid cannot shut completely, the surface of the eye may dry up and be harmed. In addition, the tear ducts may not function temporarily, which could dry the eye further.22 Treatment is needed to keep the eye moist. This normally involves frequent lubricating eye drops during the day. At night, eye ointment can be applied before closing the eye shut with tape. What is the outcome? Most studies indicate that if a steroid is not prescribed, seven of 10 patients will recover completely. If a steroid is taken, eight of 10 patients will recover completely.16 Most patients will note an improvement in their facial weakness within three weeks, with the remainder resolving by three to five months.3 In the 20-30% of cases where facial weakness does not recover fully, further interventions may be considered. These may include physiotherapy to undergo “facial retraining exercises” or Botox injections to help with muscle spasms. It is important that other potential causes of facial palsy are excluded by referral to a specialist if recovery fails to progress. A considerable proportion of patients are left with psychosocial concerns because of their limited facial function and may need psychological support.23 Surgical treatments also help improve the functional and cosmetic appearance of the face.

Table

Table 1| Clinical signs to check in lower motor neurone facial nerve palsy

Are the ears clear on otoscopy? Assess the tympanic membrane and ear canal; acute or chronic otitis media (±cholesteatoma) and malignant otitis externa can all cause lower motor neurone facial palsy and will require further urgent assessment in secondary care (ear, nose, and throat) Is there ipsilateral hearing loss? Perform Weber’s and Rinne’s tuning fork tests; although Ramsay Hunt syndrome will be associated with sensorineural hearing loss (SNHL), it is important to exclude a cerebellopontine angle lesion (normally presents with gradual onset unilateral SNHL); acute otitis media or cholesteatoma may be associated with conductive hearing loss, whereas patients with Bell’s palsy normally have abnormal sensitivity to loud sounds Is there a rash? Small vesicular eruptions that affect the tympanic membrane, ear canal, external pinna, or oral cavity may indicate Ramsay Hunt syndrome; Lyme disease is restricted to heavily forested regions and presents with a characteristic erythematous “bullseye” lesion on the limbs or trunk (70% of cases), arthralgia, and facial swelling; it is caused by a bite from a tick carrying Borrelia burgdorferi (US) or B afzalii/garinii (Europe), and an antibody test may help confirm the diagnosis13 Are there any bruises or scars in the head The palsy may be secondary to a skull base fracture (common associated signs include periorbital or mastoid bruising, and neck region? blood in the ear canal, and haemotympanum9) or recent mastoid, parotid, or submandibular gland surgery Is the corneal reflex intact on both sides In addition to facial muscle weakness, a cerebellopontine angle lesion (such as an acoustic neuroma) may cause a reduced or absent corneal reflex on the affected side ± aural fullness (owing to trigeminal nerve deficit) Is the mastoid region tender or swollen? A tender swelling of the mastoid with associated middle ear inflammation or pinna lateralisation (or both) may suggest acute mastoiditis Is the parotid gland enlarged? Palpation of a parotid lump may suggest cancer (particularly if associated with a history of regional skin cancer, delayed onset facial palsy, or pain); if cancer is suspected, thoroughly examine the rest of the head and neck and refer urgently (for example, through the “two week wait suspected head and neck cancer pathway” in the UK)

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Figure

Differential diagnosis of a unilateral facial palsy. Percentages are based on combined epidemiological data from 6024 patients with lower motor neurone facial palsy (rarer conditions including mumps, syphilis, HIV, Guillain-Barré syndrome, otitic barotrauma, myasthenia gravis, systemic lupus erythematosus, sarcoidosis, and multiple sclerosis have been excluded).3 4 *Endemic in forested regions; †misdiagnosed cerebrovascular disease evident in about 1.5% of all patients3

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