Ann Otol 88 :1979
ACUTE VESTIBULAR PARALYSIS IN HERPES ZOSTER OTICUS LEONARD PROCTOR,
MD
HENRY PERLMAN,
BALTIMORE, MARYLAND
JOHN LINDSAY,
MD
CmCAGO, ILLINOIS
MD
GREGORY
CmCAGO, ILLINOIS
MATZ, MD
CmCAGO, ILLINOIS
A case of herpes zoster oticus is presented in which the lateral and superior semicircular canals of the labyrinth were affected unilaterally. The results of several electronystagmographic examinations are described and correlated with the patient's description of symptoms. This case study indicates that disease affecting the lateral semicircular canal is reliably detected by the conventional caloric test. However, the fact that the posterior semicircular canal remained intact could not be inferred from the results of the caloric test in this case. Also, the appearance of nystagmus upon eye closure appears to have been a more sensitive index of the state of the disease process than was the caloric test.
The usual clinical manifestations of herpes zoster oticus consist of severe pain in the ear and mastoid region followed by a vesicular eruption within or near the aural canal. As the cutaneous eruption develops, facial nerve paralysis may appear. In addition, there may be auditory or vestibular symptoms. Histopathologic features':" during the acute phase consist of a dense lymphocytic infiltration of involved structures and scattered small hemorrhages. Lymphocytic aggregates around small vessels are characteristic, resembling those seen in viral inflammations of the central nervous system.v" Intense arterial inflammation may be seen, which could lead to ischemic effects. Inflammatory cells have usually disappeared within about one year, but there still may be noted a loss of sensory or motor neurons or other sensory structures, and the development of fibrous tissue. l,4,5 The disease is felt to be caused by the varicella (chickenpox) virus 1,6 and patients with Hodgkin's disease, other lymphomas, or lymphatic leukemia are unusually susceptible to both spinal and cranial zoster.':" Hunt" drew attention to the association of facial nerve paralysis with a distribution of cutaneous eruptions that he felt were caused by an isolated
lesion of the geniculate ganglion. However, subsequent investigators have found that inflammatory changes in the geniculate ganglion were mild':" or absenF,5 in zoster cases where there was moderate or extensive infiltration of the seventh or eighth nerves. Denny-Brown et aP described a patient with severe pain and a rash involving the right ear and the right head and neck. Temporary facial paralysis occurred with a fresh crop of vesicles on the 11th day of his illness, with deviation of the tongue to the right. During the seventh week a third crop of vesicles appeared, immediately behind the right pinna. The patient died of unrelated causes 64 days after onset of herpes oticus. Histologic examination revealed nearly total destruction of the second cervical dorsal root ganglion and associated neurons. The first cervical ganglion was not examined, but the first posterior root was severely degenerated. Inflammation and degeneration were found in the adjacent spinal cord and within the brain stem. The seventh nerve was infiltrated with lymphocytes but the geniculate ganglion was entirely normal. The possible involvement of the ninth and tenth cranial nerves in zoster oticus
From the Department of Otola rvngolo-ry, Johns Hopkins University, Baltimore, Maryland, and the Department of Surgery (Otolaryngology), Pritzker School of Medicine University of Chicago. This work was supported in part by USPHS Grants 2-R01 NS-09613'-05 and 5-P01 NS-03358-13. Presented at the Midwinter Research Meeting of the Association for Research in Otolaryngology, Tampa, Florida, February 1, 1978.
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has not been clarified by histopathologic study. However, this disease clearly may be associated with a more extensive and varied process than that suggested by the term "geniculate ganglionitis,"? with the potential for involvement of a variety of motor and sensory nerves," multiple cranial and spinal sensory ganglia/,2,7 sensory structures of the labyrinth or cochlea.t-" and various neural structures within the brain and spinal cord.'> In the case reported here, otalgia and a vesicular eruption were followed by severe vestibular disturbance. There was a mild temporary hearing loss, but no diminution in motor function of the seventh nerve. Histologic examination indicated a lesion involving the sensory and neural structures of the left lateral and superior semicircular canals. The left utricular, saccular and posterior canal structures were spared. Careful vestibular and audiometric tests had been done during the course of the patient's illness and therefore this case offered a unique opportunity to evaluate the results of clinical testing in a patient with a histopathologically defined lesion.
CASE REPORT This 33-year-old Caucasion female noted redness and small tender vesicles within and below the orifice of the left aural canal on June 15, 1965. There was mild ear pain. Redness and swelling increased and mild twitching of the left eyelid was noted for one day. Slight dysequilibrium began on June 21. Two days later dysequilibrium precipitously increased and vertigo, nausea, and vomiting appeared, resulting in hospitalization for 18 days at the University of Chicago Hospitals and Clinics. On admission the patient was unable to walk or even sit up and there was a spontaneous nystagmus to the right. No other physical abnormalities were found. Blood and urine analyses and a spinal tap on June 28 were all normal. Her past history included a diagnosis of cervical Hodgkin's disease for which she had received local irradiation (4000 rads) in 1956. No further evidence of this disease was detected until March, 1965, when intraabdominal masses were found and treated with 2300 rads locally. During this therapy a herpes simplex
B
Fig. 1. ENG recording obtained 6/23/65 of eye movements in the anatomic horizontal plane. At A, several blinks (") are seen with eyes open. A strong right-beating nystagmus was present when eyes were closed (ec) and this nystagmus was not affected by warm irrigation of the left ear (44 0 L). Calibration movements (B) were 20 0 visual angle.
eruption appeared on the lip but subsided spontaneously. On the day of her admission in June, 1965, eye movements in the horizontal plane were recorded from binocular leads by electronystagmography (ENG) using a Beckman curvilinear recorder with R-C coupled amplification and 100 mm/ sec paper speed. High frequency filters were set on "slow" mode and low frequency time constant was 3 seconds. Caloric responses were recorded with the patient supine, head up 30° and eyes closed. The recordings showed a strong increase in nystagmus to the right upon eye closure (Fig. 1A) and showed no change in this nystagmus following warm water (44°) irrigation of the left ear. By June 29, there was less intense nystagmus with eye closure but still no effect upon this spontaneous eyes-closed nystagmus from caloric stimulation of the left ear (Fig. 2). A
B
IR I
I SEC ~
VLJ~ Fig. 2. ENG recording obtained 6/29/65 again shows nystagmus to the right with eye closure (ec) and no response to caloric stimulation (6 °L, 44 0 L) of the left ear. Calibration of 20 0 (A) and eye tracing in forward gaze (B) were recorded in erect position.
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VESTIBULAR PARALYSIS AND HERPES
305
\ R [10· 1SEC IL ~
(EYES CLOSED)
44·R
44·L
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Fig. 3. Nystagmus with eye closure (ec) has subsided (7/6/65). No response to caloric stimulation (44°L, 6°L) of left ear, but normal response from stimulation of right ear (44°R).
Nystagmus almost completely subsided by July 6, and there was again no caloric response from the left side, though an appropriate response was obtained from warm irrigation of the right ear (Fig. 3). Eye tracking at 0.4 Hz and optokinetic responses at a single test velocity were normal (Fig. 4). During the next two weeks, nausea and vertigo continued to be severely aggravated by any movement, and especially by lying on the left side. Reading and viewing objects in the visual panorama, as well as eating, were all easier when lying on the right side. After a few days she was able to walk with assistance and gradually recovered a nearly normal gait. However, viewing objects while walking continued to result in a very unpleasant oscillopsia for about three weeks after the onset of her illness. During the second week there was a great pressure in the left side of
Fig. 5. Nystagmus was present again with eye closure on 9/22/65. Caloric responses were absent on left side (30 °L, 44°L) but present on right side (30 0R, 44°R).
her head and frequent headaches. About July 5 she noted the onset of a bitter taste on the left side of her tongue. This changed to a salty sensation and disappeared within a few weeks. How ever, the salty taste reappeared in midSeptember. By July 25 she was able to run, hop, skip, and walk a straight line, and she returned to work. About September 14 there was a recurrence of moderate dysequilibrium. She noted dizziness and slight staggering upon rapid movements again. Mild oscillopsia during walking and an uncertainty of gait on uneven terrain reappeared and persisted for several weeks. An ENG test on September 22 showed a return of strong nystagmus with eye closure (Fig. 5). The eyes-closed nystagmus was significantly increased in intensity by hot irriFRENZEL'S GOGGLES
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Fig. 4. Eye movements during patient's observation of swinging pendulum and striped drum obtained 7/6/65. Arrow shows where drum changed direction. Slow phase eye speed approximately 11 °/sec in lower tracing.
Fig. 6. Faint nystagmus with Frenzel's goggles and strong nystagmus with eyes closed on 2/16/66. No response from caloric stimulation of left ear (44° L, 6° L).
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,
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Fig. 7. Sinusoidal rotatory stimulation with eyes closed on 2/16/66 produced nystagmus only when the chair was turning to the patient's right. Dotted lines indicate leftward portion of oscillation.
gation of the right ear and was reversed by cold irrigation. However, it remained unchanged following either hot or cold irrigation of the left ear (Fig. 5). The patient's dysequilibrium again subsided though ENG testing on February 16, 1966 continued to show strong nystagmus with eye closure (Fig. 6). A manually-operated rotating chair was used on that day also, to apply a sinusoidal stimulus. A strong directional preponderance was demonstrated (Fig. 7). Nystagmus upon eye closure was still present on September 19, 1966. No definite change in intensity or direction of nystagmus during positional testing was found during any of the ENG sessions. Hearing had been normal on the day of her admission in June 1965, but a left sensorineural hearing loss appeared five days later with a pure tone average of 30 dB in the speech frequencies and normal discrimination scores. There was recruitment of loudness. By September, the audiogram was again normal. Chemotherapy and further radiotherapy were applied to control the patient's disease, hut she became progressively worse and died with lobar pneumonia on March 28, 1967, 21 months after the onset of herpes oticus. The temporal bones were removed approximately nine hours after death and processed for histologic study.
Histopathology. The ear canals, tympanic membranes, ossicles and middle ear spaces appeared normal bilaterally.
Fig. 8. Crista ( CR) and associated structures of involved (left) lateral semicircular canal. Note absence of cupula, sensory and supporting cells, and decreased density of nerve fibers (N) supplying crista. Vacuolization (V) probably IS due to postmortem autolysis.
No significant accumulation of inflammatory cells was found in either temporal bone. Except for moderate postmortem autolysis there were no abnormalities of the cochleas, utricles, or saccules of either ear. In the left ear there was slight narrowing of the membranous lateral semicircular canal (LSC) with fibrosis and new bone formation in the perilymphatic space. However, these changes by themselves would probably not have interfered with normal semicircular canal function. On the other hand, a total loss of function would be predicted from the changes in the LSC receptor organ and nerve. The LSC cupula was absent and the sensory and supporting cells of the crista were replaced with fibrous tissue (Fig. 8). There was a striking decrease in the density of nerve fibers supplying the crista. There was a definite decrease in the density of fibers supplying the left superior semicircular canal crista, but the status of the cupula and sensory cells of the crista could not be reliably assessed because of the angle of sectioning. There were no abnormalities of the cochlear nerve, spiral ganglion, facial nerve, geniculate ganglion, or chorda tympani. We wish to emphasize the fact that no abnormalities were found in the structures of the left posterior semicircular canal (PSC); this portion of the diseased left labyrinth appeared capable of normal function ( Fig. 10 ). Photomicrographs of both the left and right
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307
Fig. 9. Crista (CR), cupula (CU) and associated structures of uninvolved (right) lateral semicircular canal appeared intact. Vacuolization (V) represents postmortem autolysis.
PSC structures have been included so that the left PSC can be compared with its healthy mate on the opposite side. No significant abnormalities were observed in the right ear. In particular, the crista and associated structures of the right LSC appeared normal (Fig. 9). Additional details of the histopathology are presented in an earlier publication.'
Fig. 11. Right posterior semicircular canal structures are shown for comparison with those of the left side (Fig. 10).
Autopsy examination revealed Hodgkin's disease involving lymph nodes, lung, spleen, liver and the proximal portion of the common bile duct. There was fibrinous pericarditis, right lower lobe pneumonia, hydrothorax, chronic passive congestion of the liver, and ascites. The brain showed few significant antemortem changes. The meninges appeared normal except for a slight opalescence of the leptomeninges especially in the region of the pacchionian granulation dorsally. Within the cerebellum there was diffuse pale staining and patchy loss of cells in the dentate nucleus and a loss of cells in the granular layer. A section through the pons and substantia nigra showed no abnormalities. DISCUSSION
Fig. 10. Crista and associated structures of left posterior semicircular canal appeared intact. Compare with healthy right posterior semicircular canal (Fig. 11 ) and with diseased left lateral semicircular canal (Fig. 8).
Figure 1 shows typical findings of an acute unilateral peripheral vestibular lesion. Spontaneous right-beating nystagmus is present with eyes open looking straight ahead ( upper tracing). Nystagmus frequency increases slightly with rightward gaze during calibration (Fig. lB). The first important sign indicating that the nystagmus was probably due to a vestibular system disturbance was the fact that the direction of
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nystagmus did not change on leftward gaze. A second important sign of "vestibular" nystagmus was observed, namely, a marked increase in frequency and amplitude upon eye closure (Fig. 1). The fact that nystagmus intensity did not change after irrigation with warm water indicated the left lateral canal reflexes were paralyzed. However, these and subsequent tests did not indicate whether other semicircular canals or otolithic structures were also damaged. Furthermore, test results did not allow a conclusion as to whether the lesion was located in the labyrinth, vestibular nerve, vestibular nucleus, or some combination of structures. The patient was an unusually precise and articulate historian, and her written notes provided an interesting insight into the symptoms experienced during the development of acute vestibular paralysis. Her observations added a helpful perspective to the histopathologic findings, since the latter did not fully reveal the extent and course of the disease. For example, the patient noted a resurgence of her symptoms after three months and this event is not reflected in or explained by the postmortem examination. A progression of the inflammatory disease affecting the peripheral or central vestibular system may have occurred. It is worth noting that caloric test responses remained unaltered throughout the period of observation whereas eye closure revealed first a subsidence and then a reappearance of nystagmus, corresponding to the state of the patient's disturbance. A review of Figures 1 through 6 shows that the presence of nystagmus with eye closure was a more sensitive index of the patient's symptoms and probably more accurately reflected the state of the disease process than did the caloric test. The patient described her visual difficulties when riding home from the hospital in a car. "The view as we traveled looked exactly like a movie made bv a poor amateur from a moving train." A week later she wrote, "As I walk I can't make out the faces of people walking about 20 feet away, even though, of course, in a stationary position my sight is as good as ever," This latter is a
classic description of the problem suffered by patients with bilateral total vestibular paralysis and is related in part to the loss of dynamic vestibuleocular stabilization provided by otolithic reflexes during the vertical accelerations accompanying normal walking. In this case, the symptom must be ascribed to a unilateral vestibular lesion which, according to the final histologic examination, appeared to spare the otolithic apparatus of the involved ear. While it is possible the semicircular canal lesions may have been sufficient by themselves to explain the above symptoms, the patient's observations suggest the function of the utricular organ or nerve may have been impaired, at least temporarily. She found that vertigo could be avoided by moving slowly, but was nevertheless unable to remain on her left side because of an unspecific but severe distress in that position. Also, lying on her right side made it a great deal easier to read and look at objects in her visual environment. Generally it is felt that peripheral vestibular disturbances will produce clear and vivid sensations of spinning or other movement. However, our patient experienced an illusion of movement of her visual surroundings only briefly at the outset of her illness. Thereafter she had a vague but severe distress in her head, especially with rapid turning. The caloric test applied to her uninvolved ear produced a clear, objective vertigo that did not simulate her symptoms. The character and gradual abatement of her symptoms during the first month are in agreement with the findings after unilateral vestibular neurectomy, when imbalance with brisk head movements and oscillopsia when walking for 4 to 12 weeks are reported." The focal degeneration of the cerebellar granular layer and dentate nucleus may not have been present at the time of this patient's acute vestibular disturbance. Even with more detailed histologic examination of the brain, however, it would be difficult to exclude the possibility that an encephalitis was present during or following the acute stage.':" Although no abnormal neurological signs were present. the severe headaches and pressure during the second week suggest at least a
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VESTIBULAR PARALYSIS AND HERPES
temporary meningeal irritation and the recrudescence of signs and symptoms in September may have been caused by central nervous system disturbance rather than a progression of the labyrinthine lesion. Lindsay and Hemenway" described histopathological findings in a patient who suffered from postural vertigo for at least two years after an acute episode of severe vertigo, nausea and vomiting. When examined one month after the original attack, there was spontaneous nystagmus to the left in all positions of gaze and absence of response to hot and cold caloric stimulations of the right ear. Vertigo appeared and nystagmus increased in intensity when the patient lay on her right side. They found complete degeneration of the nerve fibers to the right lateral and superior semicircular canal and utricle. The posterior semicircular canal nerve was intact. Their case showed that sudden partial loss of labyrinthine function could lead to a condition of chronic postural vertigo. The patient reported here also suffered a sudden partial loss of vestibular function, but did not develop chronic postural vertigo. The most notable histopathologic difference between the two cases is the fact that in the present case the utricle was intact, and this could account for the failure to develop chronic postural vertigo. However, additional studies are needed to clarify the relationship between clinical manifestations and pathologic changes"? and for the present our interpretations must remain tentative.
309
It should be emphasized that the caloric test, performed in the conventional manner, failed to reveal the fact that the posterior semicircular canal structures were intact. Had the patient been tested with head tilted forward to eliminate LSC stimulation some rotatory nystagmus might possibly have been observed, though Day and Lindsay'? found no reaction when testing for vertical canal function in a case with a similar partial vestibular lesion. Dohlman's studies" indicate that the effective stimulation acting on the posterior canal is relatively insignificant, no matter what head position is assumed." At any rate, this and other cases 9 , 11 indicate that the conventional caloric test as currently applied does not provide information concerning the functional integrity of the posterior semicircular canal." SUMMARY
1. The development of herpes zoster oticus resulted in permanent destruction of a portion of the labyrinthine structures in this case and produced a clinical picture of acute vestibular paralysis. 2. The fact that the posterior semicircular canal structures remained intact was not detected by clinical observation or by repeated applications of the caloric test in the conventional manner. 3. The caloric test consistently demonstrated the presence of lateral semicircular canal disease, but was less sensitive than eye closure in detecting a subsidence and an exacerbation of the labyrinthine disturbance in this case.
REFERENCES
1. Blackley B, Friedman I, Wright I: Herpes zoster auris associated with facial nerve palsy and auditory nerve symptoms. A case report with histopathological findings. Acta Otolaryngol (Stockh) 63 :533-550, 1967 2. Denny-Brown D, Adams RD, Fitzgerald PJ: Pathologic features of herpes zoster: A note on "geniculate herpes." Arch Neurol Psychiatry 51 :216-231, 1944 3. Culdberg-Moller J, Olsen S, Kettel K: Histopathology of the facial nerve in herpes zoster oticus. Arch Otolaryngol 69:266-275, 1959 4. Zajtchuk IT, Matz GJ, Lindsay JR: Temporal bone pathology in herpes oticus. Ann Otol Rhinol Laryngol 81 :331-338, 1972 5. Sachs E, House RK: The Ramsay Hunt
syndrome: Geniculate herpes. Neurology (Minneap) 6:262-268, 1956 6. Sokal JE, Firat D: Varicella zoster infection in Hodgkin's disease. Am J Med 39: 452-463, 1965 7. Hunt JR: A further contribution to the herpetic inflammation of the geniculate ganglion. Am J Med Sci 136:226-241, 1908 8. Fisch U: The vestibular response following unilateral vestibular neurectomy. Acta Otolaryngol (Stockh) 76:229-238, 1973 9. Lindsay JR, Hemenway WG: Postural vertigo due to unilateral sudden partial loss of vestibular function. Ann Otol Rhinol Laryngol 65:692-706, 1956
10. Schuknecht HF: Pathology of the Ear. Cambridge, Harvard University Press, 1974
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11. Day KM, Lindsay JR: Hydrops of the labyrinth. Case Report. Coagulation operation, clinical course and histopathology. Laryngoscope 59:213-227, 1949 12. Dohlman G: Physikalische and physiologische Studien zur Theorie des kalorischen
Nystagmus. Acta Otolaryngol [Supp!] (Stockh) 5: 1-196, 1925 13. Proctor LR: Testing the vestibular system: Value of the caloric test, in Naunton RF ( ed) : International Vestibular Symposium. New York, Academic Press, 1975
REPlUNTS - Leonard R. Proctor, MD, Department of Otolaryngology, Johns Hopkins Hospital, Baltimore, MD 21205.
NINCDS NOTES" Research highlights of 1978 were reviewed by NINCDS Director Dr. Donald B. Tower before the Senate Subcommittee on Appropriations for the Departments of Labor and HEW on February 28. Accession of patients for the EC-IC by-pass study (an evaluation of the scalp artery to brain artery grafting procedure for prevention of stroke) is well under way, Dr. Tower reported. More than 30 centers in the U. S., Canada, Europe, and Japan are collaborating. NINCDS scientists and grantees, working with both homologous grafts and allografts of peripheral nerve tissue, have reported progress toward the goal of providing a milieu in the central nervous system and perhaps in the spinal cord for cut fibers to regenerate across gaps. Development of neural prostheses for paralyzed and disabled patients continues. An NINCDS contractor is conducting a six-month test in dogs of a prosthesis for the paralyzed urinary bladder; if successful, this test will be followed by human trials later this year. Multiple electrode prostheses for sensorineural deafness have been designed and tested, with indications that crude discriminations of pitch, and consequent improvement in auditory alerting, are now possible. Neuroimmunologists are especially interested in the immune system's suppressor lymphocyte cells, which are thought to prevent autoimmune disorders. Several studies by NINCDS scientists suggest that the suppressor cell response is abnormal in multiple sclerosis (MS). Investigators hypothesize that if tolerance to one's own tissue cells is maintained by suppressor cells, and if these suppressor cells lose their responsiveness (perhaps because of a specific viral insult), an autoimmune disease like MS could ensue. Dr. Tower also described for the Senate Subcommittee the Institute's new initiative in positron emission transverse tomography (PETT), viewing the technique as especially, or perhaps uniquely, applicable to nervous system research. Because of the extensive resources required (cyclotron, special teams of chemists, imaging devices), PETT is not likely to become a routine diagnostic technique. The research possibilities, however, are almost unlimited. "What CAT (computerized axial tomography) brain scans have done for our ability to visualize the anatomy and structural pathology of the intact living brain, PETT promises to do for the metabolism and function of the intact living brain," Dr. Tower told the subcommittee. The first applications for support of PETT development will be reviewed July 30, 1979, at a special meeting of the National Advisory Neurological and Communicative Disorders and Stroke Council. 'This column Is prepared monthly by the Office of Scientific and Health Reports, National Institute of Neurological and Communicative Disorders and Stroke. For further information, write or call Carolyn Holstein, NINCDS/OSHR. NIH Bldg. 31, Room 8A-16, Bethesda, MD 20014. (301) 496-5751.
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ACUTE VESTIBULAR PARALYSIS IN HERPES ZOSTER OTICUS LEONARD PROCTOR,
MD
HENRY PERLMAN,
BALTIMORE, MARYLAND
JOHN LINDSAY,
MD
CmCAGO, ILLINOIS
MD
GREGORY
CmCAGO, ILLINOIS
MATZ, MD
CmCAGO, ILLINOIS
A case of herpes zoster oticus is presented in which the lateral and superior semicircular canals of the labyrinth were affected unilaterally. The results of several electronystagmographic examinations are described and correlated with the patient's description of symptoms. This case study indicates that disease affecting the lateral semicircular canal is reliably detected by the conventional caloric test. However, the fact that the posterior semicircular canal remained intact could not be inferred from the results of the caloric test in this case. Also, the appearance of nystagmus upon eye closure appears to have been a more sensitive index of the state of the disease process than was the caloric test.
The usual clinical manifestations of herpes zoster oticus consist of severe pain in the ear and mastoid region followed by a vesicular eruption within or near the aural canal. As the cutaneous eruption develops, facial nerve paralysis may appear. In addition, there may be auditory or vestibular symptoms. Histopathologic features':" during the acute phase consist of a dense lymphocytic infiltration of involved structures and scattered small hemorrhages. Lymphocytic aggregates around small vessels are characteristic, resembling those seen in viral inflammations of the central nervous system.v" Intense arterial inflammation may be seen, which could lead to ischemic effects. Inflammatory cells have usually disappeared within about one year, but there still may be noted a loss of sensory or motor neurons or other sensory structures, and the development of fibrous tissue. l,4,5 The disease is felt to be caused by the varicella (chickenpox) virus 1,6 and patients with Hodgkin's disease, other lymphomas, or lymphatic leukemia are unusually susceptible to both spinal and cranial zoster.':" Hunt" drew attention to the association of facial nerve paralysis with a distribution of cutaneous eruptions that he felt were caused by an isolated
lesion of the geniculate ganglion. However, subsequent investigators have found that inflammatory changes in the geniculate ganglion were mild':" or absenF,5 in zoster cases where there was moderate or extensive infiltration of the seventh or eighth nerves. Denny-Brown et aP described a patient with severe pain and a rash involving the right ear and the right head and neck. Temporary facial paralysis occurred with a fresh crop of vesicles on the 11th day of his illness, with deviation of the tongue to the right. During the seventh week a third crop of vesicles appeared, immediately behind the right pinna. The patient died of unrelated causes 64 days after onset of herpes oticus. Histologic examination revealed nearly total destruction of the second cervical dorsal root ganglion and associated neurons. The first cervical ganglion was not examined, but the first posterior root was severely degenerated. Inflammation and degeneration were found in the adjacent spinal cord and within the brain stem. The seventh nerve was infiltrated with lymphocytes but the geniculate ganglion was entirely normal. The possible involvement of the ninth and tenth cranial nerves in zoster oticus
From the Department of Otola rvngolo-ry, Johns Hopkins University, Baltimore, Maryland, and the Department of Surgery (Otolaryngology), Pritzker School of Medicine University of Chicago. This work was supported in part by USPHS Grants 2-R01 NS-09613'-05 and 5-P01 NS-03358-13. Presented at the Midwinter Research Meeting of the Association for Research in Otolaryngology, Tampa, Florida, February 1, 1978.
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has not been clarified by histopathologic study. However, this disease clearly may be associated with a more extensive and varied process than that suggested by the term "geniculate ganglionitis,"? with the potential for involvement of a variety of motor and sensory nerves," multiple cranial and spinal sensory ganglia/,2,7 sensory structures of the labyrinth or cochlea.t-" and various neural structures within the brain and spinal cord.'> In the case reported here, otalgia and a vesicular eruption were followed by severe vestibular disturbance. There was a mild temporary hearing loss, but no diminution in motor function of the seventh nerve. Histologic examination indicated a lesion involving the sensory and neural structures of the left lateral and superior semicircular canals. The left utricular, saccular and posterior canal structures were spared. Careful vestibular and audiometric tests had been done during the course of the patient's illness and therefore this case offered a unique opportunity to evaluate the results of clinical testing in a patient with a histopathologically defined lesion.
CASE REPORT This 33-year-old Caucasion female noted redness and small tender vesicles within and below the orifice of the left aural canal on June 15, 1965. There was mild ear pain. Redness and swelling increased and mild twitching of the left eyelid was noted for one day. Slight dysequilibrium began on June 21. Two days later dysequilibrium precipitously increased and vertigo, nausea, and vomiting appeared, resulting in hospitalization for 18 days at the University of Chicago Hospitals and Clinics. On admission the patient was unable to walk or even sit up and there was a spontaneous nystagmus to the right. No other physical abnormalities were found. Blood and urine analyses and a spinal tap on June 28 were all normal. Her past history included a diagnosis of cervical Hodgkin's disease for which she had received local irradiation (4000 rads) in 1956. No further evidence of this disease was detected until March, 1965, when intraabdominal masses were found and treated with 2300 rads locally. During this therapy a herpes simplex
B
Fig. 1. ENG recording obtained 6/23/65 of eye movements in the anatomic horizontal plane. At A, several blinks (") are seen with eyes open. A strong right-beating nystagmus was present when eyes were closed (ec) and this nystagmus was not affected by warm irrigation of the left ear (44 0 L). Calibration movements (B) were 20 0 visual angle.
eruption appeared on the lip but subsided spontaneously. On the day of her admission in June, 1965, eye movements in the horizontal plane were recorded from binocular leads by electronystagmography (ENG) using a Beckman curvilinear recorder with R-C coupled amplification and 100 mm/ sec paper speed. High frequency filters were set on "slow" mode and low frequency time constant was 3 seconds. Caloric responses were recorded with the patient supine, head up 30° and eyes closed. The recordings showed a strong increase in nystagmus to the right upon eye closure (Fig. 1A) and showed no change in this nystagmus following warm water (44°) irrigation of the left ear. By June 29, there was less intense nystagmus with eye closure but still no effect upon this spontaneous eyes-closed nystagmus from caloric stimulation of the left ear (Fig. 2). A
B
IR I
I SEC ~
VLJ~ Fig. 2. ENG recording obtained 6/29/65 again shows nystagmus to the right with eye closure (ec) and no response to caloric stimulation (6 °L, 44 0 L) of the left ear. Calibration of 20 0 (A) and eye tracing in forward gaze (B) were recorded in erect position.
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305
\ R [10· 1SEC IL ~
(EYES CLOSED)
44·R
44·L
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Fig. 3. Nystagmus with eye closure (ec) has subsided (7/6/65). No response to caloric stimulation (44°L, 6°L) of left ear, but normal response from stimulation of right ear (44°R).
Nystagmus almost completely subsided by July 6, and there was again no caloric response from the left side, though an appropriate response was obtained from warm irrigation of the right ear (Fig. 3). Eye tracking at 0.4 Hz and optokinetic responses at a single test velocity were normal (Fig. 4). During the next two weeks, nausea and vertigo continued to be severely aggravated by any movement, and especially by lying on the left side. Reading and viewing objects in the visual panorama, as well as eating, were all easier when lying on the right side. After a few days she was able to walk with assistance and gradually recovered a nearly normal gait. However, viewing objects while walking continued to result in a very unpleasant oscillopsia for about three weeks after the onset of her illness. During the second week there was a great pressure in the left side of
Fig. 5. Nystagmus was present again with eye closure on 9/22/65. Caloric responses were absent on left side (30 °L, 44°L) but present on right side (30 0R, 44°R).
her head and frequent headaches. About July 5 she noted the onset of a bitter taste on the left side of her tongue. This changed to a salty sensation and disappeared within a few weeks. How ever, the salty taste reappeared in midSeptember. By July 25 she was able to run, hop, skip, and walk a straight line, and she returned to work. About September 14 there was a recurrence of moderate dysequilibrium. She noted dizziness and slight staggering upon rapid movements again. Mild oscillopsia during walking and an uncertainty of gait on uneven terrain reappeared and persisted for several weeks. An ENG test on September 22 showed a return of strong nystagmus with eye closure (Fig. 5). The eyes-closed nystagmus was significantly increased in intensity by hot irriFRENZEL'S GOGGLES
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Fig. 6. Faint nystagmus with Frenzel's goggles and strong nystagmus with eyes closed on 2/16/66. No response from caloric stimulation of left ear (44° L, 6° L).
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gation of the right ear and was reversed by cold irrigation. However, it remained unchanged following either hot or cold irrigation of the left ear (Fig. 5). The patient's dysequilibrium again subsided though ENG testing on February 16, 1966 continued to show strong nystagmus with eye closure (Fig. 6). A manually-operated rotating chair was used on that day also, to apply a sinusoidal stimulus. A strong directional preponderance was demonstrated (Fig. 7). Nystagmus upon eye closure was still present on September 19, 1966. No definite change in intensity or direction of nystagmus during positional testing was found during any of the ENG sessions. Hearing had been normal on the day of her admission in June 1965, but a left sensorineural hearing loss appeared five days later with a pure tone average of 30 dB in the speech frequencies and normal discrimination scores. There was recruitment of loudness. By September, the audiogram was again normal. Chemotherapy and further radiotherapy were applied to control the patient's disease, hut she became progressively worse and died with lobar pneumonia on March 28, 1967, 21 months after the onset of herpes oticus. The temporal bones were removed approximately nine hours after death and processed for histologic study.
Histopathology. The ear canals, tympanic membranes, ossicles and middle ear spaces appeared normal bilaterally.
Fig. 8. Crista ( CR) and associated structures of involved (left) lateral semicircular canal. Note absence of cupula, sensory and supporting cells, and decreased density of nerve fibers (N) supplying crista. Vacuolization (V) probably IS due to postmortem autolysis.
No significant accumulation of inflammatory cells was found in either temporal bone. Except for moderate postmortem autolysis there were no abnormalities of the cochleas, utricles, or saccules of either ear. In the left ear there was slight narrowing of the membranous lateral semicircular canal (LSC) with fibrosis and new bone formation in the perilymphatic space. However, these changes by themselves would probably not have interfered with normal semicircular canal function. On the other hand, a total loss of function would be predicted from the changes in the LSC receptor organ and nerve. The LSC cupula was absent and the sensory and supporting cells of the crista were replaced with fibrous tissue (Fig. 8). There was a striking decrease in the density of nerve fibers supplying the crista. There was a definite decrease in the density of fibers supplying the left superior semicircular canal crista, but the status of the cupula and sensory cells of the crista could not be reliably assessed because of the angle of sectioning. There were no abnormalities of the cochlear nerve, spiral ganglion, facial nerve, geniculate ganglion, or chorda tympani. We wish to emphasize the fact that no abnormalities were found in the structures of the left posterior semicircular canal (PSC); this portion of the diseased left labyrinth appeared capable of normal function ( Fig. 10 ). Photomicrographs of both the left and right
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307
Fig. 9. Crista (CR), cupula (CU) and associated structures of uninvolved (right) lateral semicircular canal appeared intact. Vacuolization (V) represents postmortem autolysis.
PSC structures have been included so that the left PSC can be compared with its healthy mate on the opposite side. No significant abnormalities were observed in the right ear. In particular, the crista and associated structures of the right LSC appeared normal (Fig. 9). Additional details of the histopathology are presented in an earlier publication.'
Fig. 11. Right posterior semicircular canal structures are shown for comparison with those of the left side (Fig. 10).
Autopsy examination revealed Hodgkin's disease involving lymph nodes, lung, spleen, liver and the proximal portion of the common bile duct. There was fibrinous pericarditis, right lower lobe pneumonia, hydrothorax, chronic passive congestion of the liver, and ascites. The brain showed few significant antemortem changes. The meninges appeared normal except for a slight opalescence of the leptomeninges especially in the region of the pacchionian granulation dorsally. Within the cerebellum there was diffuse pale staining and patchy loss of cells in the dentate nucleus and a loss of cells in the granular layer. A section through the pons and substantia nigra showed no abnormalities. DISCUSSION
Fig. 10. Crista and associated structures of left posterior semicircular canal appeared intact. Compare with healthy right posterior semicircular canal (Fig. 11 ) and with diseased left lateral semicircular canal (Fig. 8).
Figure 1 shows typical findings of an acute unilateral peripheral vestibular lesion. Spontaneous right-beating nystagmus is present with eyes open looking straight ahead ( upper tracing). Nystagmus frequency increases slightly with rightward gaze during calibration (Fig. lB). The first important sign indicating that the nystagmus was probably due to a vestibular system disturbance was the fact that the direction of
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nystagmus did not change on leftward gaze. A second important sign of "vestibular" nystagmus was observed, namely, a marked increase in frequency and amplitude upon eye closure (Fig. 1). The fact that nystagmus intensity did not change after irrigation with warm water indicated the left lateral canal reflexes were paralyzed. However, these and subsequent tests did not indicate whether other semicircular canals or otolithic structures were also damaged. Furthermore, test results did not allow a conclusion as to whether the lesion was located in the labyrinth, vestibular nerve, vestibular nucleus, or some combination of structures. The patient was an unusually precise and articulate historian, and her written notes provided an interesting insight into the symptoms experienced during the development of acute vestibular paralysis. Her observations added a helpful perspective to the histopathologic findings, since the latter did not fully reveal the extent and course of the disease. For example, the patient noted a resurgence of her symptoms after three months and this event is not reflected in or explained by the postmortem examination. A progression of the inflammatory disease affecting the peripheral or central vestibular system may have occurred. It is worth noting that caloric test responses remained unaltered throughout the period of observation whereas eye closure revealed first a subsidence and then a reappearance of nystagmus, corresponding to the state of the patient's disturbance. A review of Figures 1 through 6 shows that the presence of nystagmus with eye closure was a more sensitive index of the patient's symptoms and probably more accurately reflected the state of the disease process than did the caloric test. The patient described her visual difficulties when riding home from the hospital in a car. "The view as we traveled looked exactly like a movie made bv a poor amateur from a moving train." A week later she wrote, "As I walk I can't make out the faces of people walking about 20 feet away, even though, of course, in a stationary position my sight is as good as ever," This latter is a
classic description of the problem suffered by patients with bilateral total vestibular paralysis and is related in part to the loss of dynamic vestibuleocular stabilization provided by otolithic reflexes during the vertical accelerations accompanying normal walking. In this case, the symptom must be ascribed to a unilateral vestibular lesion which, according to the final histologic examination, appeared to spare the otolithic apparatus of the involved ear. While it is possible the semicircular canal lesions may have been sufficient by themselves to explain the above symptoms, the patient's observations suggest the function of the utricular organ or nerve may have been impaired, at least temporarily. She found that vertigo could be avoided by moving slowly, but was nevertheless unable to remain on her left side because of an unspecific but severe distress in that position. Also, lying on her right side made it a great deal easier to read and look at objects in her visual environment. Generally it is felt that peripheral vestibular disturbances will produce clear and vivid sensations of spinning or other movement. However, our patient experienced an illusion of movement of her visual surroundings only briefly at the outset of her illness. Thereafter she had a vague but severe distress in her head, especially with rapid turning. The caloric test applied to her uninvolved ear produced a clear, objective vertigo that did not simulate her symptoms. The character and gradual abatement of her symptoms during the first month are in agreement with the findings after unilateral vestibular neurectomy, when imbalance with brisk head movements and oscillopsia when walking for 4 to 12 weeks are reported." The focal degeneration of the cerebellar granular layer and dentate nucleus may not have been present at the time of this patient's acute vestibular disturbance. Even with more detailed histologic examination of the brain, however, it would be difficult to exclude the possibility that an encephalitis was present during or following the acute stage.':" Although no abnormal neurological signs were present. the severe headaches and pressure during the second week suggest at least a
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temporary meningeal irritation and the recrudescence of signs and symptoms in September may have been caused by central nervous system disturbance rather than a progression of the labyrinthine lesion. Lindsay and Hemenway" described histopathological findings in a patient who suffered from postural vertigo for at least two years after an acute episode of severe vertigo, nausea and vomiting. When examined one month after the original attack, there was spontaneous nystagmus to the left in all positions of gaze and absence of response to hot and cold caloric stimulations of the right ear. Vertigo appeared and nystagmus increased in intensity when the patient lay on her right side. They found complete degeneration of the nerve fibers to the right lateral and superior semicircular canal and utricle. The posterior semicircular canal nerve was intact. Their case showed that sudden partial loss of labyrinthine function could lead to a condition of chronic postural vertigo. The patient reported here also suffered a sudden partial loss of vestibular function, but did not develop chronic postural vertigo. The most notable histopathologic difference between the two cases is the fact that in the present case the utricle was intact, and this could account for the failure to develop chronic postural vertigo. However, additional studies are needed to clarify the relationship between clinical manifestations and pathologic changes"? and for the present our interpretations must remain tentative.
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It should be emphasized that the caloric test, performed in the conventional manner, failed to reveal the fact that the posterior semicircular canal structures were intact. Had the patient been tested with head tilted forward to eliminate LSC stimulation some rotatory nystagmus might possibly have been observed, though Day and Lindsay'? found no reaction when testing for vertical canal function in a case with a similar partial vestibular lesion. Dohlman's studies" indicate that the effective stimulation acting on the posterior canal is relatively insignificant, no matter what head position is assumed." At any rate, this and other cases 9 , 11 indicate that the conventional caloric test as currently applied does not provide information concerning the functional integrity of the posterior semicircular canal." SUMMARY
1. The development of herpes zoster oticus resulted in permanent destruction of a portion of the labyrinthine structures in this case and produced a clinical picture of acute vestibular paralysis. 2. The fact that the posterior semicircular canal structures remained intact was not detected by clinical observation or by repeated applications of the caloric test in the conventional manner. 3. The caloric test consistently demonstrated the presence of lateral semicircular canal disease, but was less sensitive than eye closure in detecting a subsidence and an exacerbation of the labyrinthine disturbance in this case.
REFERENCES
1. Blackley B, Friedman I, Wright I: Herpes zoster auris associated with facial nerve palsy and auditory nerve symptoms. A case report with histopathological findings. Acta Otolaryngol (Stockh) 63 :533-550, 1967 2. Denny-Brown D, Adams RD, Fitzgerald PJ: Pathologic features of herpes zoster: A note on "geniculate herpes." Arch Neurol Psychiatry 51 :216-231, 1944 3. Culdberg-Moller J, Olsen S, Kettel K: Histopathology of the facial nerve in herpes zoster oticus. Arch Otolaryngol 69:266-275, 1959 4. Zajtchuk IT, Matz GJ, Lindsay JR: Temporal bone pathology in herpes oticus. Ann Otol Rhinol Laryngol 81 :331-338, 1972 5. Sachs E, House RK: The Ramsay Hunt
syndrome: Geniculate herpes. Neurology (Minneap) 6:262-268, 1956 6. Sokal JE, Firat D: Varicella zoster infection in Hodgkin's disease. Am J Med 39: 452-463, 1965 7. Hunt JR: A further contribution to the herpetic inflammation of the geniculate ganglion. Am J Med Sci 136:226-241, 1908 8. Fisch U: The vestibular response following unilateral vestibular neurectomy. Acta Otolaryngol (Stockh) 76:229-238, 1973 9. Lindsay JR, Hemenway WG: Postural vertigo due to unilateral sudden partial loss of vestibular function. Ann Otol Rhinol Laryngol 65:692-706, 1956
10. Schuknecht HF: Pathology of the Ear. Cambridge, Harvard University Press, 1974
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11. Day KM, Lindsay JR: Hydrops of the labyrinth. Case Report. Coagulation operation, clinical course and histopathology. Laryngoscope 59:213-227, 1949 12. Dohlman G: Physikalische and physiologische Studien zur Theorie des kalorischen
Nystagmus. Acta Otolaryngol [Supp!] (Stockh) 5: 1-196, 1925 13. Proctor LR: Testing the vestibular system: Value of the caloric test, in Naunton RF ( ed) : International Vestibular Symposium. New York, Academic Press, 1975
REPlUNTS - Leonard R. Proctor, MD, Department of Otolaryngology, Johns Hopkins Hospital, Baltimore, MD 21205.
NINCDS NOTES" Research highlights of 1978 were reviewed by NINCDS Director Dr. Donald B. Tower before the Senate Subcommittee on Appropriations for the Departments of Labor and HEW on February 28. Accession of patients for the EC-IC by-pass study (an evaluation of the scalp artery to brain artery grafting procedure for prevention of stroke) is well under way, Dr. Tower reported. More than 30 centers in the U. S., Canada, Europe, and Japan are collaborating. NINCDS scientists and grantees, working with both homologous grafts and allografts of peripheral nerve tissue, have reported progress toward the goal of providing a milieu in the central nervous system and perhaps in the spinal cord for cut fibers to regenerate across gaps. Development of neural prostheses for paralyzed and disabled patients continues. An NINCDS contractor is conducting a six-month test in dogs of a prosthesis for the paralyzed urinary bladder; if successful, this test will be followed by human trials later this year. Multiple electrode prostheses for sensorineural deafness have been designed and tested, with indications that crude discriminations of pitch, and consequent improvement in auditory alerting, are now possible. Neuroimmunologists are especially interested in the immune system's suppressor lymphocyte cells, which are thought to prevent autoimmune disorders. Several studies by NINCDS scientists suggest that the suppressor cell response is abnormal in multiple sclerosis (MS). Investigators hypothesize that if tolerance to one's own tissue cells is maintained by suppressor cells, and if these suppressor cells lose their responsiveness (perhaps because of a specific viral insult), an autoimmune disease like MS could ensue. Dr. Tower also described for the Senate Subcommittee the Institute's new initiative in positron emission transverse tomography (PETT), viewing the technique as especially, or perhaps uniquely, applicable to nervous system research. Because of the extensive resources required (cyclotron, special teams of chemists, imaging devices), PETT is not likely to become a routine diagnostic technique. The research possibilities, however, are almost unlimited. "What CAT (computerized axial tomography) brain scans have done for our ability to visualize the anatomy and structural pathology of the intact living brain, PETT promises to do for the metabolism and function of the intact living brain," Dr. Tower told the subcommittee. The first applications for support of PETT development will be reviewed July 30, 1979, at a special meeting of the National Advisory Neurological and Communicative Disorders and Stroke Council. 'This column Is prepared monthly by the Office of Scientific and Health Reports, National Institute of Neurological and Communicative Disorders and Stroke. For further information, write or call Carolyn Holstein, NINCDS/OSHR. NIH Bldg. 31, Room 8A-16, Bethesda, MD 20014. (301) 496-5751.
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