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Clinical Significance of Biphasic Head-Shaking Nystagmus
Auris·Nasus·Larynx (Tokyo) 13 (Suppl. II), S 199-S 204,1986
CLINICAL SIGNIFICANCE OF BIPHASIC HEAD-SHAKING NYSTAGMUS Sachiko
TAKAHASHI,
M.D.
Department of Otorhinolaryngology, School of Medicine, Gunma University. Maebashi, Japan
Biphasic head-shaking nystagmus (b-HSN) is a rather uncommon phenomenon which was first reported by KAMEl in 1975 (Arch. Otorhinolaryngol. 209: 59-67, 1975). He pointed out that b-HSN appeared in cases of peripheral vestibular :disturbance especially in the cases of unilateral disturbance, and that the direction of the second phase indicated the damaged side. While investigating cases of b-HSN, the author encountered some cases which did not correspond to b-HSN reported by Kamei. Nineteen patients in whom b-HSN was recorded by e1ectronystagmography were investigated. These patients visited our clinic in the last two years and showed no spontaneous nystagmus in the narrow sense. Of these, 16 were cases of unilateral peripheral vestibular disturbance. In 13 of these (81 %), as Kamei had described, the first phase beat toward the healthy side and the second phase beat toward the damaged side, while in the remaining 3 cases (19%), the first phase beat toward the damaged side, and the second phase toward the healthy side. This is contrary to Kamei's report. This time the b-HSN was also observed in 3 cases of central vestibular disturbance, which indicates that b-HSN occurs not only in cases of peripheral vestibular disturbance but also in cases of central vestibular disturbance. Head-shaking nystagmus (HSN) is the nystagmus provoked after rapid head-shaking. The significance of HSN as a useful screening test for vertigo has already been reported. Its provocation rate is very high in vertiginous patients. On the other hand, it does not appear in normal subjects (KAMEl et al., 1964). In most cases HSN does not change its direction (monophasic). In many of these cases the nystagmus is directed toward the healthy side as a deficiency nystagmus, while in some cases the nystagmus is directed toward the damaged side as a recovery nystagmus (KAMEl et al., 1984). KAMEl was the first to report on b-HSN in 1975 as a rather uncommon Received for publication July 11, 1986 S 199
S200
S. TAKAHASHI
phenomenon. Kamei described that b-HSN is seen in cases of peripheral vestibular disturbance, especially in cases of unilateral disturbance, and that the direction of the second phase indicated the damaged side. He stressed that the b-HSN is very important for the diagnosis of the damaged side. The author investigated cases of b-HSN and encountered some cases of b-HSN which did not correspond to b-HSN reported by Kamei. SUBJECTS AND METHOD
Nineteen patients in whom b-HSN was recorded by electronystagmography were investigated. These patients visited the vestibular clinic of Gunma University from June 1983 to April 1985. They showed no spontaneous nystagmus with eyes closed and/or with eyes open in the darkness. According to the method which was described by KAMEl (1975) and KAMEl et al. (1984) the head-shaking procedure is performed as follows: the patient is placed in a completely dark room with the head lowered forward by ca. 30°. The patient's head is shaken to and fro 30 times with an amplitude of ca. 90° in about 15 sec. The eyes are kept closed during the head-shaking. Immediately after the shaking the patient opens his eyes. The provoked nystagmus is recorded byelectronystagmography. RESULTS
Table 1 shows a list of 19 cases in whom b-HSN was recorded by electronystagmography. Sixteen cases (Cases 1-16) were cases of unilateral peripheral vestibular disturbance, in whom the damaged side was obvious. The remaining 3 cases (Cases 17-19) were cases of central vestibular disturbance, in whom the damaged side was unknown. The relationship between the damaged side and the direction of HSN was studied among the 16 cases in whom the damaged side was obvious. In the majority (13 cases, 81 %) the first phase was directed to the healthy side, and the second phase to the damaged side, while in the minority (3 cases, 19%), the first phase was directed to the damaged side, and the second phase to the healthy side. The former 13 cases were categorized as group I in Table 1, for the sake of convenience; the latter 3 cases, as group II; and the 3 cases of central disturbance, as group III. In 9 cases of group I (Cases 1-9), hearing loss in the damaged side was observed. Among them, 3 cases were accompanied by hearing loss of less severe degree in the opposite ear. Hearing was normal bilaterally in 4 cases (Cases 10-13). In 10 cases of group I the caloric response was decreased in the damaged side; however, the labyrinth function was not totally lost. In Case
HEAD-SHAKING NYSTAGMUS
S 201
12 the caloric test was not performed and Cases I and 5 showed directional preponderance to the left and to the right respectively. Table 1. Case
Biphasic head-shaking nystagmus.
Disease
Sex Age
Damaged side
-
--
Direction of HSN -.---~~~
1st phase
2nd phase
•• •• •• • • ••• • •
1* 2* 3* 4* 5* 6* 7* 8* 9* 10* 11* 12* 13*
Meniere's disease Meniere's disease Meniere's disease Meniere's disease Meniere's disease Meniere's disease Sudden deafness Sudden deafness Mumps deafness Recurrent labyrinthopathy Recurrent labyrinthopathy Acoustic trauma Vestibular neuronitis
M F F M M F F F F F F F F
55 50 61 34 34 41 38 53 11 61 37 50 44
Left Right Left Right Left Right Left Right Left Left Right Right Left
Right Left Right Left Right Left Right Left Right Right Left Left Right
0 0 0 0 0 0 0 0 0 0 0 0 0
Left Right Left Right Left Right Left Right Left Left Right Right Left
14** 15** 16**
Meniere's disease Meniere's disease Meniere's disease
M72 M 42 F 57
Right Left Left
Right Left Left
• ••
Left 0 Right 0 Right 0
17***
Spinocerebellar degeneration Multiple sclerosis Truncal ataxia
M71 F 54 F 57
18*** 19***
Right Left Left
Left Right Right
*, group I; **, group II; ***, group III; 0, nystagmus directed toward the healthy side; . , nystagmus directed toward the damaged side.
PHASE
PHASE
2
i
1
----.-J 10°
5 sec Fig. 1. B-HSN in group I. YM ,38F (Case 7 in Table 1) suffered from sudden deafness of left ear for 6 months. Severe sensorineural hearing loss was observed in left ear. Hearing was normal in right ear. Caloric test revealed canal paresis in left ear, but the labyrinth function was not totally lost. After the head-shaking, nystagmus (1st phase) is elicited to right (healthy side), then after the short latency period, nystagmus (2nd phase) is elicited to left (damaged side).
S. TAKAHASHI
S202
l'
PHASE
2
__ ,~___~~_~_~--~ - '~ JIO' l'
I sec
Fig. 2. b-HSN in group II. TT 12M (Case 14 in Table 1) suffered from Meniere's disease in right ear for IO years. Bilateral hearing loss which is more severe in right ear was observed. Caloric test revealed canal paresis in right ear, but the labyrinth function was not totally lost. After the head-shaking, nystagmus (1st phase) is elicited to right (damaged side), then after the 5hort latency period, nystagmus (2nd phase) is elicited to left (healthy side).
~
_ _ _.-JIIO'
tiD
sec
Fig. 3. b-HSN in group III. AS 71M (Case 17 in Table 1) suffered from inability of gait for a month and the diagnosis of spinocerebellar degeneration was made. He had bilateral hearing loss of almost the same degree in both ears. The caloric response was normal in both ears. Except for the fact that he had dysdiadochokinesis in his right hand, no remarkable lateralization of neurological symptom was found. After the head shaking, nystagmus (1st phase) was elicited to the right, then after the short latency period, nystagmus (2nd phase) was elicited to the left.
Patients in group II all suffered from Meniere's disease. Bilateral hearing loss which was more severe in the damaged side was observed in all cases. The caloric test revealed the canal paresis in the damaged side in all cases; however, there was no complete loss of labyrinthine function. In all cases of group III sensorineural hearing loss of almost the same degree
HEAD-SHAKING NYSTAGMUS
S 203
in both ears was observed. Caloric response was normal in all. Truncal ataxia was remarkable in all cases. Except for the fact that Case 17 showed dysdiadochokinesis in his right hand, there was no remarkable neurological lateralization in any of the cases. The electronystagmographic findings of b-HSN was surprisingly similar in all 3 groups, except for the mixture of many square wave jerks in the electronystagmogram (ENG) of group III. Namely, immediately after the head-shaking, strong, lively nystagmus occurs and regresses rapidly in ca. 5-15 sec (I st phase), then after a short latency period, a fine-beating and somewhat irregular nystagmus occurs to the opposite side and lasts over 30 sec (2nd phase). These are the common features of ENG in all 3 groups. As an example, the ENG of Case 7 from group I is shown in Fig. I. The ENG of Case 14 from group II is shown in Fig. 2. And the ENG of Case 17 from group III is shown in Fig. 3. DISCUSSION
The b-HSN of group I was considered to be equivalent to the b-HSN reported by KAMEl in 1975 from the point of view of direction of nystagmus. Kamei also described that the b-HSN was observed in cases of peripheral vestibular disturbance, especially unilateral disturbance, and that the function of the diseased labyrinth was not entirely lost. All cases of group I also had these features. For the origin of nystagmus Kamei considered the first phase as deficiency nystagmus and the second phase, recovery nystagmus. This interpretation could also be applied to the b-HSN of group I. In 1982, Spindler mentioned in his personal communication to Kamei (KAMEl et ai., 1984) that such b-HSN also exists where the first phase is directed toward the damaged side and the second phase toward the healthy side. Therefore, the b-HSN in group II corresponds to the b-HSN that Spindler previously pointed out. The cases of group II also suffered from unilateral peripheral vestibular disturbance and the function of the diseased labyrinth had not been entirely lost. Up to the present time, b-HSN has only been reported in cases of peripheral vestibular disturbance. However, this time b-HSN was observed in 3 cases of central vestibular disturbance. Because of the smaIl number of patients, the origin of the HSN in group II and group HI remains unknown. CONCLUSION
Through the electronystagmographic investigation of b-HSN, the author has confirmed the existence of some cases of b-HSN, which do not correspond
S204
S. TAKAHASHI
to the b-HSN previously reported by Kamei. In the majority of cases the second phase was directed toward the damaged side as Kamei had described, but in the minority of cases, in contrast to Kamei's report, the first phase was directed toward the damaged side. In addition, b-HSN was also observed in cases of central vestibular disturbance. I would like to thank Professor Tamio Kamei for his pertinent advice on the manuscript.
REFERENCES KAMEl, T.: Der biphasisch auftretende Kopfschuttelnystagmus. Arch. Otorhinolaryngol. 209: 59-67, 1975. KAMEl, T., KIMURA, K., KANEKO, H., and NORO, K.: Revaluation of the head-shaking test as a method of nystagmus provocation. Part 1: Its nystagmus eliciting effect. Jpn. J. Otol. (in Japanese) 67: 1530--1534, 1964. KAMEl, T., TAKAHASHI, S., KAMADA, H., and MUROI, M.: Revaluation of the head-shaking test as a method of nystagmus provocation. Part 2: Its diagnostic significance for the site of lesion. Equil. Res. (in Japanese) 43: 236-242, 1984.
Request reprints to:
Dr. S. Takahashi, Department of Otorhinolaryngology, School of Medicine, Gunma University, 3-39-15 Showamachi, Maebashi 371, Japan
CLINICAL SIGNIFICANCE OF BIPHASIC HEAD-SHAKING NYSTAGMUS Sachiko
TAKAHASHI,
M.D.
Department of Otorhinolaryngology, School of Medicine, Gunma University. Maebashi, Japan
Biphasic head-shaking nystagmus (b-HSN) is a rather uncommon phenomenon which was first reported by KAMEl in 1975 (Arch. Otorhinolaryngol. 209: 59-67, 1975). He pointed out that b-HSN appeared in cases of peripheral vestibular :disturbance especially in the cases of unilateral disturbance, and that the direction of the second phase indicated the damaged side. While investigating cases of b-HSN, the author encountered some cases which did not correspond to b-HSN reported by Kamei. Nineteen patients in whom b-HSN was recorded by e1ectronystagmography were investigated. These patients visited our clinic in the last two years and showed no spontaneous nystagmus in the narrow sense. Of these, 16 were cases of unilateral peripheral vestibular disturbance. In 13 of these (81 %), as Kamei had described, the first phase beat toward the healthy side and the second phase beat toward the damaged side, while in the remaining 3 cases (19%), the first phase beat toward the damaged side, and the second phase toward the healthy side. This is contrary to Kamei's report. This time the b-HSN was also observed in 3 cases of central vestibular disturbance, which indicates that b-HSN occurs not only in cases of peripheral vestibular disturbance but also in cases of central vestibular disturbance. Head-shaking nystagmus (HSN) is the nystagmus provoked after rapid head-shaking. The significance of HSN as a useful screening test for vertigo has already been reported. Its provocation rate is very high in vertiginous patients. On the other hand, it does not appear in normal subjects (KAMEl et al., 1964). In most cases HSN does not change its direction (monophasic). In many of these cases the nystagmus is directed toward the healthy side as a deficiency nystagmus, while in some cases the nystagmus is directed toward the damaged side as a recovery nystagmus (KAMEl et al., 1984). KAMEl was the first to report on b-HSN in 1975 as a rather uncommon Received for publication July 11, 1986 S 199
S200
S. TAKAHASHI
phenomenon. Kamei described that b-HSN is seen in cases of peripheral vestibular disturbance, especially in cases of unilateral disturbance, and that the direction of the second phase indicated the damaged side. He stressed that the b-HSN is very important for the diagnosis of the damaged side. The author investigated cases of b-HSN and encountered some cases of b-HSN which did not correspond to b-HSN reported by Kamei. SUBJECTS AND METHOD
Nineteen patients in whom b-HSN was recorded by electronystagmography were investigated. These patients visited the vestibular clinic of Gunma University from June 1983 to April 1985. They showed no spontaneous nystagmus with eyes closed and/or with eyes open in the darkness. According to the method which was described by KAMEl (1975) and KAMEl et al. (1984) the head-shaking procedure is performed as follows: the patient is placed in a completely dark room with the head lowered forward by ca. 30°. The patient's head is shaken to and fro 30 times with an amplitude of ca. 90° in about 15 sec. The eyes are kept closed during the head-shaking. Immediately after the shaking the patient opens his eyes. The provoked nystagmus is recorded byelectronystagmography. RESULTS
Table 1 shows a list of 19 cases in whom b-HSN was recorded by electronystagmography. Sixteen cases (Cases 1-16) were cases of unilateral peripheral vestibular disturbance, in whom the damaged side was obvious. The remaining 3 cases (Cases 17-19) were cases of central vestibular disturbance, in whom the damaged side was unknown. The relationship between the damaged side and the direction of HSN was studied among the 16 cases in whom the damaged side was obvious. In the majority (13 cases, 81 %) the first phase was directed to the healthy side, and the second phase to the damaged side, while in the minority (3 cases, 19%), the first phase was directed to the damaged side, and the second phase to the healthy side. The former 13 cases were categorized as group I in Table 1, for the sake of convenience; the latter 3 cases, as group II; and the 3 cases of central disturbance, as group III. In 9 cases of group I (Cases 1-9), hearing loss in the damaged side was observed. Among them, 3 cases were accompanied by hearing loss of less severe degree in the opposite ear. Hearing was normal bilaterally in 4 cases (Cases 10-13). In 10 cases of group I the caloric response was decreased in the damaged side; however, the labyrinth function was not totally lost. In Case
HEAD-SHAKING NYSTAGMUS
S 201
12 the caloric test was not performed and Cases I and 5 showed directional preponderance to the left and to the right respectively. Table 1. Case
Biphasic head-shaking nystagmus.
Disease
Sex Age
Damaged side
-
--
Direction of HSN -.---~~~
1st phase
2nd phase
•• •• •• • • ••• • •
1* 2* 3* 4* 5* 6* 7* 8* 9* 10* 11* 12* 13*
Meniere's disease Meniere's disease Meniere's disease Meniere's disease Meniere's disease Meniere's disease Sudden deafness Sudden deafness Mumps deafness Recurrent labyrinthopathy Recurrent labyrinthopathy Acoustic trauma Vestibular neuronitis
M F F M M F F F F F F F F
55 50 61 34 34 41 38 53 11 61 37 50 44
Left Right Left Right Left Right Left Right Left Left Right Right Left
Right Left Right Left Right Left Right Left Right Right Left Left Right
0 0 0 0 0 0 0 0 0 0 0 0 0
Left Right Left Right Left Right Left Right Left Left Right Right Left
14** 15** 16**
Meniere's disease Meniere's disease Meniere's disease
M72 M 42 F 57
Right Left Left
Right Left Left
• ••
Left 0 Right 0 Right 0
17***
Spinocerebellar degeneration Multiple sclerosis Truncal ataxia
M71 F 54 F 57
18*** 19***
Right Left Left
Left Right Right
*, group I; **, group II; ***, group III; 0, nystagmus directed toward the healthy side; . , nystagmus directed toward the damaged side.
PHASE
PHASE
2
i
1
----.-J 10°
5 sec Fig. 1. B-HSN in group I. YM ,38F (Case 7 in Table 1) suffered from sudden deafness of left ear for 6 months. Severe sensorineural hearing loss was observed in left ear. Hearing was normal in right ear. Caloric test revealed canal paresis in left ear, but the labyrinth function was not totally lost. After the head-shaking, nystagmus (1st phase) is elicited to right (healthy side), then after the short latency period, nystagmus (2nd phase) is elicited to left (damaged side).
S. TAKAHASHI
S202
l'
PHASE
2
__ ,~___~~_~_~--~ - '~ JIO' l'
I sec
Fig. 2. b-HSN in group II. TT 12M (Case 14 in Table 1) suffered from Meniere's disease in right ear for IO years. Bilateral hearing loss which is more severe in right ear was observed. Caloric test revealed canal paresis in right ear, but the labyrinth function was not totally lost. After the head-shaking, nystagmus (1st phase) is elicited to right (damaged side), then after the 5hort latency period, nystagmus (2nd phase) is elicited to left (healthy side).
~
_ _ _.-JIIO'
tiD
sec
Fig. 3. b-HSN in group III. AS 71M (Case 17 in Table 1) suffered from inability of gait for a month and the diagnosis of spinocerebellar degeneration was made. He had bilateral hearing loss of almost the same degree in both ears. The caloric response was normal in both ears. Except for the fact that he had dysdiadochokinesis in his right hand, no remarkable lateralization of neurological symptom was found. After the head shaking, nystagmus (1st phase) was elicited to the right, then after the short latency period, nystagmus (2nd phase) was elicited to the left.
Patients in group II all suffered from Meniere's disease. Bilateral hearing loss which was more severe in the damaged side was observed in all cases. The caloric test revealed the canal paresis in the damaged side in all cases; however, there was no complete loss of labyrinthine function. In all cases of group III sensorineural hearing loss of almost the same degree
HEAD-SHAKING NYSTAGMUS
S 203
in both ears was observed. Caloric response was normal in all. Truncal ataxia was remarkable in all cases. Except for the fact that Case 17 showed dysdiadochokinesis in his right hand, there was no remarkable neurological lateralization in any of the cases. The electronystagmographic findings of b-HSN was surprisingly similar in all 3 groups, except for the mixture of many square wave jerks in the electronystagmogram (ENG) of group III. Namely, immediately after the head-shaking, strong, lively nystagmus occurs and regresses rapidly in ca. 5-15 sec (I st phase), then after a short latency period, a fine-beating and somewhat irregular nystagmus occurs to the opposite side and lasts over 30 sec (2nd phase). These are the common features of ENG in all 3 groups. As an example, the ENG of Case 7 from group I is shown in Fig. I. The ENG of Case 14 from group II is shown in Fig. 2. And the ENG of Case 17 from group III is shown in Fig. 3. DISCUSSION
The b-HSN of group I was considered to be equivalent to the b-HSN reported by KAMEl in 1975 from the point of view of direction of nystagmus. Kamei also described that the b-HSN was observed in cases of peripheral vestibular disturbance, especially unilateral disturbance, and that the function of the diseased labyrinth was not entirely lost. All cases of group I also had these features. For the origin of nystagmus Kamei considered the first phase as deficiency nystagmus and the second phase, recovery nystagmus. This interpretation could also be applied to the b-HSN of group I. In 1982, Spindler mentioned in his personal communication to Kamei (KAMEl et ai., 1984) that such b-HSN also exists where the first phase is directed toward the damaged side and the second phase toward the healthy side. Therefore, the b-HSN in group II corresponds to the b-HSN that Spindler previously pointed out. The cases of group II also suffered from unilateral peripheral vestibular disturbance and the function of the diseased labyrinth had not been entirely lost. Up to the present time, b-HSN has only been reported in cases of peripheral vestibular disturbance. However, this time b-HSN was observed in 3 cases of central vestibular disturbance. Because of the smaIl number of patients, the origin of the HSN in group II and group HI remains unknown. CONCLUSION
Through the electronystagmographic investigation of b-HSN, the author has confirmed the existence of some cases of b-HSN, which do not correspond
S204
S. TAKAHASHI
to the b-HSN previously reported by Kamei. In the majority of cases the second phase was directed toward the damaged side as Kamei had described, but in the minority of cases, in contrast to Kamei's report, the first phase was directed toward the damaged side. In addition, b-HSN was also observed in cases of central vestibular disturbance. I would like to thank Professor Tamio Kamei for his pertinent advice on the manuscript.
REFERENCES KAMEl, T.: Der biphasisch auftretende Kopfschuttelnystagmus. Arch. Otorhinolaryngol. 209: 59-67, 1975. KAMEl, T., KIMURA, K., KANEKO, H., and NORO, K.: Revaluation of the head-shaking test as a method of nystagmus provocation. Part 1: Its nystagmus eliciting effect. Jpn. J. Otol. (in Japanese) 67: 1530--1534, 1964. KAMEl, T., TAKAHASHI, S., KAMADA, H., and MUROI, M.: Revaluation of the head-shaking test as a method of nystagmus provocation. Part 2: Its diagnostic significance for the site of lesion. Equil. Res. (in Japanese) 43: 236-242, 1984.
Request reprints to:
Dr. S. Takahashi, Department of Otorhinolaryngology, School of Medicine, Gunma University, 3-39-15 Showamachi, Maebashi 371, Japan