To the Editor: As a practicing internist, I found Dr. Alexandra Villa-Forte’s review of giant-cell arteritis (Cleve Clin J Med 2011; 78:265–270) both interesting and useful, as usual for the Cleveland Clinic Journal of Medicine. However, she did not mention the recommendation by some experts that patients who have had temporal arteritis should receive annual chest x-rays, for a decade or longer, to screen for the development of thoracic aortic aneurysm. Does she agree with this precaution? Is it advisable, in addition, to screen for abdominal aortic aneurysm by means of abdominal ultrasonography? If so, at what time intervals should this be done?

To the Editor: As a practicing internist, I found Dr. Alexandra Villa-Forte’s review of giant-cell arteritis (Cleve Clin J Med 2011; 78:265–270) both interesting and useful, as usual for the Cleveland Clinic Journal of Medicine. However, she did not mention the recommendation by some experts that patients who have had temporal arteritis should receive annual chest x-rays, for a decade or longer, to screen for the development of thoracic aortic aneurysm. Does she agree with this precaution? Is it advisable, in addition, to screen for abdominal aortic aneurysm by means of abdominal ultrasonography? If so, at what time intervals should this be done?

To the Editor: As a practicing internist, I found Dr. Alexandra Villa-Forte’s review of giant-cell arteritis (Cleve Clin J Med 2011; 78:265–270) both interesting and useful, as usual for the Cleveland Clinic Journal of Medicine. However, she did not mention the recommendation by some experts that patients who have had temporal arteritis should receive annual chest x-rays, for a decade or longer, to screen for the development of thoracic aortic aneurysm. Does she agree with this precaution? Is it advisable, in addition, to screen for abdominal aortic aneurysm by means of abdominal ultrasonography? If so, at what time intervals should this be done?

A 72-year-old man with a 15-year history of a heart murmur presents to his cardiologist with shortness of breath on exertion over the past 12 months. He otherwise feels well and reports no chest discomfort, palpitations, or swelling of his legs or feet. He is not taking any cardiac drugs, and his health has previously been excellent.

Q: Which of the following findings on 12-lead ECG is not commonly reported in chronic severe aortic regurgitation?

• Left ventricular hypertrophy
• QRS complex left-axis deviation
• A negative U wave
• Atrial fibrillation

A: The correct answer is a negative U wave.

In long-standing left ventricular volume overload, such as in chronic aortic regurgitation, characteristic findings on ECG include lateral precordial narrow Q waves and left ventricular hypertrophy. The ST segment and T wave are often normal or nearly normal. The QRS complex vector may demonstrate left-axis deviation, but this is not absolute. In contrast, pressure overload conditions such as aortic stenosis and systemic hypertension commonly manifest as left ventricular hypertrophy with strain pattern of ST depression in lateral precordial leads and asymmetric T-wave inversion.

A negative U wave, best identified in leads V₄ to V₆, is a common finding in left ventricular volume overload. A negative U wave represents a negative deflection of small amplitude (normally < 0.1 to 3 mV) immediately following the T wave. Although not routinely reported, the negative U wave is an indicator of underlying structural heart disease.¹

Q: A negative U wave has been associated with which of the following conditions?

• Aortic or mitral regurgitation
• Myocardial ischemia
• Hypertension
• All of the above

A: The correct answer is all of the above.

Negative U waves have been identified in regurgitant valvular heart disease with left ventricular volume overload, in myocardial ischemia, ^2,3 and in hypertension.⁴ During exercise stress testing, the transient appearance of negative U waves strongly suggests flow-limiting coronary artery disease. Moreover, changes in the U wave during exercise stress testing may be a sign of well-developed coronary collaterals.⁵ Therefore, it is prudent to note their presence on resting ECG and to investigate further with cardiac stress testing and imaging.

The pathogenesis of the negative U wave remains unclear. Of the various hypotheses put forth, a mechano-electric phenomenon may best explain its diverse pathology.

A 72-year-old man with a 15-year history of a heart murmur presents to his cardiologist with shortness of breath on exertion over the past 12 months. He otherwise feels well and reports no chest discomfort, palpitations, or swelling of his legs or feet. He is not taking any cardiac drugs, and his health has previously been excellent.

Q: Which of the following findings on 12-lead ECG is not commonly reported in chronic severe aortic regurgitation?

• Left ventricular hypertrophy
• QRS complex left-axis deviation
• A negative U wave
• Atrial fibrillation

A: The correct answer is a negative U wave.

In long-standing left ventricular volume overload, such as in chronic aortic regurgitation, characteristic findings on ECG include lateral precordial narrow Q waves and left ventricular hypertrophy. The ST segment and T wave are often normal or nearly normal. The QRS complex vector may demonstrate left-axis deviation, but this is not absolute. In contrast, pressure overload conditions such as aortic stenosis and systemic hypertension commonly manifest as left ventricular hypertrophy with strain pattern of ST depression in lateral precordial leads and asymmetric T-wave inversion.

A negative U wave, best identified in leads V₄ to V₆, is a common finding in left ventricular volume overload. A negative U wave represents a negative deflection of small amplitude (normally < 0.1 to 3 mV) immediately following the T wave. Although not routinely reported, the negative U wave is an indicator of underlying structural heart disease.¹

Q: A negative U wave has been associated with which of the following conditions?

• Aortic or mitral regurgitation
• Myocardial ischemia
• Hypertension
• All of the above

A: The correct answer is all of the above.

Negative U waves have been identified in regurgitant valvular heart disease with left ventricular volume overload, in myocardial ischemia, ^2,3 and in hypertension.⁴ During exercise stress testing, the transient appearance of negative U waves strongly suggests flow-limiting coronary artery disease. Moreover, changes in the U wave during exercise stress testing may be a sign of well-developed coronary collaterals.⁵ Therefore, it is prudent to note their presence on resting ECG and to investigate further with cardiac stress testing and imaging.

The pathogenesis of the negative U wave remains unclear. Of the various hypotheses put forth, a mechano-electric phenomenon may best explain its diverse pathology.

A 72-year-old man with a 15-year history of a heart murmur presents to his cardiologist with shortness of breath on exertion over the past 12 months. He otherwise feels well and reports no chest discomfort, palpitations, or swelling of his legs or feet. He is not taking any cardiac drugs, and his health has previously been excellent.

Q: Which of the following findings on 12-lead ECG is not commonly reported in chronic severe aortic regurgitation?

• Left ventricular hypertrophy
• QRS complex left-axis deviation
• A negative U wave
• Atrial fibrillation

A: The correct answer is a negative U wave.

In long-standing left ventricular volume overload, such as in chronic aortic regurgitation, characteristic findings on ECG include lateral precordial narrow Q waves and left ventricular hypertrophy. The ST segment and T wave are often normal or nearly normal. The QRS complex vector may demonstrate left-axis deviation, but this is not absolute. In contrast, pressure overload conditions such as aortic stenosis and systemic hypertension commonly manifest as left ventricular hypertrophy with strain pattern of ST depression in lateral precordial leads and asymmetric T-wave inversion.

A negative U wave, best identified in leads V₄ to V₆, is a common finding in left ventricular volume overload. A negative U wave represents a negative deflection of small amplitude (normally < 0.1 to 3 mV) immediately following the T wave. Although not routinely reported, the negative U wave is an indicator of underlying structural heart disease.¹

Q: A negative U wave has been associated with which of the following conditions?

• Aortic or mitral regurgitation
• Myocardial ischemia
• Hypertension
• All of the above

A: The correct answer is all of the above.

Negative U waves have been identified in regurgitant valvular heart disease with left ventricular volume overload, in myocardial ischemia, ^2,3 and in hypertension.⁴ During exercise stress testing, the transient appearance of negative U waves strongly suggests flow-limiting coronary artery disease. Moreover, changes in the U wave during exercise stress testing may be a sign of well-developed coronary collaterals.⁵ Therefore, it is prudent to note their presence on resting ECG and to investigate further with cardiac stress testing and imaging.

The pathogenesis of the negative U wave remains unclear. Of the various hypotheses put forth, a mechano-electric phenomenon may best explain its diverse pathology.

A study of twins finds that shared environmental factors influence the risk of autism more than previously thought and challenges previous findings about the significance of genetics.

Among identical and fraternal twins in whom at least one child has autism or autism spectrum disorder (ASD), shared environmental factors have a more substantial impact regarding development of the condition than do genetics, according to a study in the July 4 online Archives of General Psychiatry.
“A large proportion of the variance in liability can be explained by shared environmental factors (55% for autism and 58% for ASD) in addition to moderate genetic heritability (37% for autism and 38% for ASD),” reported Joachim Hallmayer, MD, Associate Professor of Psychiatry and Behavioral Sciences, Stanford University School of Medicine in Palo Alto, California, and colleagues. “Our study provides evidence that the rate of concordance in dizygotic twins may have been seriously underestimated in previous studies and the influence of genetic factors on the susceptibility to develop autism, overestimated.”
A Shift in the Environment Versus Genetics Debate?
The study included data from monozygotic twin pairs (45 male, nine female) and dizygotic twin pairs (45 male, 13 female, and 80 sex-discordant) who were born between 1987 and 2004. The monozygotic twins were slightly older and had shorter gestation periods. The mothers of the dizygotic twins were also older than the mothers of the monozygotic twins, “consistent with the known increase in dizygotic twinning with maternal age, and more likely to be white and non-Hispanic,” noted the investigators.
For twins with strict autism, the researchers found that the probandwise concordance for male twins was 0.58 for 40 monozygotic pairs and 0.21 for 31 dizygotic pairs; for female twins, concordance was 0.60 for seven monozygotic twin pairs and 0.27 for 10 dizygotic pairs. For children with ASD, the probandwise concordance for male twins was 0.77 for 45 monozygotic pairs and 0.31 for 45 dizygotic pairs; for female twins, concordance was 0.50 for nine monozygotic pairs and 0.36 for 13 dizygotic pairs.
“Because of the reported high heritability of autism, a major focus of research in autism has been on finding the underlying genetic causes, with less emphasis on potential environmental triggers or causes,” Dr. Hallmayer and colleagues wrote. “The finding of significant influence of the shared environment, experiences that are common to both twin individuals, may be important for future research paradigms.”
Increasing evidence has shown that overt symptoms of autism emerge toward the end of the first year of life, the authors noted. “Because the prenatal environment and early postnatal environment are shared between twin individuals, we hypothesize that at least some of the environmental factors impacting susceptibility to autism exert their effect during this critical period of life,” Dr. Hallmayer’s group commented. “Nongenetic risk factors that may index environmental influences included parental age, low birth weight, multiple births, and maternal infections during pregnancy. Future studies that seek to elucidate such factors and their role in enhancing or suppressing genetic susceptibility are likely to enhance our understanding of autism.”
A Disorder of Fetal Programming?
In an accompanying editorial, Peter Szatmari, MD, of the Offord Centre for Child Studies, McMaster University in Hamilton, Ontario, Canada, stated, “Perhaps ASD can be considered, at least in part, a disorder of fetal programming. There is in fact evidence that certain risk factors that affect the maternal fetal environment may place the fetus at increased risk for ASD. Clearly a renewed effort needs to be undertaken through the use of well-designed community-based epidemiologic studies.
“Whatever happens in the future, the finding by Hallmayer and colleagues is an extraordinarily important one and has the potential to shift autism research into a new field of study in much the same way that the original twin study by Folstein and Rutter accomplished back in 1977,” Dr. Szatmari concluded.

A Link Between Maternal Antidepressant Use and Autism Risk in Offspring?
Exposure to selective serotonin reuptake inhibitors (SSRIs) among pregnant women, especially during the first trimester, may modestly increase the risk of autism spectrum disorder (ASD) in their children, according to a study in the July 4 online Archives of General Psychiatry.
The findings were based on 298 children with ASD and 1,507 randomly selected control children and their mothers enrolled in the Kaiser Permanente Medical Care Program in Northern California. Data regarding prenatal exposure to antidepressants were available for 20 children and 50 controls. After adjusted logistic regression, the researchers found a twofold increased risk of ASD associated with SSRI treatment in mothers in the year before delivery (adjusted odds ratio, 2.2). The strongest effect was linked with treatment during the first trimester (adjusted odds ratio, 3.8). No increased risk was observed among mothers with a history of mental health treatment in the absence of prenatal exposure to SSRIs.
“The fraction of cases of ASD that may be attributed to use of antidepressants by the mother during pregnancy is less than 3% in our population, and it is reasonable to conclude that prenatal SSRI exposure is very unlikely to be a major risk factor for ASD,” stated Lisa A. Croen, PhD, of the Division of Research, Kaiser Permanente Northern California in Oakland, and colleagues. “Although these findings indicate that maternal treatment with SSRIs during pregnancy may confer some risk to the fetus with regard to neurodevelopment, this potential risk must be balanced with the risk to the mother or fetus of untreated mental health disorders.”
“Perhaps it is a coincidence that the odds ratio for ASD risk in the study by Croen and colleagues increases when first-trimester exposure to SSRIs is the sole factor,” stated Pat Levitt, PhD, of the Keck School of Medicine, University of Southern California, Los Angeles, in a related commentary. “However, it is exactly that time of human brain development during which cortical and subcortical neuronal populations are being produced, migrating to their final destinations and beginning the long process of wiring. While much occurs later, the establishment of a strong foundation developmentally may be an essential component of healthy brain development.”

A study of twins finds that shared environmental factors influence the risk of autism more than previously thought and challenges previous findings about the significance of genetics.

Among identical and fraternal twins in whom at least one child has autism or autism spectrum disorder (ASD), shared environmental factors have a more substantial impact regarding development of the condition than do genetics, according to a study in the July 4 online Archives of General Psychiatry.
“A large proportion of the variance in liability can be explained by shared environmental factors (55% for autism and 58% for ASD) in addition to moderate genetic heritability (37% for autism and 38% for ASD),” reported Joachim Hallmayer, MD, Associate Professor of Psychiatry and Behavioral Sciences, Stanford University School of Medicine in Palo Alto, California, and colleagues. “Our study provides evidence that the rate of concordance in dizygotic twins may have been seriously underestimated in previous studies and the influence of genetic factors on the susceptibility to develop autism, overestimated.”
A Shift in the Environment Versus Genetics Debate?
The study included data from monozygotic twin pairs (45 male, nine female) and dizygotic twin pairs (45 male, 13 female, and 80 sex-discordant) who were born between 1987 and 2004. The monozygotic twins were slightly older and had shorter gestation periods. The mothers of the dizygotic twins were also older than the mothers of the monozygotic twins, “consistent with the known increase in dizygotic twinning with maternal age, and more likely to be white and non-Hispanic,” noted the investigators.
For twins with strict autism, the researchers found that the probandwise concordance for male twins was 0.58 for 40 monozygotic pairs and 0.21 for 31 dizygotic pairs; for female twins, concordance was 0.60 for seven monozygotic twin pairs and 0.27 for 10 dizygotic pairs. For children with ASD, the probandwise concordance for male twins was 0.77 for 45 monozygotic pairs and 0.31 for 45 dizygotic pairs; for female twins, concordance was 0.50 for nine monozygotic pairs and 0.36 for 13 dizygotic pairs.
“Because of the reported high heritability of autism, a major focus of research in autism has been on finding the underlying genetic causes, with less emphasis on potential environmental triggers or causes,” Dr. Hallmayer and colleagues wrote. “The finding of significant influence of the shared environment, experiences that are common to both twin individuals, may be important for future research paradigms.”
Increasing evidence has shown that overt symptoms of autism emerge toward the end of the first year of life, the authors noted. “Because the prenatal environment and early postnatal environment are shared between twin individuals, we hypothesize that at least some of the environmental factors impacting susceptibility to autism exert their effect during this critical period of life,” Dr. Hallmayer’s group commented. “Nongenetic risk factors that may index environmental influences included parental age, low birth weight, multiple births, and maternal infections during pregnancy. Future studies that seek to elucidate such factors and their role in enhancing or suppressing genetic susceptibility are likely to enhance our understanding of autism.”
A Disorder of Fetal Programming?
In an accompanying editorial, Peter Szatmari, MD, of the Offord Centre for Child Studies, McMaster University in Hamilton, Ontario, Canada, stated, “Perhaps ASD can be considered, at least in part, a disorder of fetal programming. There is in fact evidence that certain risk factors that affect the maternal fetal environment may place the fetus at increased risk for ASD. Clearly a renewed effort needs to be undertaken through the use of well-designed community-based epidemiologic studies.
“Whatever happens in the future, the finding by Hallmayer and colleagues is an extraordinarily important one and has the potential to shift autism research into a new field of study in much the same way that the original twin study by Folstein and Rutter accomplished back in 1977,” Dr. Szatmari concluded.

A Link Between Maternal Antidepressant Use and Autism Risk in Offspring?
Exposure to selective serotonin reuptake inhibitors (SSRIs) among pregnant women, especially during the first trimester, may modestly increase the risk of autism spectrum disorder (ASD) in their children, according to a study in the July 4 online Archives of General Psychiatry.
The findings were based on 298 children with ASD and 1,507 randomly selected control children and their mothers enrolled in the Kaiser Permanente Medical Care Program in Northern California. Data regarding prenatal exposure to antidepressants were available for 20 children and 50 controls. After adjusted logistic regression, the researchers found a twofold increased risk of ASD associated with SSRI treatment in mothers in the year before delivery (adjusted odds ratio, 2.2). The strongest effect was linked with treatment during the first trimester (adjusted odds ratio, 3.8). No increased risk was observed among mothers with a history of mental health treatment in the absence of prenatal exposure to SSRIs.
“The fraction of cases of ASD that may be attributed to use of antidepressants by the mother during pregnancy is less than 3% in our population, and it is reasonable to conclude that prenatal SSRI exposure is very unlikely to be a major risk factor for ASD,” stated Lisa A. Croen, PhD, of the Division of Research, Kaiser Permanente Northern California in Oakland, and colleagues. “Although these findings indicate that maternal treatment with SSRIs during pregnancy may confer some risk to the fetus with regard to neurodevelopment, this potential risk must be balanced with the risk to the mother or fetus of untreated mental health disorders.”
“Perhaps it is a coincidence that the odds ratio for ASD risk in the study by Croen and colleagues increases when first-trimester exposure to SSRIs is the sole factor,” stated Pat Levitt, PhD, of the Keck School of Medicine, University of Southern California, Los Angeles, in a related commentary. “However, it is exactly that time of human brain development during which cortical and subcortical neuronal populations are being produced, migrating to their final destinations and beginning the long process of wiring. While much occurs later, the establishment of a strong foundation developmentally may be an essential component of healthy brain development.”

A study of twins finds that shared environmental factors influence the risk of autism more than previously thought and challenges previous findings about the significance of genetics.

Among identical and fraternal twins in whom at least one child has autism or autism spectrum disorder (ASD), shared environmental factors have a more substantial impact regarding development of the condition than do genetics, according to a study in the July 4 online Archives of General Psychiatry.
“A large proportion of the variance in liability can be explained by shared environmental factors (55% for autism and 58% for ASD) in addition to moderate genetic heritability (37% for autism and 38% for ASD),” reported Joachim Hallmayer, MD, Associate Professor of Psychiatry and Behavioral Sciences, Stanford University School of Medicine in Palo Alto, California, and colleagues. “Our study provides evidence that the rate of concordance in dizygotic twins may have been seriously underestimated in previous studies and the influence of genetic factors on the susceptibility to develop autism, overestimated.”
A Shift in the Environment Versus Genetics Debate?
The study included data from monozygotic twin pairs (45 male, nine female) and dizygotic twin pairs (45 male, 13 female, and 80 sex-discordant) who were born between 1987 and 2004. The monozygotic twins were slightly older and had shorter gestation periods. The mothers of the dizygotic twins were also older than the mothers of the monozygotic twins, “consistent with the known increase in dizygotic twinning with maternal age, and more likely to be white and non-Hispanic,” noted the investigators.
For twins with strict autism, the researchers found that the probandwise concordance for male twins was 0.58 for 40 monozygotic pairs and 0.21 for 31 dizygotic pairs; for female twins, concordance was 0.60 for seven monozygotic twin pairs and 0.27 for 10 dizygotic pairs. For children with ASD, the probandwise concordance for male twins was 0.77 for 45 monozygotic pairs and 0.31 for 45 dizygotic pairs; for female twins, concordance was 0.50 for nine monozygotic pairs and 0.36 for 13 dizygotic pairs.
“Because of the reported high heritability of autism, a major focus of research in autism has been on finding the underlying genetic causes, with less emphasis on potential environmental triggers or causes,” Dr. Hallmayer and colleagues wrote. “The finding of significant influence of the shared environment, experiences that are common to both twin individuals, may be important for future research paradigms.”
Increasing evidence has shown that overt symptoms of autism emerge toward the end of the first year of life, the authors noted. “Because the prenatal environment and early postnatal environment are shared between twin individuals, we hypothesize that at least some of the environmental factors impacting susceptibility to autism exert their effect during this critical period of life,” Dr. Hallmayer’s group commented. “Nongenetic risk factors that may index environmental influences included parental age, low birth weight, multiple births, and maternal infections during pregnancy. Future studies that seek to elucidate such factors and their role in enhancing or suppressing genetic susceptibility are likely to enhance our understanding of autism.”
A Disorder of Fetal Programming?
In an accompanying editorial, Peter Szatmari, MD, of the Offord Centre for Child Studies, McMaster University in Hamilton, Ontario, Canada, stated, “Perhaps ASD can be considered, at least in part, a disorder of fetal programming. There is in fact evidence that certain risk factors that affect the maternal fetal environment may place the fetus at increased risk for ASD. Clearly a renewed effort needs to be undertaken through the use of well-designed community-based epidemiologic studies.
“Whatever happens in the future, the finding by Hallmayer and colleagues is an extraordinarily important one and has the potential to shift autism research into a new field of study in much the same way that the original twin study by Folstein and Rutter accomplished back in 1977,” Dr. Szatmari concluded.

A Link Between Maternal Antidepressant Use and Autism Risk in Offspring?
Exposure to selective serotonin reuptake inhibitors (SSRIs) among pregnant women, especially during the first trimester, may modestly increase the risk of autism spectrum disorder (ASD) in their children, according to a study in the July 4 online Archives of General Psychiatry.
The findings were based on 298 children with ASD and 1,507 randomly selected control children and their mothers enrolled in the Kaiser Permanente Medical Care Program in Northern California. Data regarding prenatal exposure to antidepressants were available for 20 children and 50 controls. After adjusted logistic regression, the researchers found a twofold increased risk of ASD associated with SSRI treatment in mothers in the year before delivery (adjusted odds ratio, 2.2). The strongest effect was linked with treatment during the first trimester (adjusted odds ratio, 3.8). No increased risk was observed among mothers with a history of mental health treatment in the absence of prenatal exposure to SSRIs.
“The fraction of cases of ASD that may be attributed to use of antidepressants by the mother during pregnancy is less than 3% in our population, and it is reasonable to conclude that prenatal SSRI exposure is very unlikely to be a major risk factor for ASD,” stated Lisa A. Croen, PhD, of the Division of Research, Kaiser Permanente Northern California in Oakland, and colleagues. “Although these findings indicate that maternal treatment with SSRIs during pregnancy may confer some risk to the fetus with regard to neurodevelopment, this potential risk must be balanced with the risk to the mother or fetus of untreated mental health disorders.”
“Perhaps it is a coincidence that the odds ratio for ASD risk in the study by Croen and colleagues increases when first-trimester exposure to SSRIs is the sole factor,” stated Pat Levitt, PhD, of the Keck School of Medicine, University of Southern California, Los Angeles, in a related commentary. “However, it is exactly that time of human brain development during which cortical and subcortical neuronal populations are being produced, migrating to their final destinations and beginning the long process of wiring. While much occurs later, the establishment of a strong foundation developmentally may be an essential component of healthy brain development.”

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.

DISCUSSION
Ramsay Hunt syndrome (RHS, also known as geniculate herpes^5,6) 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

Diagnosis
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 auricle^6,14), 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

Treatment
Data used to support the use of corticosteroids for treatment of Bell’s palsy^10,21,22 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 replication^9,23 (valacyclovir and famciclovir have also been mentioned^12,18). 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.     

REFERENCES
1. Kim D, Bhimani M. Ramsay Hunt syndrome presenting as simple otitis externa. CJEM. 2008;10(3):247-250.

2. Agius AM, Pickles JM, Burch KL. A prospective study of otitis externa. Clin Otolaryngol. 1992;17(2):150-154.

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.

4. Holland J, Bernstein J. Bell’s palsy. Clin Evid (Online). 2011 Mar 7;2011.pii:1204.

5. Jacewicz M. Bell’s palsy (2007). www.merckmanuals.com/professional/sec16/ch219/ch219i.html. Accessed May 26, 2011.

6. Harrison K. Discussion: the Ramsay Hunt Syndrome. Proc Royal Soc Med. 1953;47(371):11-24.

7. Bhupal HK. Ramsay Hunt syndrome presenting in primary care. Practitioner. 2010;254(1727):33-35.

8. Aizawa H, Ohtani F, Furuta Y, et al. Variable patterns of varicella-zoster virus reactivation in Ramsay Hunt syndrome. J Med Virol. 2004;74(2):355-360.

9. Gondivkar S, Parikh V, Parikh R. Herpes zoster oticus: a rare clinical entity. Contemp Clin Dent. 2010;1(2):127-129.

10. Sullivan FM, Swan IRC, Donnan PT, et al. Early treatment with prednisolone or acyclovir in Bell’s palsy. N Engl J Med. 2007;357(16):1598-1607.

11. Gilden DH. Bell’s palsy. N Engl J Med. 2004;351(13):1323-1331.

12. Diaz GA, Rakita RM, Koelle DM. A case of Ramsay Hunt–like syndrome caused by herpes simplex virus type 2. Clin Infect Dis. 2005;40(10):1545-1547.

13. Miravalle AA. Ramsay Hunt syndrome. http://emedicine.medscape.com/article/1166804-over iew. Accessed July 22, 2011.

14. Hunt JR. On herpetic inflammation of the geniculate ganglion: a new syndrome and its complications. J Nerv Ment Dis. 1907;34:73-96.

15. Sandoval CC, Núñez FA, Lizama CM, et al. Ramsay Hunt syndrome in children: four cases and review [in Spanish]. Rev Chilena Infectol. 2008; 25(6):458-464.

16. Murakami S, Nakashiro Y, Mizobuchi M, et al. Varicella-zoster virus distribution in Ramsay Hunt syndrome revealed by polymerase chain reaction. Acta Otolaryngol. 1998;118(2):145-149.

17. Hiroshige K, Ikeda M, Hondo R. Detection of varicella zoster virus DNA in tear fluid and saliva of patients with Ramsay Hunt syndrome. Otol Neurol. 2002;23(4):602-607.

18. Sweeney CJ, Gilden DH. Ramsay Hunt syndrome. J Neurol Neurosurg Psychiatr. 2001;71(2):148-154.

19. Murakami S, Honda N, Mizobuchi M, et al. Rapid diagnosis of varicella zoster virus infection in acute facial palsy. Neurology. 1998;51(4):1202-1205.

20. Kim IS, Shin SH, Kim J, et al. Correlation between MRI and operative findings in Bell’s palsy and Ramsay Hunt syndrome. Yonsei Med J. 2007;48(6):963-968.

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.

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.

DISCUSSION
Ramsay Hunt syndrome (RHS, also known as geniculate herpes^5,6) 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

Diagnosis
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 auricle^6,14), 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

Treatment
Data used to support the use of corticosteroids for treatment of Bell’s palsy^10,21,22 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 replication^9,23 (valacyclovir and famciclovir have also been mentioned^12,18). 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.     

REFERENCES
1. Kim D, Bhimani M. Ramsay Hunt syndrome presenting as simple otitis externa. CJEM. 2008;10(3):247-250.

2. Agius AM, Pickles JM, Burch KL. A prospective study of otitis externa. Clin Otolaryngol. 1992;17(2):150-154.

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.

4. Holland J, Bernstein J. Bell’s palsy. Clin Evid (Online). 2011 Mar 7;2011.pii:1204.

5. Jacewicz M. Bell’s palsy (2007). www.merckmanuals.com/professional/sec16/ch219/ch219i.html. Accessed May 26, 2011.

6. Harrison K. Discussion: the Ramsay Hunt Syndrome. Proc Royal Soc Med. 1953;47(371):11-24.

7. Bhupal HK. Ramsay Hunt syndrome presenting in primary care. Practitioner. 2010;254(1727):33-35.

8. Aizawa H, Ohtani F, Furuta Y, et al. Variable patterns of varicella-zoster virus reactivation in Ramsay Hunt syndrome. J Med Virol. 2004;74(2):355-360.

9. Gondivkar S, Parikh V, Parikh R. Herpes zoster oticus: a rare clinical entity. Contemp Clin Dent. 2010;1(2):127-129.

10. Sullivan FM, Swan IRC, Donnan PT, et al. Early treatment with prednisolone or acyclovir in Bell’s palsy. N Engl J Med. 2007;357(16):1598-1607.

11. Gilden DH. Bell’s palsy. N Engl J Med. 2004;351(13):1323-1331.

12. Diaz GA, Rakita RM, Koelle DM. A case of Ramsay Hunt–like syndrome caused by herpes simplex virus type 2. Clin Infect Dis. 2005;40(10):1545-1547.

13. Miravalle AA. Ramsay Hunt syndrome. http://emedicine.medscape.com/article/1166804-over iew. Accessed July 22, 2011.

14. Hunt JR. On herpetic inflammation of the geniculate ganglion: a new syndrome and its complications. J Nerv Ment Dis. 1907;34:73-96.

15. Sandoval CC, Núñez FA, Lizama CM, et al. Ramsay Hunt syndrome in children: four cases and review [in Spanish]. Rev Chilena Infectol. 2008; 25(6):458-464.

16. Murakami S, Nakashiro Y, Mizobuchi M, et al. Varicella-zoster virus distribution in Ramsay Hunt syndrome revealed by polymerase chain reaction. Acta Otolaryngol. 1998;118(2):145-149.

17. Hiroshige K, Ikeda M, Hondo R. Detection of varicella zoster virus DNA in tear fluid and saliva of patients with Ramsay Hunt syndrome. Otol Neurol. 2002;23(4):602-607.

18. Sweeney CJ, Gilden DH. Ramsay Hunt syndrome. J Neurol Neurosurg Psychiatr. 2001;71(2):148-154.

19. Murakami S, Honda N, Mizobuchi M, et al. Rapid diagnosis of varicella zoster virus infection in acute facial palsy. Neurology. 1998;51(4):1202-1205.

20. Kim IS, Shin SH, Kim J, et al. Correlation between MRI and operative findings in Bell’s palsy and Ramsay Hunt syndrome. Yonsei Med J. 2007;48(6):963-968.

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.

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.

DISCUSSION
Ramsay Hunt syndrome (RHS, also known as geniculate herpes^5,6) 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

Diagnosis
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 auricle^6,14), 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

Treatment
Data used to support the use of corticosteroids for treatment of Bell’s palsy^10,21,22 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 replication^9,23 (valacyclovir and famciclovir have also been mentioned^12,18). 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.     

REFERENCES
1. Kim D, Bhimani M. Ramsay Hunt syndrome presenting as simple otitis externa. CJEM. 2008;10(3):247-250.

2. Agius AM, Pickles JM, Burch KL. A prospective study of otitis externa. Clin Otolaryngol. 1992;17(2):150-154.

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.

4. Holland J, Bernstein J. Bell’s palsy. Clin Evid (Online). 2011 Mar 7;2011.pii:1204.

5. Jacewicz M. Bell’s palsy (2007). www.merckmanuals.com/professional/sec16/ch219/ch219i.html. Accessed May 26, 2011.

6. Harrison K. Discussion: the Ramsay Hunt Syndrome. Proc Royal Soc Med. 1953;47(371):11-24.

7. Bhupal HK. Ramsay Hunt syndrome presenting in primary care. Practitioner. 2010;254(1727):33-35.

8. Aizawa H, Ohtani F, Furuta Y, et al. Variable patterns of varicella-zoster virus reactivation in Ramsay Hunt syndrome. J Med Virol. 2004;74(2):355-360.

9. Gondivkar S, Parikh V, Parikh R. Herpes zoster oticus: a rare clinical entity. Contemp Clin Dent. 2010;1(2):127-129.

10. Sullivan FM, Swan IRC, Donnan PT, et al. Early treatment with prednisolone or acyclovir in Bell’s palsy. N Engl J Med. 2007;357(16):1598-1607.

11. Gilden DH. Bell’s palsy. N Engl J Med. 2004;351(13):1323-1331.

12. Diaz GA, Rakita RM, Koelle DM. A case of Ramsay Hunt–like syndrome caused by herpes simplex virus type 2. Clin Infect Dis. 2005;40(10):1545-1547.

13. Miravalle AA. Ramsay Hunt syndrome. http://emedicine.medscape.com/article/1166804-over iew. Accessed July 22, 2011.

14. Hunt JR. On herpetic inflammation of the geniculate ganglion: a new syndrome and its complications. J Nerv Ment Dis. 1907;34:73-96.

15. Sandoval CC, Núñez FA, Lizama CM, et al. Ramsay Hunt syndrome in children: four cases and review [in Spanish]. Rev Chilena Infectol. 2008; 25(6):458-464.

16. Murakami S, Nakashiro Y, Mizobuchi M, et al. Varicella-zoster virus distribution in Ramsay Hunt syndrome revealed by polymerase chain reaction. Acta Otolaryngol. 1998;118(2):145-149.

17. Hiroshige K, Ikeda M, Hondo R. Detection of varicella zoster virus DNA in tear fluid and saliva of patients with Ramsay Hunt syndrome. Otol Neurol. 2002;23(4):602-607.

18. Sweeney CJ, Gilden DH. Ramsay Hunt syndrome. J Neurol Neurosurg Psychiatr. 2001;71(2):148-154.

19. Murakami S, Honda N, Mizobuchi M, et al. Rapid diagnosis of varicella zoster virus infection in acute facial palsy. Neurology. 1998;51(4):1202-1205.

20. Kim IS, Shin SH, Kim J, et al. Correlation between MRI and operative findings in Bell’s palsy and Ramsay Hunt syndrome. Yonsei Med J. 2007;48(6):963-968.

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.