Grand Rounds: Man, 46, With a Curious Ear Pain
A 46-year-old man presented to a hospital emergency department (ED) with a four-day history of right ear pain. He described the pain as a constant, dull, burning pain radiating to the neck and face, associated with a feeling of congestion. The patient also stated that the right side of his face had felt numb for about one day.
Three days earlier, the man had been seen by his primary health care provider, who told him that his ear looked normal and free of infection. The day before his current presentation to the ED, however, he noticed what he described as an “acne-like” rash on his ear lobe. Shortly before coming to the ED, the patient also developed numbness over his right upper lip, which he likened to the effects of procaine during a dental visit. He reported drooling from the right side of his mouth while drinking water and difficulty blinking his right eye.
He denied any tinnitus, fever, headache, or change in hearing. A review of symptoms was positive only for mild dizziness during the previous two to three days.
The patient was a well-appearing white man. He was alert and oriented to identity, time, and place. His skin was warm, dry, and intact. The examiner noticed a small area of erythematous rash with vesicles on the man’s right ear lobe. The external auditory canals appeared within normal limits, with no erythema or edema, and were nontender bilaterally. The tympanic membranes were normal bilaterally, without bulging or discernible fluid levels.
The ocular exam was normal with no visual acuity changes and no fluorescein uptake; external ocular movements were intact. A slight droop was noted in the right eyelid, but there was no droop on the contralateral side of his face. When asked to puff up his cheeks, the patient found it difficult to do so on the right side of his mouth without releasing air from his lips.
The remainder of the cranial nerves were intact. Muscle strength was 5/5 in all extremities and equal bilaterally. The man’s gait was within normal limits, and the remaining findings in the physical exam were normal.
The initial diagnosis considered in the differential was otitis externa, because it is a common explanation for ear pain in patients who present to the ED.1,2 Also, in otitis, pain is characteristically present in the affected ear, and erythema is often found in the external auditory canal.3 However, this diagnosis was deemed unlikely because otitis externa would not explain the neurologic findings or the vesicular rash.1
Bell’s palsy was next in the differential, as it was considered consistent with the patient’s unilateral neurologic deficits.4 In addition to weakness or palsy of the facial nerve, many patients with Bell’s palsy complain of mastoid pain, which can be confused with a complaint of ear pain.5 However, patients with Bell’s palsy have no rash, and this diagnosis was considered unlikely.
The painful, burning rash on the patient’s face was characteristic of herpes zoster (shingles), which was next in the differential. Infrequently, shingles can also cause weakness in the nerve it affects. In the case patient, weakness that was evident in the affected nerve resembled that seen in Bell’s palsy. This combination of symptoms is referred to as Ramsay Hunt syndrome—which in this case was decided to be the correct diagnosis.
Ramsay Hunt syndrome (RHS, also known as geniculate herpes5,6) is caused by the varicella-zoster virus, most commonly known as the cause of chickenpox. In the United States, RHS is believed to affect only about one in 1,500 persons, although 20% to 30% of persons experience herpes zoster infection at some time.7
Soon after a chickenpox infection subsides, the virus spreads along the sensory nerve fibers of the peripheral and cranial nerves. The virus then becomes dormant in the dorsal root ganglion, where in some patients it later reactivates in the form of shingles.8
In RHS, the ganglia of cranial nerve VII (CN VII, the facial nerve, which innervates the facial muscles) are infected; for this reason, the condition is also referred to as zoster oticus.9 Because of the involvement and weakening of the facial nerve, the presentation of RHS often resembles that of Bell’s palsy or facial nerve palsy.
While most cases of Bell’s palsy are idiopathic,10,11 RHS can usually be attributed to viral infection—most commonly, infection with herpes simplex virus type 1 (HSV-1).12 RHS can be differentiated from Bell’s palsy by the presence of a rash on the ipsilateral side. The rash appears in the form of inflamed vesicles on an erythematous base and may be present around the ear (see figure), the eardrum, the hard and soft palate, or the tongue.6 When the rash is painful, it is often described as a burning pain. Loss of taste may occur in the anterior portion of the tongue.9,12
Unlike shingles, which usually manifests as a sensory neuropathy, RHS is distinguished by motor neuropathy.7 The patient usually reports weakness in the facial muscles on one side, leading to difficulty drinking water or puffing out the cheek and to drooling on one side of the face. A complaint of dryness in the ipsilateral eye may result from weakness or an inability to close the eyelid.
It is important to note that as in Bell’s palsy, RHS can be differentiated from stroke by the patient’s inability to wrinkle the forehead. The motor muscles of the forehead are innervated by both sides of the brain; in the case of stroke, only one side of the brain is affected, and movement of the forehead remains possible on the contralateral side. In facial nerve palsy, the nerve itself is affected; thus, no movement of the forehead is possible.13 Other common complaints in patients with facial nerve palsy include vertigo, hearing loss, and changes in facial sensation.
RHS was first described in 1907 as herpes zoster associated with Bell’s palsy by the neurologist J. Ramsay Hunt, for whom the condition is named.9,14 RHS is more common in men than women. It occurs most commonly in adults and is rare in children younger than 6.13,15
In most cases, a diagnosis of RHS is made on a clinical basis.1 However, a polymerase chain reaction (PCR) assay can be performed on samples of tear fluid or submandibular saliva to detect the zoster virus.16,17 PCR can also be performed using exudates from the geniculate zone of the ear (a small area in the center of the auricle6,14), which is more sensitive than tears or blood.18,19 Findings from a complete blood count and the erythrocyte sedimentation rate can be used to differentiate between infectious and inflammatory causes.13
Head CT or MRI can be obtained to rule out any structural lesions. In one study, Kim et al20 examined MRI changes in patients with either Bell’s palsy or RHS. In both conditions, researchers were able to identify swelling of the labyrinthine segment of the facial nerve on temporal MRI scans.20 Although CT has not been shown to have any prognostic or diagnostic application, it can occasionally be used if decompression of the facial nerve is warranted.11
Data used to support the use of corticosteroids for treatment of Bell’s palsy10,21,22 have been extrapolated to justify their use for treatment of RHS,23 and prednisolone is the most common choice.10 Steroids reduce the associated inflammation, resulting in decreased pain and neurologic symptoms. A daily dose for one to two weeks, followed by a slow taper, is the preferred prescribing method.10
The addition of acyclovir has been recommended to inhibit viral DNA replication9,23 (valacyclovir and famciclovir have also been mentioned12,18). If started within three days of symptom onset, acyclovir can help reduce pain and hasten resolution of symptoms.
In a large retrospective study, it was demonstrated that patients treated with prednisone at 1.0 mg/kg/d for five days, followed by a 10-day taper, combined with acyclovir, showed long-term improvement that was statistically significant.23 Complete facial recovery was reported in only 52% of patients, however. Risk factors for a poor prognosis include hypertension, diabetes mellitus, and advancing age.7
Artificial tears are also prescribed to keep the affected eye from becoming irritated and dry. The patient can be instructed to tape the eye closed at night.10
Early diagnosis and treatment (ie, within three days of symptom onset, and preferably with a combination of acyclovir and steroids23) is an important factor in a good prognosis.7,23 Because RHS-affected patients have only about a 50% chance of full recovery,23 proper follow-up care is extremely important. Follow-up visits are recommended at two weeks, six weeks, and three months.13 For optimal outcomes in patients with this neurologic diagnosis, referral to a neurologist is recommended for ongoing management. This practitioner is likely to detect subtle changes in patient presentation and can perform follow-up testing as needed.
THE CASE PATIENT
One week after the patient’s visit to the ED, he was contacted by hospital staff for a standard satisfaction and quality control survey. The patient (who had been treated with steroids and acyclovir, ibuprofen, and artificial tears) reported almost complete resolution of his pain; any mild pain, he said, was easily tolerated or could be resolved with OTC medication. He reported only minimal persistent facial weakness, stating that he was able to eat, drink, and speak normally.
The patient had not been seen by any health care provider for follow-up, but he agreed to make an appointment as soon as possible.
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3. Rosenfeld RM, Brown L, Cannon CR, et al; American Academy of Otolaryngology—Head and Neck Surgery Foundation. Clinical practice guideline: acute otitis externa. Otolaryngol Head Neck Surg. 2006;134(4 suppl):S4-S23.
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21. Engström M, Berg T, Stjernquist-Desatnik A, et al. Prednisolone and valaciclovir in Bell’s palsy: a randomised, double-blind, placebo-controlled, multicentre trial. Lancet Neurol. 2008;7(11):993-1000.
22. Hato N, Yamada H, Kohno H, et al. Valacyclovir and prednisolone treatment for Bell’s palsy: a multicenter, randomized, placebo-controlled study. Otol Neurotol. 2007;28(3):408-413.
23. Murakami S, Hato N, Horiuchi J, et al. Treatment of Ramsay Hunt syndrome with acyclovir-prednisone: significance of early diagnosis and treatment. Ann Neurol. 1997;41(3):353-357.