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A Case of Polyradiculitis with Hearing Loss Analysis of Auditory Brain Stem Response
Auris' Nasus' Larynx (Tokyo) 12, 59-66 (1985)
A CASE OF POLYRADICULITIS WITH HEARING LOSS: ANALYSIS OF AUDITORY BRAIN STEM RESPONSE Kishiko SUGIYAMA, M. D., Shigeru INAFUKU, M. D., Isao TAKIMOTO, M. D.,' Mikihiro KIHARA, M. D., and Akira TAKAHASHI, M. D.2 1Depratment of Otorhinolaryngology, 2Fourth Department of Internal Medicine, Aichi Medical University, Acichi, Japan
A case of acute polyradiculoneuritis accompanied by rare complications of the VII, VIII and IX cranial nerve disorder was described. There was an elevation of the rubella virus antibody titer, which suggested the case had been infected before. Thirty-eight year-old woman complained of bilateral tinnitus and was proven audiometrically to have sensori-neural hearing loss. Electroacoustical examination disclosed that all the waves were suppressed and that wave I and V were barely detected, while wave II, III and IV were absent. The neurological and audiological disorders were successfully treated with corticosteroid, and the patient had neither sequelae nor recurrence. In order to investigate the etiopathogenesis of idiopathic sensori-neural hearing loss, it is very important to investigate the underlying generalized or systemic diseases. Many neuromuscular syndromes associated with hearing loss have been independently described without testing auditory functions in details. In this paper, a case of actute polyradiculitis with hearing loss and elevated rubella virus HI titer is reported in which a series of audiological examinations were performed. A CASE REPORT
Case. 38 year-old woman (housewife). Chief complaints. Hearing impairment with tinnitus in both ears. Past and family histories. Non-contributory. Present history. The patient began to have sore throat and cough on Oct. 10, 1981. Four days later, she complained of lumbago, back pain, general fatigability and weakness of the lower extremities. The symptoms worsened, and she Received for publication May 4, 1984 59
K. SUGIYAMA et al.
60
could not walk on Oct. 18, because of complete weakness in the lower extremities. At that time, tinnitus on both sides and hearing loss appeared. The patient was admitted to a local medical clinic on Oct. 20. She experienced complete weakness in her upper limbs and difficulty in sitting on Oct. 23, drooping sensation of the right corner of her mouth, and impaired speech appeared, and she no longer could use a drinking straw. Then she was found to have facial palsy on both sides. Because of progressive and generalized paralysis, she was referred to the Fourth Department of Internal Medicine of Aichi Medical University on Oct. 27. Neurologic examinations showed severe facial and moderate pharyngeal weakness on both sides. Eye and tongue movements were apparently normal. Her four limbs were generally paralytic, more prominently in the lower limbs and more markedly in the proximal muscles. The deep reflexes were generally paralytic, more prominently in the lower limbs and more markedly in the proximal muscles. The deep reflexes were generally absent in the limbs. Babinski's sign was negative. There was glove-and-stocking type sensory impariment both to touch, pain and temperature. Lumbago and back pain with positive Lasegue sign suggested the involvement of the nerve roots. Other results of her physical examination were normal. The blood chemistry and urine were normal. STS were negative. Viral and serological examinations disclosed the elevation of serum HI titer of rubella virus. Lumbar punctures yielded increase of both cells and protein. The titer of rubella antibody in CSF was within normal range. The administration of adrenocorticotropic hormone resulted in rapid improve1981. 11. 6. -20
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80 90
80 90
100
100
125
1982. 1. 27
250
500
1000 2000 4000 8000 1500 3000 6000 10000
125
250
500
1000 2000 4000 8000 1500 3000 6000 10000
Fig. 1. Audiograms of the present case. A shows a slight sensori-neural hearing loss and B shows an almost normal pattern after recovery.
ABR IN POLYRADICULITIS
61
ment of cranial nerve dysfunctions and tetraparesis. Her tinnitus persisted, though milder, when she was referred to the Department of Otorhinolaryngology several days after (on Nov. 6.) Course. The clinical course is shown in Fig. 4. A diagnosis of acute polyradiculitis was made, and 60 mg/day of prednisolone in tapering dosage was prescribed. All the neurological signs, especially that of the facial palsy and muscular L-ABR (click)
6.32
1l. 7. Stim. 198 l. I
120dB SPL 110
I I
100
90
R-ABR
6.50
120 110 100
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n: 1000: 1 msec
Fig. 2. The latency of wave V is prolonged and wave II, III, IV, disappear. L-ECoG (click)
1981. 11. 7.
60 50
~~~-
40 n: 1000: 30 "7',--~-------t----<
1 msec
Fig. 3. AP manifestation is almost good, but slight delayed latency and broad-shaped.
62
K. SUGIYAMA et al. Mrs. Kik Take (38 y. 0.) 1982
1981 10/0e!. ,
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Fig. 4. The clinical course of the present case is showed. The neurological findings were improved following to administration of prednisolone. R-ABR (click) Stirn.
1981. 11. 7. : --'120dB SPL 1981. 12. 1.
I
1982. 1. 7. 1-----1
1 msec L-ABR 1981. 11. 7. 1981. 12.1. 1982. 1. 7. '--<
1 msec
Fig. 5. ABR improved in parallel with neurologic recovery. The latency of wave V shortened and other waves clearly identified.
ABR IN POLYRADICULITIS
63
weakness of extremities improved steadily. The patient could walk without aid in three months. On the tenth day after initiation of prednisolone therapy, the first otological examinations were done. At that time she had no vertigo, and pathological nystagmus was not detected. There was only slight paralysis of the pharynx. She had difficulty in hearing, complaining about the roaring in the ears. The pure tone audiometry revealed slight hearing loss of convex curve type on both sides (Fig. In). A caloric test disclosed no evidence of subnuclear vestibular disorder. The ABR (Fig. 2) showed appearance of wave I and V on both sides only to the highest sound level (click, 120 dBpeSPL), and extremely prolonged latency of wave V. Wave II, III and IV disappeared. An electrocochleogram (ECochG) demonstrated broad AP with slight delayed latency (Fig. 3). Within a few days, hearing impairment remarkably improved so that she came to have no difficulty in everyday conversation. The hearing impairment as well as ABR abnormalities were improved (Figs. 1 and 5). Wave III was first identified, a little later wave II and IV reappeared. And the latency of wave V became shorter. The following audiological, and vestibular function tests after further improvement of the general condition were normal: speech discrimination test, Bekesy's audiogram, tone decay test, SISI scores, the eye tracking test and optokinetic test. DISCUSSION
The case is characterized by the progressive paralysis of four extremities from the lower to upper limbs, and association of the VII, VIII and IX cranial nerve impairment. The disease is considered to be one of acute polyradiculitis from clinical pictures and CSF findings. The symptoms were allied to Guillain-Barre syndrome (G. B.S.), but no alubumino-cytologic dissociation in CSF was found. Since the titer of rubella virus had little changed, it is somewhat difficult to conclude that the etiology of the case was a direct infection of rubella virus. However, it is possible to assume that past rubella infection was a trigger for the allergic reaction (TAKAHASHI, SAHASHI, SOBUE, GOTO, NAKAO, FUJII, KIHARA, MURAKAMI, TSUCHlYA, TAKIMOTO, INAFUKU, and SUGIYAMA, 1982). Neurological complications with rubella are infrequent; the incidence being one to 5,000-6,000 cases, encephalitis common and polyradiculitis rare. In Japan, only two cases of the latter have so far been reported by NAGASHIMA, KANEHlSA, OIKAWA, YAMADA, and UONO (1977) and KOBAYASHI, NAKANISHI, HAYASHI, KUNIMATSU, and TOGl (1977). In polyradiculitis, facial and lower cranial nerves IX, X, XI, XII are the most frequently involved among the cranial nerve (OSLER and SIDELL, 1960; TAKAHASHI, 1979). Tongue and extraocular muscle paralyses are occasionally present. Involvements of the II and VIII cranial nerves are seldom, and their absence is regarded as one of the diagnostic criteria in G.B.S. (ASBURY, 1981).
64
K. SUGIYAMA et al.
In the world literatures, LASSEN and FOG (1943) described a case of G.B.S. involving the VII, VIII cranial nerves, MIYAZAKI and MUKAI (1971) stated a case of sensory polyradiculitis in his report, and ROSEMAN and ARING (1941) reported involvement of the I, V, VIII, IX cranial nerves. Generally speaking, acute polyradiculitis is self-limiting and regressive in course and fairly good in prognosis. The laboratory findings vary, therefore, as the stage of illness. In this case, the abnormal findings of ABR were distinct as compared with the threshold of the puretone audiogram. Though the pure tone threshold recovered within normal limits, the ABR findings were still abnormal. Providing that the otological examinations in the present case were done later, involvement of the VIII cranial nerve would not be detected. In this reason, if ABR tests are done routinely, the incidence of VIII cranial nerve involvement in polyradiculitis may be greater. The pathology of G.B.S. is characterized by acute demyelination of myelin sheaths. Strictly speaking, the present case differs from G.B.S. with respect to the lack of albumino-cytologic dissociation in CSF, the etiopathogenesis was, however, regarded to be similar. In a variety of neurological diseases, including cerebello-pontine angle lesions, brain stem disorders, spinocerebellar degenerations and multiple sclerosis (MS), the diagnostic value of ABR has been established (STARR and ACHOR, 1975; STOCKARD and ROSSITER, 1977; ROBINSON and RUDGE, 1977; ONISHI and KUJIRAI, 1978; GLASSCOCK, JACKSON, JOSEY, DICKINS, and WIET, 1979; HOSOKAWA, 1979; SHANON, GOLD, HIMMELFARB, and CARASSO, 1979; ROSENHALL, HEDNER, and BJORKMAN, 1981). A high incidence (65-93 %) of ABR abnormality in MS patients was reported by PARVING, ELBERLING, and SMITH (1981). The diagnostic value of ABR were reported in two chronic alcholics (suspected central pontine myelinolysis) by STOCKARD, ROSSITER, WIEDERHOLT, and KOBAYASHI (1976). Fisher syndrome predominantly involving midbrain by KAGA, HANAMURA, YAMADA, and SUZUKI (1977), neonatal hyperbililubinemia by CHISIN, PERLMAN, and SOHMER (1978), leukodystrophy by OCHS, MARKAND, and DEMYER (1979), myoclonus by KASHIWAGI, ICHIKAWA, ANDO, SUETSUGU, and Ko (1979), adrenoleukodystrophy and kernicterus by TANAKA and KAGA (1980), and Shilder's disease by MORINAKA and YAMAMOTO (1981). No report of ABR findings in G.B.S. is found, since the VIII cranial nerve is very rarely involved in G.B.S. STOCKARD et al. (1976) commented that ABR aided in the diagnosis by indicating a slowing of conduction in the pontine auditory pathway, which varied in degree with the severity of the clinical manifestation of pontine demyelination. It is interesting how ABR findings vary with the course of the illness. Also, in this case, improvement of ABR occurred rapidly in pararell with remyelination. In cases of other etiology, however, ABR may remain unchanged regardless of the change in the state of the lesion. Hence the diagnostic value of ABR varies case by case. The anatomical sites of origin of ABR have been established by experimental studies and clinical investigations, but there may remain
ABR IN POLYRADICULITIS
65
unsolved problems in abnormal ABR. In this paper, it was pointed out that ABR may be valuable as a parameter to detect the course of illness in demyelinating diseases. SUMMARY
A rare case of acute polyradiculitis with elevated rubella virus antibody titer and the VII, VIII and IX cranial nerve involvement was reported. The pure tone threshold was slightly elevated, but ABR abnormalities were distinct. Wave V of ABR was markedly prolonged and wave II, III and IV was not identified. AP was well manifested in the electrocochleogram. Corticosteroid therapy improved both ABR abnormalities and neurological signs. REFERENCES ASBURY, A. K.: Diagnostic consideration in Guillain-Barre syndrome. Ann. Neurol. 9: 1-5, 1981. CHISIN, R., PERLMAN, M., and SOHMER, H.: Cochlear and brain stem responses in hearing loss following neonatal hyperbilirubinemia. Ann. Otolaryngol. 88: 352-357, 1978. GLASSCOCK, M. E., JACKSON, C. G., JOSEY, A. F., DICKINS, J. R. E., and WIET, R. J.: Brain stem evoked response audiometry in a clinical practice. Laryngoscope 89: 1021-1035, 1979. HOSOKAWA, T.: BSR in central nervous diseases. J. Otolarynogol. Jpn. (in Japanese) 82: 12-26, 1979. KAGA, K., HANAMURA, T., YAMADA, 0., and SUZUKI, J.: Auditory brain stem responses in neurological disease of brain stem. Brain and Nerve (in Japanese) 29: 791-801, 1977. KASHIWAGI, J., ICHIKAWA, G., ANDO, 1., SUETSUGU, Y., and Ko, M.: BSR in the brain stem lesion. Audiol. Jpn. (in Japanese) 22: 41-50, 1979. KOBAYASHI, G., NAKANISHI, H., HAYASHI, Y., KUNIMATSU, S., and TOGI, H.: A case of GuillainBarre syndrome following rubella infection. J. Showa Med. Univ. Assoc. (in Japanese) 37: 57-59, 1977. LASSEN, H. C., and FOG, M.: Acute polyradiculitis. Acta Med. Scand., 115: 117, 1943. MIYAZAKI, S., and MUKAI, T.: A case of sensory polyradiculoneuritis. Clin. Neurol. (Tokyo) (in Japanese) 11: 196-200, 1971. MORINAKA, S., and YAMAMOTO, E.: BSR findings in healthy adults with normal hearing and patients with intracranial diseases. Pract. Otol. (Kyoto) (in Japanese) 74: 1697-1714, 1981. NAGASHIMA, T., KANEHISA, Y., OIKAWA, S., YAMADA, K., and UONO, M.: A case of polyradiculitis with marked amyotrophy following rubella infection. Neurol. Med. (in Japanese) 6: 423-430, 1977. OCHS, R., MARKAND, O. N., and DEMYER, W. E.: Brain stem auditory evoked response in leukodystrophies. Neurology (Minneap.) 29: 1089-1093, 1979. ONISHI, S., and KUJIRAI, K.: Auditory brain stem response in the diagnosis of brain stem lesion. Audiol. Jpn. (in Japanese) 21: 688-696, 1978. OSLER, L. D., and SIDELL, A. D.: The Guillain-Barre syndrome-The need for exact diagnostic criteria. N. Engl. J. Med. 262: 964-969, 1960. PARVING, A., ELBERLING, C., and SMITH, T.: Auditory electrophysiology-Findings in multiple sclerosis. Audiology 20: 123-142, 1981. ROBINSON, K., and RUDGE, P.: Abnormalities of the auditory evoked potentials in patients with multiple sclerosis. Brain 100: 19-40, 1977.
K. SUGIYAMA et al.
66
ROSEMAN, E., and ARING, C. D.: Infectious polyneuritis. Medicine 20: 463 1941. ROSENHALL, U., HEDNER, M. L., and BJORKMAN, G.: ABR in brain stem lesions. Scand. Audiol. 13: 117-123, 1981. SHANON, E., GOLD, S., HIMMELFARB, M. Z., and CARASSO, R.: Auditory potentials of cochlear nerve and brain stem in multiple sclerosis. Arch. Otolaryngol. 105: 505-508, 1979. STARR, A., and ACHOR, L. J.: Auditory brain stem responses in neurological disease. Arch. Neurol. 32: 761-768, 1975. STOCKARD, J. J., ROSSITER, V. S., WIEDERHOLT, W. C., and KOBAYASHI, R. M.: Brain stem audiology-Evoked responses in suspected central pontine myelinolysis. Arch. Neurol. 33: 726--728, 1976. STOCKARD, J. J., and ROSSITER, V. S.: Clinical and pathologic correlates of brain stem auditory response abnormalities. Neurology (Minneap.) 27: 316--325, 1977. TAKAHASHI, A.: The VIIIth cranial nerve. Its embryological consideration and clinicopathological situation in cranial neurophathy. Annual Report of the Research Committee of Idiopathic Sensorineural Hearing Loss, the Ministry of Health and Welfare of Japan (in Japanese), 1979. TAKAHASHI, A., SAHASHI, I., SOBUE, G., GOTO, S., NAKAO, N., FUJII, K., KIHARA, M., MURAKAMI, K., TSUCHIYA, I., TAKIMOTO, I., INAFUKU, S., and SUGIYAMA, K.: Sensorineural hearing loss appearing in generalized or systemic diseases. Otologia (Fukuoka) (in Japanese) 28: 1026-1043, 1982. TANAKA, Y., and KAGA, K.: Application of brain stem response in brain-injured children. Brain Develop. 2: 45-56, 1980.
Request reprints from:
Dr. K. Sugiyama, Department of Otorhinolaryngology, Aichi Medical University, Nagakute-cho, Aichi-gun, Aichi 480-11, Japan
A CASE OF POLYRADICULITIS WITH HEARING LOSS: ANALYSIS OF AUDITORY BRAIN STEM RESPONSE Kishiko SUGIYAMA, M. D., Shigeru INAFUKU, M. D., Isao TAKIMOTO, M. D.,' Mikihiro KIHARA, M. D., and Akira TAKAHASHI, M. D.2 1Depratment of Otorhinolaryngology, 2Fourth Department of Internal Medicine, Aichi Medical University, Acichi, Japan
A case of acute polyradiculoneuritis accompanied by rare complications of the VII, VIII and IX cranial nerve disorder was described. There was an elevation of the rubella virus antibody titer, which suggested the case had been infected before. Thirty-eight year-old woman complained of bilateral tinnitus and was proven audiometrically to have sensori-neural hearing loss. Electroacoustical examination disclosed that all the waves were suppressed and that wave I and V were barely detected, while wave II, III and IV were absent. The neurological and audiological disorders were successfully treated with corticosteroid, and the patient had neither sequelae nor recurrence. In order to investigate the etiopathogenesis of idiopathic sensori-neural hearing loss, it is very important to investigate the underlying generalized or systemic diseases. Many neuromuscular syndromes associated with hearing loss have been independently described without testing auditory functions in details. In this paper, a case of actute polyradiculitis with hearing loss and elevated rubella virus HI titer is reported in which a series of audiological examinations were performed. A CASE REPORT
Case. 38 year-old woman (housewife). Chief complaints. Hearing impairment with tinnitus in both ears. Past and family histories. Non-contributory. Present history. The patient began to have sore throat and cough on Oct. 10, 1981. Four days later, she complained of lumbago, back pain, general fatigability and weakness of the lower extremities. The symptoms worsened, and she Received for publication May 4, 1984 59
K. SUGIYAMA et al.
60
could not walk on Oct. 18, because of complete weakness in the lower extremities. At that time, tinnitus on both sides and hearing loss appeared. The patient was admitted to a local medical clinic on Oct. 20. She experienced complete weakness in her upper limbs and difficulty in sitting on Oct. 23, drooping sensation of the right corner of her mouth, and impaired speech appeared, and she no longer could use a drinking straw. Then she was found to have facial palsy on both sides. Because of progressive and generalized paralysis, she was referred to the Fourth Department of Internal Medicine of Aichi Medical University on Oct. 27. Neurologic examinations showed severe facial and moderate pharyngeal weakness on both sides. Eye and tongue movements were apparently normal. Her four limbs were generally paralytic, more prominently in the lower limbs and more markedly in the proximal muscles. The deep reflexes were generally paralytic, more prominently in the lower limbs and more markedly in the proximal muscles. The deep reflexes were generally absent in the limbs. Babinski's sign was negative. There was glove-and-stocking type sensory impariment both to touch, pain and temperature. Lumbago and back pain with positive Lasegue sign suggested the involvement of the nerve roots. Other results of her physical examination were normal. The blood chemistry and urine were normal. STS were negative. Viral and serological examinations disclosed the elevation of serum HI titer of rubella virus. Lumbar punctures yielded increase of both cells and protein. The titer of rubella antibody in CSF was within normal range. The administration of adrenocorticotropic hormone resulted in rapid improve1981. 11. 6. -20
-20
-10 r-A dB O
-10 -8 dB O
10 20 30
,J!
(~
V
.,~
1::1)
"3
10
:jr.. ~
20
)-<
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40
.J
c.....--j ~
-- -~-
... -
"'(
~V \ c
~ ~
30
40 50
50
60
60
70
70
80 90
80 90
100
100
125
1982. 1. 27
250
500
1000 2000 4000 8000 1500 3000 6000 10000
125
250
500
1000 2000 4000 8000 1500 3000 6000 10000
Fig. 1. Audiograms of the present case. A shows a slight sensori-neural hearing loss and B shows an almost normal pattern after recovery.
ABR IN POLYRADICULITIS
61
ment of cranial nerve dysfunctions and tetraparesis. Her tinnitus persisted, though milder, when she was referred to the Department of Otorhinolaryngology several days after (on Nov. 6.) Course. The clinical course is shown in Fig. 4. A diagnosis of acute polyradiculitis was made, and 60 mg/day of prednisolone in tapering dosage was prescribed. All the neurological signs, especially that of the facial palsy and muscular L-ABR (click)
6.32
1l. 7. Stim. 198 l. I
120dB SPL 110
I I
100
90
R-ABR
6.50
120 110 100
90
I
n: 1000: 1 msec
Fig. 2. The latency of wave V is prolonged and wave II, III, IV, disappear. L-ECoG (click)
1981. 11. 7.
60 50
~~~-
40 n: 1000: 30 "7',--~-------t----<
1 msec
Fig. 3. AP manifestation is almost good, but slight delayed latency and broad-shaped.
62
K. SUGIYAMA et al. Mrs. Kik Take (38 y. 0.) 1982
1981 10/0e!. ,
l/Dee. ,
l/Nov. admission
J
1!Feb.
I
hospitalization
Hyporefl exi a
J
)
~..#@$$MM//dmm2;;Z21
Hypoaeousis
l/Jan.
(No.81-10455)
.~ t
I
Cell s Protein (mg/dl) CSF
200
300~3[
600 400
100
200
o
m
\ \ 0"~.
-----------
-'
128
~~16
He rpes si m pi ex
.
8
4
Prednisolone (mgjday)
Ce II s
------;ub~\-\~(Kl)~-----'
,---- ____, _ _ ,..:.:=___
x[
Antibody (titer)
_____, . . __
\ ...."or-~---
0
Herpes zoster
60 45
~
Fig. 4. The clinical course of the present case is showed. The neurological findings were improved following to administration of prednisolone. R-ABR (click) Stirn.
1981. 11. 7. : --'120dB SPL 1981. 12. 1.
I
1982. 1. 7. 1-----1
1 msec L-ABR 1981. 11. 7. 1981. 12.1. 1982. 1. 7. '--<
1 msec
Fig. 5. ABR improved in parallel with neurologic recovery. The latency of wave V shortened and other waves clearly identified.
ABR IN POLYRADICULITIS
63
weakness of extremities improved steadily. The patient could walk without aid in three months. On the tenth day after initiation of prednisolone therapy, the first otological examinations were done. At that time she had no vertigo, and pathological nystagmus was not detected. There was only slight paralysis of the pharynx. She had difficulty in hearing, complaining about the roaring in the ears. The pure tone audiometry revealed slight hearing loss of convex curve type on both sides (Fig. In). A caloric test disclosed no evidence of subnuclear vestibular disorder. The ABR (Fig. 2) showed appearance of wave I and V on both sides only to the highest sound level (click, 120 dBpeSPL), and extremely prolonged latency of wave V. Wave II, III and IV disappeared. An electrocochleogram (ECochG) demonstrated broad AP with slight delayed latency (Fig. 3). Within a few days, hearing impairment remarkably improved so that she came to have no difficulty in everyday conversation. The hearing impairment as well as ABR abnormalities were improved (Figs. 1 and 5). Wave III was first identified, a little later wave II and IV reappeared. And the latency of wave V became shorter. The following audiological, and vestibular function tests after further improvement of the general condition were normal: speech discrimination test, Bekesy's audiogram, tone decay test, SISI scores, the eye tracking test and optokinetic test. DISCUSSION
The case is characterized by the progressive paralysis of four extremities from the lower to upper limbs, and association of the VII, VIII and IX cranial nerve impairment. The disease is considered to be one of acute polyradiculitis from clinical pictures and CSF findings. The symptoms were allied to Guillain-Barre syndrome (G. B.S.), but no alubumino-cytologic dissociation in CSF was found. Since the titer of rubella virus had little changed, it is somewhat difficult to conclude that the etiology of the case was a direct infection of rubella virus. However, it is possible to assume that past rubella infection was a trigger for the allergic reaction (TAKAHASHI, SAHASHI, SOBUE, GOTO, NAKAO, FUJII, KIHARA, MURAKAMI, TSUCHlYA, TAKIMOTO, INAFUKU, and SUGIYAMA, 1982). Neurological complications with rubella are infrequent; the incidence being one to 5,000-6,000 cases, encephalitis common and polyradiculitis rare. In Japan, only two cases of the latter have so far been reported by NAGASHIMA, KANEHlSA, OIKAWA, YAMADA, and UONO (1977) and KOBAYASHI, NAKANISHI, HAYASHI, KUNIMATSU, and TOGl (1977). In polyradiculitis, facial and lower cranial nerves IX, X, XI, XII are the most frequently involved among the cranial nerve (OSLER and SIDELL, 1960; TAKAHASHI, 1979). Tongue and extraocular muscle paralyses are occasionally present. Involvements of the II and VIII cranial nerves are seldom, and their absence is regarded as one of the diagnostic criteria in G.B.S. (ASBURY, 1981).
64
K. SUGIYAMA et al.
In the world literatures, LASSEN and FOG (1943) described a case of G.B.S. involving the VII, VIII cranial nerves, MIYAZAKI and MUKAI (1971) stated a case of sensory polyradiculitis in his report, and ROSEMAN and ARING (1941) reported involvement of the I, V, VIII, IX cranial nerves. Generally speaking, acute polyradiculitis is self-limiting and regressive in course and fairly good in prognosis. The laboratory findings vary, therefore, as the stage of illness. In this case, the abnormal findings of ABR were distinct as compared with the threshold of the puretone audiogram. Though the pure tone threshold recovered within normal limits, the ABR findings were still abnormal. Providing that the otological examinations in the present case were done later, involvement of the VIII cranial nerve would not be detected. In this reason, if ABR tests are done routinely, the incidence of VIII cranial nerve involvement in polyradiculitis may be greater. The pathology of G.B.S. is characterized by acute demyelination of myelin sheaths. Strictly speaking, the present case differs from G.B.S. with respect to the lack of albumino-cytologic dissociation in CSF, the etiopathogenesis was, however, regarded to be similar. In a variety of neurological diseases, including cerebello-pontine angle lesions, brain stem disorders, spinocerebellar degenerations and multiple sclerosis (MS), the diagnostic value of ABR has been established (STARR and ACHOR, 1975; STOCKARD and ROSSITER, 1977; ROBINSON and RUDGE, 1977; ONISHI and KUJIRAI, 1978; GLASSCOCK, JACKSON, JOSEY, DICKINS, and WIET, 1979; HOSOKAWA, 1979; SHANON, GOLD, HIMMELFARB, and CARASSO, 1979; ROSENHALL, HEDNER, and BJORKMAN, 1981). A high incidence (65-93 %) of ABR abnormality in MS patients was reported by PARVING, ELBERLING, and SMITH (1981). The diagnostic value of ABR were reported in two chronic alcholics (suspected central pontine myelinolysis) by STOCKARD, ROSSITER, WIEDERHOLT, and KOBAYASHI (1976). Fisher syndrome predominantly involving midbrain by KAGA, HANAMURA, YAMADA, and SUZUKI (1977), neonatal hyperbililubinemia by CHISIN, PERLMAN, and SOHMER (1978), leukodystrophy by OCHS, MARKAND, and DEMYER (1979), myoclonus by KASHIWAGI, ICHIKAWA, ANDO, SUETSUGU, and Ko (1979), adrenoleukodystrophy and kernicterus by TANAKA and KAGA (1980), and Shilder's disease by MORINAKA and YAMAMOTO (1981). No report of ABR findings in G.B.S. is found, since the VIII cranial nerve is very rarely involved in G.B.S. STOCKARD et al. (1976) commented that ABR aided in the diagnosis by indicating a slowing of conduction in the pontine auditory pathway, which varied in degree with the severity of the clinical manifestation of pontine demyelination. It is interesting how ABR findings vary with the course of the illness. Also, in this case, improvement of ABR occurred rapidly in pararell with remyelination. In cases of other etiology, however, ABR may remain unchanged regardless of the change in the state of the lesion. Hence the diagnostic value of ABR varies case by case. The anatomical sites of origin of ABR have been established by experimental studies and clinical investigations, but there may remain
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unsolved problems in abnormal ABR. In this paper, it was pointed out that ABR may be valuable as a parameter to detect the course of illness in demyelinating diseases. SUMMARY
A rare case of acute polyradiculitis with elevated rubella virus antibody titer and the VII, VIII and IX cranial nerve involvement was reported. The pure tone threshold was slightly elevated, but ABR abnormalities were distinct. Wave V of ABR was markedly prolonged and wave II, III and IV was not identified. AP was well manifested in the electrocochleogram. Corticosteroid therapy improved both ABR abnormalities and neurological signs. REFERENCES ASBURY, A. K.: Diagnostic consideration in Guillain-Barre syndrome. Ann. Neurol. 9: 1-5, 1981. CHISIN, R., PERLMAN, M., and SOHMER, H.: Cochlear and brain stem responses in hearing loss following neonatal hyperbilirubinemia. Ann. Otolaryngol. 88: 352-357, 1978. GLASSCOCK, M. E., JACKSON, C. G., JOSEY, A. F., DICKINS, J. R. E., and WIET, R. J.: Brain stem evoked response audiometry in a clinical practice. Laryngoscope 89: 1021-1035, 1979. HOSOKAWA, T.: BSR in central nervous diseases. J. Otolarynogol. Jpn. (in Japanese) 82: 12-26, 1979. KAGA, K., HANAMURA, T., YAMADA, 0., and SUZUKI, J.: Auditory brain stem responses in neurological disease of brain stem. Brain and Nerve (in Japanese) 29: 791-801, 1977. KASHIWAGI, J., ICHIKAWA, G., ANDO, 1., SUETSUGU, Y., and Ko, M.: BSR in the brain stem lesion. Audiol. Jpn. (in Japanese) 22: 41-50, 1979. KOBAYASHI, G., NAKANISHI, H., HAYASHI, Y., KUNIMATSU, S., and TOGI, H.: A case of GuillainBarre syndrome following rubella infection. J. Showa Med. Univ. Assoc. (in Japanese) 37: 57-59, 1977. LASSEN, H. C., and FOG, M.: Acute polyradiculitis. Acta Med. Scand., 115: 117, 1943. MIYAZAKI, S., and MUKAI, T.: A case of sensory polyradiculoneuritis. Clin. Neurol. (Tokyo) (in Japanese) 11: 196-200, 1971. MORINAKA, S., and YAMAMOTO, E.: BSR findings in healthy adults with normal hearing and patients with intracranial diseases. Pract. Otol. (Kyoto) (in Japanese) 74: 1697-1714, 1981. NAGASHIMA, T., KANEHISA, Y., OIKAWA, S., YAMADA, K., and UONO, M.: A case of polyradiculitis with marked amyotrophy following rubella infection. Neurol. Med. (in Japanese) 6: 423-430, 1977. OCHS, R., MARKAND, O. N., and DEMYER, W. E.: Brain stem auditory evoked response in leukodystrophies. Neurology (Minneap.) 29: 1089-1093, 1979. ONISHI, S., and KUJIRAI, K.: Auditory brain stem response in the diagnosis of brain stem lesion. Audiol. Jpn. (in Japanese) 21: 688-696, 1978. OSLER, L. D., and SIDELL, A. D.: The Guillain-Barre syndrome-The need for exact diagnostic criteria. N. Engl. J. Med. 262: 964-969, 1960. PARVING, A., ELBERLING, C., and SMITH, T.: Auditory electrophysiology-Findings in multiple sclerosis. Audiology 20: 123-142, 1981. ROBINSON, K., and RUDGE, P.: Abnormalities of the auditory evoked potentials in patients with multiple sclerosis. Brain 100: 19-40, 1977.
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ROSEMAN, E., and ARING, C. D.: Infectious polyneuritis. Medicine 20: 463 1941. ROSENHALL, U., HEDNER, M. L., and BJORKMAN, G.: ABR in brain stem lesions. Scand. Audiol. 13: 117-123, 1981. SHANON, E., GOLD, S., HIMMELFARB, M. Z., and CARASSO, R.: Auditory potentials of cochlear nerve and brain stem in multiple sclerosis. Arch. Otolaryngol. 105: 505-508, 1979. STARR, A., and ACHOR, L. J.: Auditory brain stem responses in neurological disease. Arch. Neurol. 32: 761-768, 1975. STOCKARD, J. J., ROSSITER, V. S., WIEDERHOLT, W. C., and KOBAYASHI, R. M.: Brain stem audiology-Evoked responses in suspected central pontine myelinolysis. Arch. Neurol. 33: 726--728, 1976. STOCKARD, J. J., and ROSSITER, V. S.: Clinical and pathologic correlates of brain stem auditory response abnormalities. Neurology (Minneap.) 27: 316--325, 1977. TAKAHASHI, A.: The VIIIth cranial nerve. Its embryological consideration and clinicopathological situation in cranial neurophathy. Annual Report of the Research Committee of Idiopathic Sensorineural Hearing Loss, the Ministry of Health and Welfare of Japan (in Japanese), 1979. TAKAHASHI, A., SAHASHI, I., SOBUE, G., GOTO, S., NAKAO, N., FUJII, K., KIHARA, M., MURAKAMI, K., TSUCHIYA, I., TAKIMOTO, I., INAFUKU, S., and SUGIYAMA, K.: Sensorineural hearing loss appearing in generalized or systemic diseases. Otologia (Fukuoka) (in Japanese) 28: 1026-1043, 1982. TANAKA, Y., and KAGA, K.: Application of brain stem response in brain-injured children. Brain Develop. 2: 45-56, 1980.
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Dr. K. Sugiyama, Department of Otorhinolaryngology, Aichi Medical University, Nagakute-cho, Aichi-gun, Aichi 480-11, Japan