Ramsay-Hunt syndrome in a patient with HIV infection JOSEPH H. MISHELL, MD, and EDWARD L. APPLEBAUM, MD, Chicago, Illinois
T h e acquired immune deficiency syndrome (AIDS) was first described in 1981because of cases of impaired cell-mediated immunity, opportunistic infections, and malignancies in homosexual males. I Currently, it is estimated that 1 to 1.5 million persons are infected with the AIDS virus in the United States, and 40,051 cases that fulfill the Center for Disease Control (CDC) case definition for AIDS have been reported.' While tremendous knowledge has been gained about the immunology, virology, and pathophysiology of the disease, measures for treatment, and prevention remain elusive. As the incidence of AIDS increases, more patients with the syndrome will be encountered by otolaryngologists. In a study by Marcusen and Sooy,' 399 cases of AIDS were reviewed with respect to their head and neck manifestations. Forty-one percent of the patients reported symptoms referable to the head and neck. Kaposi's sarcoma (both mucosal and cutaneous), candidiasis, and chronic cough were the most common findings. Herpes zoster infections have been long-recognized as occurring more frequently in immunocompromised hosts, and they have also been seen frequently in patients with AIDS.4 Herpes zoster has been shown in immunologic and biologic studies to be indistinguishable from varicella or the chickenpox virus.5 It is believed that the chickenpox virus remains dormant in neural tissue after primary infection. A patient's immunocompromised state allows the virus to escape normal, immune surveillance, and it then causes secondary infection. Zoster most commonly involves the trunk in a dennatomal distribution consistent with neurogenic spread (i.e., shingle^).^ Herpes zoster oticus is a form of head and neck zoster that is associated with a motor neuropathy-namely,
From the Department of Otolaryngology-Head and Neck Surgery, University of Illinois College of Medicine at Chicago. Submitted for publication Jan. 14, 1989; revision received July 17, 1989; accepted July 19, 1989. Reprint requests: Joseph H. Mishell, MD, Department of Otolaryngology-Head and Neck Surgery, University of Illinois Eye and Ear Infirmary, 1855 West Taylor St., Chicago, IL 60612. 23/41 15320
facial nerve palsy. The classic syndrome involves the triad of ear pain, facial paralysis, and a varicelliform rash of the auricle and external auditory canal. Taste, lacrimation, and hearing are affected variably.6 We report a patient with HIV infection who manifested herpes zoster oticus. CASE REPORT
A 29-year-old man was transferred to the University of Illinois Eye and Ear Infirmary with a 1-week history of left ear pain and swelling, fever, and sore throat. Associated with the pain was an ipsilateral hearing loss. The patient had been treated with oral antibiotics by his private physician, but his symptoms progressed. He was hospitalized elsewhere for intravenous antibiotic therapy, but he failed to improve. On the day of transfer, a left facial palsy and a left facial and body rash developed. Excision of a benign lymph node from his neck had been performed 2 months before his admission. He was homosexual and had had multiple partners. There was no history of intravenous drug abuse or blood transfusions. On admssion, his oral temperature was 100.6" F (38.1' C). The left auricle was markedly erythematous, edematous, and indurated with an area of ulceration along the concha (Fig. 1). The external auditory canal was also edematous, and the tympanic membrane was only partially visible. There were vesicles along the left lateral tongue border, and soft palate and ipsilateral, tender adenopathy. No other lymph nodes or masses were found. The skin had a maculopapular rash over the left side of the face, neck, and back, but these lesions were not tender. No skin vesicles were noted at this time. There was a complete seventh lower motor neuron paralysis and first-degree right nystagmus. White blood cell count was 3.5 X 103/mm3with 39% lymphs. The platelet count was also mildly depressed at 141,000/mm3. An audiogram showed normal hearing in the right ear, except for a moderate sensorineural loss at 8 kHz. The left ear, however, had a moderate sensorineural hearing loss, from 250 Hz to 2000 Hz, which then dropped to a profound loss at higher frequencies. Discrimination scores were 100% for the right ear and 12% for the left ear. Acoustic reflexes were consistent with a left seventh nerve paralysis (Fig. 2). Serologic tests for HIV were positive. Cultures of the blood, skin lesions, and auricular exudate that were sent for bacterial and viral study demonstrated no pathogens. After 7 days of intravenous nafcillin and acyclovir administration, the ear swelling, induration, and pain resolved, and the skin lesions crusted over and healed. 177
Downloaded from oto.sagepub.com at UNIV NEBRASKA LIBRARIES on June 5, 2016
OtolaryngologyHead and Neck Surgery
178 CaseRepotts
t
I
1 SRT 1 DISC1 I
I
Fig. 1. Leftauricle with edema, erythema, and ulcerationalong the concha, and diffuse serous discharge.
After discharge from the hospital, he began to experience weight loss and night sweats. Lymphocyte studies disclosed lymphopenia with a T-helper/T-suppressorratio of 1.6. He was lost to followup weeks after the onset of his symptoms. There had been no return of facial or auditory function when he was last seen.
DISCUSSION
The syndrome of facial nerve palsy and inner ear symptoms associated with herpes zoster involvement of the auricle or external canal is known as the RamsayHunt syndrome. Although J. Ramsay Hunt was not the first person to describe this syndrome, it was named for him because of his intensive investigations and theories concerning the disease. He first reported herpes zoster oticus in 1907.6.7 His concept was that zoster involvement of the geniculate ganglion was responsible for the The typical clinical course is characterized by pain, followed by a vesicular rash and facial palsy. The rash is usually over the auricle or the external auditory canal or both. Different sites, however, on the face, oral cavity, and neck have been associated with the syndrome." Auditory and vestibular disturbances are also encountered frequently, with ves-
Fig. 2. Audiogram obtained during patient's hospitalization. A, Right ear has a mild sensorineural loss at 8 Hz.Left ear has a moderate sensorineural loss from 250 Hz to 2 Wz and a profound loss in the higher frequencies. 8, Discrimination scores are poor in the left ear.
tibular disturbances pred~minating.~.'~ Facial nerve palsy is more often complete, compared to idiopathic Bell's palsy, with a resulting worse prognosis for recovery of function.6." Most investigators consider herpes zoster oticus a polycranial neuropathy.8.10Support for this contention is the high incidence of multiple cranial nerve palsies, as well as various dermatomal distributions of the rash. The patient had demonstrated vesicles on the tongue, soft palate, and auricle, as well as hearing loss, nystagmus, and a complete facial nerve paralysis. These findings indicate polycranial involvement by the virus. Additionally, the disease became disseminated over his entire body. Cutaneous dissemination occurs in about 2% of patients with zoster, typically in immunocompromised h o s t ~ In . ~ keeping with the severity of his illness, he did not demonstrate any return of facial or auditory function.
Downloaded from oto.sagepub.com at UNIV NEBRASKA LIBRARIES on June 5, 2016
Volume 102 Number 2 February 1990
Case Reports 179
Several different classifications have been proposed for the spectrum of disease that is caused by HIV infection.’.I2 The newer CDC classification divides HIV infection into four distinct subgroups: Croup I-acute infection; Group 11-asymptomatic infection; Group III- persistent generalized lymphadenopathy; and Group ZV-other disease. Group IV includes patients with constitutional symptoms, neurologic disease, and secondary infectious disease and malignancies. An older classification still uses the terms “full-blown AIDS” and “AIDS-related complex”, or ARC (a constellation of findings in persons who are HIV-positive but not fulfilling the criteria for AIDS). The signs and symptoms include weight loss, generalized lymphadenopathy, fever, chronic diarrhea, and lymphopenia. Although our patient did not demonstrate the typical reversal of T-helper to T-suppressor cells seen in the full-blown syndrome, he demonstrated sufficient findings to be classified in the ARC category. Further followup would be required to determine the development of AIDS. Increasing reports appear in the otolaryngologic literature regarding unusual conditions seen in HIVpositive patients. Candida supraglottitis, pneumocystis of the temporal bone, and aphthous ulcers of the pharynx and esophagus are among the diseases reported. I3-l5 Otolaryngologists must consider the diagnosis of HIV infection when confronted with an unusual or atypical disease presentation in the head and neck, especially in high-risk patients. Specifically, when evaluating a patient with Ramsay-Hunt syndrome and disseminated herpes zoster, HIV infection should be investigated. REFERENCES
2. Centers for Disease Control. Human immunodeficiency virus infection in the United States: A review of current knowledge. MMWR 1987;36:Supplement 6. 3. Marcusen DC, Sooy CD. Otolaryngologic and head and neck manifestations of acquired immunodeficiency syndrome. Laryngoscope 1985;95:401-5. 4. Fauci AS, Masur H, Gelmann EP, Markham PD, Hahn BH, Lane HC. The acquired immunodeficiency syndrome: An update. Ann Intern Med 1985;102:800-13. 5. Dolin R, Reichrnan RC, Mazur MH, Whitley RJ. Herpes zoster-varicella infections in immunocompromised patients. Ann Intern Med 1978;89:375-88. 6. Crabtree JA. Herpes zoster oticus. Laryngoscope 1968;78:185378. 7. Hunt JR. Herpetic inflammations of the geniculate ganglion: a new syndrome and its complications J Nerv Ment Dis 1907; 34:73. 8. Payton RJ, Dawes JDK. Herpes zoster of the head and neck. J Laryngol Otol 1972;86:1031-55. 9. By1 FN, Adour KR. Auditory symptoms associated with herpes zoster or idiopathic facial paralysis. Laryngoscope 1977;87: 372-9. 10. Aviel A, Marshak G . Ramsay-Hunt syndrome: a cranial polyneuropathy. Am J Otolaryngol 1982;3:61-6. 11. Robillard RB , Hilsinger RL, Adour KR , Ramsay-Hunt paralysis: clinical analysis of 185 patients. OTOLARYNGOL HEADNECKSURG 1986;95:292-7. 12. Centers for Disease Control. Classification systems for human T-lyrnphotropic virus type III/lymphadenopathy-associated virus infections. MMWR 1986;35:20 13 Bye MR, Palomba A, Bernstein L, Shah K. Clinical candida supraglottitis in an infant with AIDS-related complex. Pediatr Pulmonol 1987;3(4):280-1. 14 Breda SD, Harnmerschlag PE, Gigliotti F, Schinella K. Pneumocystis carinii in the temporal bone as a primary manifestation of the acquired immunodeficiency syndrome. Ann Otol Rhino1 Larvneol 1988;97:427-31. 15. Back MC, Valenti AJ, Howell DA, Smith TJ. Odynophagia from apthous ulcers of the pharynx and esophagus in the acquired immunodeficiency syndrome (AIDS). Ann Intern Med 1988; 109(4):338-9. _
I
I . Centers for Disease Control. Kaposi’s sarcoma and pneumocystis pneumonia among homosexual men: New York City and California. MMWR 1981;25:305-8.
Downloaded from oto.sagepub.com at UNIV NEBRASKA LIBRARIES on June 5, 2016
T h e acquired immune deficiency syndrome (AIDS) was first described in 1981because of cases of impaired cell-mediated immunity, opportunistic infections, and malignancies in homosexual males. I Currently, it is estimated that 1 to 1.5 million persons are infected with the AIDS virus in the United States, and 40,051 cases that fulfill the Center for Disease Control (CDC) case definition for AIDS have been reported.' While tremendous knowledge has been gained about the immunology, virology, and pathophysiology of the disease, measures for treatment, and prevention remain elusive. As the incidence of AIDS increases, more patients with the syndrome will be encountered by otolaryngologists. In a study by Marcusen and Sooy,' 399 cases of AIDS were reviewed with respect to their head and neck manifestations. Forty-one percent of the patients reported symptoms referable to the head and neck. Kaposi's sarcoma (both mucosal and cutaneous), candidiasis, and chronic cough were the most common findings. Herpes zoster infections have been long-recognized as occurring more frequently in immunocompromised hosts, and they have also been seen frequently in patients with AIDS.4 Herpes zoster has been shown in immunologic and biologic studies to be indistinguishable from varicella or the chickenpox virus.5 It is believed that the chickenpox virus remains dormant in neural tissue after primary infection. A patient's immunocompromised state allows the virus to escape normal, immune surveillance, and it then causes secondary infection. Zoster most commonly involves the trunk in a dennatomal distribution consistent with neurogenic spread (i.e., shingle^).^ Herpes zoster oticus is a form of head and neck zoster that is associated with a motor neuropathy-namely,
From the Department of Otolaryngology-Head and Neck Surgery, University of Illinois College of Medicine at Chicago. Submitted for publication Jan. 14, 1989; revision received July 17, 1989; accepted July 19, 1989. Reprint requests: Joseph H. Mishell, MD, Department of Otolaryngology-Head and Neck Surgery, University of Illinois Eye and Ear Infirmary, 1855 West Taylor St., Chicago, IL 60612. 23/41 15320
facial nerve palsy. The classic syndrome involves the triad of ear pain, facial paralysis, and a varicelliform rash of the auricle and external auditory canal. Taste, lacrimation, and hearing are affected variably.6 We report a patient with HIV infection who manifested herpes zoster oticus. CASE REPORT
A 29-year-old man was transferred to the University of Illinois Eye and Ear Infirmary with a 1-week history of left ear pain and swelling, fever, and sore throat. Associated with the pain was an ipsilateral hearing loss. The patient had been treated with oral antibiotics by his private physician, but his symptoms progressed. He was hospitalized elsewhere for intravenous antibiotic therapy, but he failed to improve. On the day of transfer, a left facial palsy and a left facial and body rash developed. Excision of a benign lymph node from his neck had been performed 2 months before his admission. He was homosexual and had had multiple partners. There was no history of intravenous drug abuse or blood transfusions. On admssion, his oral temperature was 100.6" F (38.1' C). The left auricle was markedly erythematous, edematous, and indurated with an area of ulceration along the concha (Fig. 1). The external auditory canal was also edematous, and the tympanic membrane was only partially visible. There were vesicles along the left lateral tongue border, and soft palate and ipsilateral, tender adenopathy. No other lymph nodes or masses were found. The skin had a maculopapular rash over the left side of the face, neck, and back, but these lesions were not tender. No skin vesicles were noted at this time. There was a complete seventh lower motor neuron paralysis and first-degree right nystagmus. White blood cell count was 3.5 X 103/mm3with 39% lymphs. The platelet count was also mildly depressed at 141,000/mm3. An audiogram showed normal hearing in the right ear, except for a moderate sensorineural loss at 8 kHz. The left ear, however, had a moderate sensorineural hearing loss, from 250 Hz to 2000 Hz, which then dropped to a profound loss at higher frequencies. Discrimination scores were 100% for the right ear and 12% for the left ear. Acoustic reflexes were consistent with a left seventh nerve paralysis (Fig. 2). Serologic tests for HIV were positive. Cultures of the blood, skin lesions, and auricular exudate that were sent for bacterial and viral study demonstrated no pathogens. After 7 days of intravenous nafcillin and acyclovir administration, the ear swelling, induration, and pain resolved, and the skin lesions crusted over and healed. 177
Downloaded from oto.sagepub.com at UNIV NEBRASKA LIBRARIES on June 5, 2016
OtolaryngologyHead and Neck Surgery
178 CaseRepotts
t
I
1 SRT 1 DISC1 I
I
Fig. 1. Leftauricle with edema, erythema, and ulcerationalong the concha, and diffuse serous discharge.
After discharge from the hospital, he began to experience weight loss and night sweats. Lymphocyte studies disclosed lymphopenia with a T-helper/T-suppressorratio of 1.6. He was lost to followup weeks after the onset of his symptoms. There had been no return of facial or auditory function when he was last seen.
DISCUSSION
The syndrome of facial nerve palsy and inner ear symptoms associated with herpes zoster involvement of the auricle or external canal is known as the RamsayHunt syndrome. Although J. Ramsay Hunt was not the first person to describe this syndrome, it was named for him because of his intensive investigations and theories concerning the disease. He first reported herpes zoster oticus in 1907.6.7 His concept was that zoster involvement of the geniculate ganglion was responsible for the The typical clinical course is characterized by pain, followed by a vesicular rash and facial palsy. The rash is usually over the auricle or the external auditory canal or both. Different sites, however, on the face, oral cavity, and neck have been associated with the syndrome." Auditory and vestibular disturbances are also encountered frequently, with ves-
Fig. 2. Audiogram obtained during patient's hospitalization. A, Right ear has a mild sensorineural loss at 8 Hz.Left ear has a moderate sensorineural loss from 250 Hz to 2 Wz and a profound loss in the higher frequencies. 8, Discrimination scores are poor in the left ear.
tibular disturbances pred~minating.~.'~ Facial nerve palsy is more often complete, compared to idiopathic Bell's palsy, with a resulting worse prognosis for recovery of function.6." Most investigators consider herpes zoster oticus a polycranial neuropathy.8.10Support for this contention is the high incidence of multiple cranial nerve palsies, as well as various dermatomal distributions of the rash. The patient had demonstrated vesicles on the tongue, soft palate, and auricle, as well as hearing loss, nystagmus, and a complete facial nerve paralysis. These findings indicate polycranial involvement by the virus. Additionally, the disease became disseminated over his entire body. Cutaneous dissemination occurs in about 2% of patients with zoster, typically in immunocompromised h o s t ~ In . ~ keeping with the severity of his illness, he did not demonstrate any return of facial or auditory function.
Downloaded from oto.sagepub.com at UNIV NEBRASKA LIBRARIES on June 5, 2016
Volume 102 Number 2 February 1990
Case Reports 179
Several different classifications have been proposed for the spectrum of disease that is caused by HIV infection.’.I2 The newer CDC classification divides HIV infection into four distinct subgroups: Croup I-acute infection; Group 11-asymptomatic infection; Group III- persistent generalized lymphadenopathy; and Group ZV-other disease. Group IV includes patients with constitutional symptoms, neurologic disease, and secondary infectious disease and malignancies. An older classification still uses the terms “full-blown AIDS” and “AIDS-related complex”, or ARC (a constellation of findings in persons who are HIV-positive but not fulfilling the criteria for AIDS). The signs and symptoms include weight loss, generalized lymphadenopathy, fever, chronic diarrhea, and lymphopenia. Although our patient did not demonstrate the typical reversal of T-helper to T-suppressor cells seen in the full-blown syndrome, he demonstrated sufficient findings to be classified in the ARC category. Further followup would be required to determine the development of AIDS. Increasing reports appear in the otolaryngologic literature regarding unusual conditions seen in HIVpositive patients. Candida supraglottitis, pneumocystis of the temporal bone, and aphthous ulcers of the pharynx and esophagus are among the diseases reported. I3-l5 Otolaryngologists must consider the diagnosis of HIV infection when confronted with an unusual or atypical disease presentation in the head and neck, especially in high-risk patients. Specifically, when evaluating a patient with Ramsay-Hunt syndrome and disseminated herpes zoster, HIV infection should be investigated. REFERENCES
2. Centers for Disease Control. Human immunodeficiency virus infection in the United States: A review of current knowledge. MMWR 1987;36:Supplement 6. 3. Marcusen DC, Sooy CD. Otolaryngologic and head and neck manifestations of acquired immunodeficiency syndrome. Laryngoscope 1985;95:401-5. 4. Fauci AS, Masur H, Gelmann EP, Markham PD, Hahn BH, Lane HC. The acquired immunodeficiency syndrome: An update. Ann Intern Med 1985;102:800-13. 5. Dolin R, Reichrnan RC, Mazur MH, Whitley RJ. Herpes zoster-varicella infections in immunocompromised patients. Ann Intern Med 1978;89:375-88. 6. Crabtree JA. Herpes zoster oticus. Laryngoscope 1968;78:185378. 7. Hunt JR. Herpetic inflammations of the geniculate ganglion: a new syndrome and its complications J Nerv Ment Dis 1907; 34:73. 8. Payton RJ, Dawes JDK. Herpes zoster of the head and neck. J Laryngol Otol 1972;86:1031-55. 9. By1 FN, Adour KR. Auditory symptoms associated with herpes zoster or idiopathic facial paralysis. Laryngoscope 1977;87: 372-9. 10. Aviel A, Marshak G . Ramsay-Hunt syndrome: a cranial polyneuropathy. Am J Otolaryngol 1982;3:61-6. 11. Robillard RB , Hilsinger RL, Adour KR , Ramsay-Hunt paralysis: clinical analysis of 185 patients. OTOLARYNGOL HEADNECKSURG 1986;95:292-7. 12. Centers for Disease Control. Classification systems for human T-lyrnphotropic virus type III/lymphadenopathy-associated virus infections. MMWR 1986;35:20 13 Bye MR, Palomba A, Bernstein L, Shah K. Clinical candida supraglottitis in an infant with AIDS-related complex. Pediatr Pulmonol 1987;3(4):280-1. 14 Breda SD, Harnmerschlag PE, Gigliotti F, Schinella K. Pneumocystis carinii in the temporal bone as a primary manifestation of the acquired immunodeficiency syndrome. Ann Otol Rhino1 Larvneol 1988;97:427-31. 15. Back MC, Valenti AJ, Howell DA, Smith TJ. Odynophagia from apthous ulcers of the pharynx and esophagus in the acquired immunodeficiency syndrome (AIDS). Ann Intern Med 1988; 109(4):338-9. _
I
I . Centers for Disease Control. Kaposi’s sarcoma and pneumocystis pneumonia among homosexual men: New York City and California. MMWR 1981;25:305-8.
Downloaded from oto.sagepub.com at UNIV NEBRASKA LIBRARIES on June 5, 2016
