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Sudden Deafness Due to Jugular Foramen Tumor
A.N.L.A., 1, 109-116, 1974
SUDDEN DEAFNESS DUE TO JUGULAR FORAMEN TUMOR -ITS NEUROTOLOGICAL FINDINGSMichihiko
M.D., Hajime WATANABE,* M.D., and Fuminori YAMADA,* M.D.
NOZUE,
Department of Oto-rhino-laryngo!ogy, Faculty of Medicine, University of Tokyo, Hongo , Bunkyo-ku, Tokyo, Japan *Department of Oto-rhino-laryngology, Tokyo Toritsu Bokulo Hospital, Sumida-ku, Tokyo, Japan
The origin of sudden deafness has as yet to be fully clarified and several causative factors have been suggested. This report concerns a case of sudden deafness resulting from a jugular foramen tumor and detailed neurotological findings in this case were documented. The neurotological examination included oto-rhino-Iaryngological examination, hearing tests, the caloric test, balance tests (Rom berg test, standing test on one foot and stepping test), the observation of nystagmus (gaze nystagmus, positional and positioning nystagmus), test of optokinetic nystagmus (OKN) and the neurological examination. In this particular case, the patient exhibited strong ataxia and staggering on the Romberg, standing test on one foot and stepping test. The hearing in the patient's left ear was completely abolished. Furthermore, the patient showed gaze nystagmus on both right and left lateral gaze, no caloric response on the left ear, strong inhibition of nystagmus on the OKN test, disturbances of the left V, VII and IX cranial nerves and cerebellar symptoms. These findings indicated the presence of a cerebellopontine angle tumor, specifically an acoustic neurinoma. In surgery, a large cerebellopontine angle tumor was found and this tumor pushed upon cranial nerveS VII and VIII. During the removal of this tumor, it was verified that it originated in the jugular foramen and that the internal auditory canal was intact. Several reports have mentioned sudden deafness resulting from a cerebellopontine angle tumor, however, the mechanism of sudden deafness in these cases has been obscure. In this case, sudden deafness presumably resulted from a vascular occlusion due to pressure of the tumor on the VIII cranial nerve and the labyrinthine artery. Received for publication August 25, 1973 109
110
M. NOZUE, H. WATANABE, and F. YAMADA
Several causative factors of sudden deafness have been suggested, however, the pathological processes have not as yet been fully clarified. This report concerns the case of a patient with a jugular foramen tumor and the initial symptom presented by this patient was the onset of sudden deafness. The following is a detailed account of the neurotological findings in this case. METHODS
The following examinations were performed: oto-rhino-laryngological examination, hearing tests, the caloric test, balance tests (Romberg test, standing test on one foot and stepping test), observation of nystagmus (gaze nystagmus, positional and positioning nystagmus), test of optokinetic nystagmus (OKN) and the neurological examination. Gaze nystagmus was observed during forward, right, left, upward and downward gaze. The amount of eye deviation to all gaze positions was about 30°. Positional nystagmus was observed by using Frenzel glasses. The patient was first placed in the supine position with the head held straight, then the patient's head was slowly rotated to the right and then to the left. The same procedure was repeated with the patient in the head-hanging position. Positioning nystagmus was also observed with Frenzel glasses. In this test, the patient was placed in a sitting position initially and then he was quickly brought to the supine position with the head hanging straight. This position was maintained for a moment and then the sitting position was resumed. Optokinetic stimuli were delivered by an electrically controlled rotating drum with the patient sitting inside the drum at the center. The dimensions of the drum were 1.5 m in diameter and 1.2 m in height. There were 12 black vertical stripes, each 3 em wide, drawn on the inside surface. The optokinetic pattern test shown in Fig. 2 is the result of a drum acceleration from 0° to 180° sec-I, with an angular acceleration of 4° sec- 2 • The drum was immediately decelerated to zero with an identical but negative acceleration. Nystagmus induced by optokinetic stimulation was recorded by electronystagmograph (ENG) at a paper speed of 0.1 em sec-I. Pen deflection upward is nystagmus to the right and downward pen deflection is nystagmus to the left. CASE
Case. T.K., a 23-year old chauffer, noticed sudden hearing loss in his left ear beginning in early October of 1969. He had had no previous episodes of hearing loss, tinnitus nor vertigo. One and a half months after the onset of his sudden deafness the patient noticed a slight dizziness and headache. On November 21, 1969 the patient visited the ENT clinic of Tokyo University and was diagnosed as a case of sudden deafness. At that time he was advised to undergo more detailed
SUDDEN DEAFNESS DUE TO JUGULAR FORAMEN TUMOR
111
examination as his initial neurotological examination indicated a possible central lesion. However, in that the patient's only complaint was a hearing loss in his left ear, he declined any further examination and continued with his employment. In January, 1971 he noticed that he had begun to occasionally choke on his food while eating, but this symptom caused him little concern. In the middle of November of 1971 the patient began to feel a disturbance with his gait, and as a result he visited the clinic of neurosurgery and ENT at Tokyo Toritsu Bokuto Hospital. He was suspected of suffering from a cerebellopontine angle tumor. Nov. 21, 1969
Dec. 14, 1971
Gaze Nystagmus
Posit ional Nystagmus head hanging
right side dawn
0 0
0 <----
"----'"
n n
~
n
<----
---+
<----
<----
left side down
supine
Posit ion ing Nystag mils head hanging straight
[Ql3-4X ~5_6X
OJ
2x
[ [ ] 5-6x
sitting with head straight
Fig. 1. On the left side of this figure are shown the nystagmus findings during the patient first visit to the clinic. Gaze nystagmus to the right and to the left was observed. On the right side of the figure are shown the nystagmus findings approximately two years after the first examination. Gaze nystagmus to the right and to the left was noted. Also, direction changing nystagmus in the positional nystagmus test and vertical nystagmus in the positioning nystagmus test were noted. Arrows indicate the direction of the quick phase of nystagmus, circles indicate no nystagmus and a circle around the arrows indicates vertiginous sensation. The numbers in the positioning nystagmus test are the number of nystagmic beats seen during the test.
M. NOZUE, H. WATANABE, and F. YAMADA
112
Results of neurotological examination
On November 21, 1969, during his first examination, it was found that the hearing in his left ear was totally abolished. The Romberg test and the standing test on one foot with the eyes closed indicated a slight falling tendency. During the stepping test staggering was noticed. The test for gaze nystagmus showed combined horizontal and rotatory nystagmus beating toward the side of lateral gaze. Positional and positioning nystagmus was seen, either of purely rotatory type or combined rotatory and horizontal nystagmus as illustrated in Fig. 1. Caloric responses were preserved equally in both ears. Optokinetic nystagmus (OKN) to the right was fairly normal, however OKN to the left seemed to be slightly inhibited as shown in Fig. 2. Neurological examination revealed a disturbance in movement of the left soft palate. Disdiadochokinesis was noticed on the left hand and the finger-to finger test was also poorly executed. Nov. 21 , 1969
Dec. 14, 1971 .f.".~J"l"l.n .. tLl.il.iilii'htWi .. iHi.L"lIIiill.hMili",,' ....:!) .• I ...
10'
R-DIN
L-Oll
-m________~,"--~,~II--------_,~. 10 sec.
10'
R-DIN
L-DIN
. . . . . . . .~I,----·_mIl......~~,P 10 sec.
~~ I
Fig. 2. The left side of this figure shows the patient's OKN during his first visit to the clinic. OKN to the left is slightly reduced. The right side of the figure show the OKN approximately two years after the first examination. OKN to the right is infrequent and irregular and OKN to the left is strongly inhibited. Four traces are shown in the figure: the top trace is the paper speed; the second shows the direct ENG recording (time constant 3 sec); third is the differenciated recording (time constant 0.03 sec); bottom tracing is the recording of photo cell response indicating the movement of the stripes on the OKN drum. R-OKN indicates drum rotation to the left inducing nystagmus to the right. L-OKN indicates drum rotation to the right inducing nystamus to the left.
On December 14, 1971, during the second examination, it was found that the patient's hearing had remained unchanged. The Romberg test was positive and the standing test on one foot showed stronger falling tendency than during the first examination. Also, in the stepping test greater degree of staggering was noticed. The test for gaze nystagmus revealed large amplitude, low frequency horizontal nystagmus to the left on left lateral gaze and small amplitude, high
SUDDEN DEAFNESS DUE TO JUGULAR FORAMEN TUMOR
113
frequency nystagmus to the right on right lateral gaze (Fig. 1). Positional testing showed direction changing nystagmus and the positioning test showed vertical nystagmus (Fig. 1). Ice water caloric irrigations were performed bilaterally; the reaction on the right ear was preserved and the left ear gave no response. The OKN test gave infrequent and irregular responses, and especially the OKN to the left was strongly inhibited as shown in Fig. 2. Neurological examinations revealed distrubances of left facial sensation, corneal reflex and taste sensation. Movement of the left soft palate was completely paralysed and the gag reflex was abolished. Left disdiadochokinesis was again noticed and the finger-to-finger test was poorly executed. The skull X-rays were unremarkable. Prior to surgery, complete radiological examinations, such as angiogram and pneumoencephalogram, were performed. Surgery
Left suboccipital craniectomy was performed and a large cerebellopontine angle tumor, pushing upon nerves VII, VIII and some lower cranial nerves, was found as illustrated in Fig. 3. Following the removal of this tumor, it was ascertained that this tumor originated from the jugular foramen, presumably at cranial nerve IX, and that the internal auditory canal was intact (Fig. 4). Pathological examination of the tumor identified it as a neurinoma Antoni A type.
Fig. 3. This figure is a schematic illustration of the exposed tumor just prior to surgical removal. The tumor is pushing upon VII, VIII, IX and some lower cranial nerves.
114
M. NOZUE, H. WATANABE, and F. YAMADA
Fig. 4. This figure is a schematic illustration of the surgical field after removal of the tumor. The internal acoustic meatus is intact and the remains of the tumor can be seen lying within the jugular foramen.
COMMENT
Several possible factors have been suggested concerning the etiology of sudden deafness, i.e.: 1) vascular lesions such as hemorrhage, thrombosis or spasm; 2) viral infections; 3) allergy and others. Sudden deafness resulting from a cere bellopontine angle tumor is rare. CUSHING (1917) mentioned two cases of sudden deafness which resulted from cerebellopontine angle tumors. MEYER (1941), EDWARDS and PATERSON (1951) and HALLBERG et al. (1959) also described cases of cerebellopontine angle tumors in which the loss of hearing appeared abruptly. In fact, the mechanism of sudden deafness in these cases has as yet to be fully clarified, however vascular occlusion due to pressure of the tumor on the internal auditory canal can be suggested. In this particular case, during the patient's first examination consideration was given to the presence of a central lesion. This consideration was based on the findings of staggering on the stepping test, gaze nystagmus to the right and left, OKN inhibition to the left, disturbance of movement of the left soft palate and cerebellar symptoms. However, because the patient complained only of hearing loss in his left ear, he did not return for the second examination until about two years after the first examination. In the second examination stronger ataxia and staggering were noticed on the Romberg test, standing test on one foot and
SUDDEN DEAFNESS DUE TO JUGULAR FORAMEN TUMOR
115
on the stepping test. Furthermore, the patient showed gaze nystagmus to the right and to the left, no caloric response on the left ear, strong inhibition on the OKN test, disturbances of the left V, VII, VIII and IX cranial nerves and cerebellar symptoms. These findings indicated the presence of a cerebellopontine angle tumor, specifically an acoustic neurinoma. In surgery, a large jugular foramen tumor was found as mentioned above. This was an unusual case because this tumor did not produce the usual jugular foramen syndrome, but rather showed the symptoms of an acoustic neurinoma. ARENBERG and McCREARY (1971) mentioned in their paper that a neurilemmoma of the jugular foramen could be mistaken clinically for an acoustic neurilemmoma, in the abscence of a jugular foramen-like syndrome. In this case also, the signs and symptoms of V, VII and VIII cranial nerves and the cerebellar symptoms were manifest and the jugular foramen syndrome was rather poorly developed. These findings were consistent with the pre-operative diagnosis of a cerebellopontine angle tumor. It is interesting to note that these signs and symptoms seemed to be the result of compression by the tumor, and sudden deafness was the initial symptom from this patient. Sudden deafness, in this case, presumably might have come from vascular occlusion due to pressure on the VIII cranial nerve and the labyrinthine artery. Postoperative examination of this patient showed no recovery of hearing nor caloric response in the left ear, however his staggering and other symptoms have greatly diminished. SUMMARY
A case of sudden deafness due to a jugular foramen tumor has been documented with detailed neurotological findings. From neurotological findings the presence of a cerebellopontine angle tumor, specifically an acoustic neurinoma was suggested. In surgery, a large cerebellopontine angle tumor was found and this tumor pushed upon cranial nerves VII and VIII and some of the lower cranial nerves. Following the removal of this tumor, it was ascertained that its origin was from the jugular foramen and that the internal auditory canal was intact. Generally, the mechanism of sudden deafness due to a cerebellopontine angle tumor has been obscure. However, in this case, sudden deafness might presumably have originated from vascular occlusion due to pressure on the VIII cranial nerve and the labyrinthine artery. We wish to thank Professor Yasuo Sato, Head of the Department of Tokyo University, neurosurgeons of Tokyo Toritsu Bokuto Hospital for their assistance and Mr. Dominic W. Hughes for his help in English compilation.
116
M. NOZUE, H. WATANABE, and F. YAMADA REFERENCES
ARENBERG, I. K. and MCCREARY, H. S. : Neurilemmoma of the jugular foramen. Laryngoscope 4: 544-557, 1971. CUSHING, H. W.: Tumors of Nervus Acusticus and the Syndrome of the Cerebellopontine Angle. Philadelphia, W.B. Saunders Company, 1917. EDWARDS, C. H. and PATERSON, J. H.: Review of symptoms and signs of acoustic neurofibromata. Brain. 74: 144-190, 1951. HALLBERG, O. E., VIHLEIN, A. and SIEKERT, R. G . : Sudden deafness due to cerebellopontineangle tumor. Arch. Otolaryng. 69: 160-162, 1959. MEYER, B. C. : Neoplasm of posterior fossa stimulating cerebral vascular disease: Report of 5 cases with reference to role of medulla in production of arterial hypertension. Arch. Neurol. Psychiat. 45: 468-480, 1941. Request reprints from: Dr. M. NOZUE, Department of Otolaryngology, Faculty of Medicine, University of Tokyo, Hongo, Bunkyo-ku, Tokyo, Japan.
SUDDEN DEAFNESS DUE TO JUGULAR FORAMEN TUMOR -ITS NEUROTOLOGICAL FINDINGSMichihiko
M.D., Hajime WATANABE,* M.D., and Fuminori YAMADA,* M.D.
NOZUE,
Department of Oto-rhino-laryngo!ogy, Faculty of Medicine, University of Tokyo, Hongo , Bunkyo-ku, Tokyo, Japan *Department of Oto-rhino-laryngology, Tokyo Toritsu Bokulo Hospital, Sumida-ku, Tokyo, Japan
The origin of sudden deafness has as yet to be fully clarified and several causative factors have been suggested. This report concerns a case of sudden deafness resulting from a jugular foramen tumor and detailed neurotological findings in this case were documented. The neurotological examination included oto-rhino-Iaryngological examination, hearing tests, the caloric test, balance tests (Rom berg test, standing test on one foot and stepping test), the observation of nystagmus (gaze nystagmus, positional and positioning nystagmus), test of optokinetic nystagmus (OKN) and the neurological examination. In this particular case, the patient exhibited strong ataxia and staggering on the Romberg, standing test on one foot and stepping test. The hearing in the patient's left ear was completely abolished. Furthermore, the patient showed gaze nystagmus on both right and left lateral gaze, no caloric response on the left ear, strong inhibition of nystagmus on the OKN test, disturbances of the left V, VII and IX cranial nerves and cerebellar symptoms. These findings indicated the presence of a cerebellopontine angle tumor, specifically an acoustic neurinoma. In surgery, a large cerebellopontine angle tumor was found and this tumor pushed upon cranial nerveS VII and VIII. During the removal of this tumor, it was verified that it originated in the jugular foramen and that the internal auditory canal was intact. Several reports have mentioned sudden deafness resulting from a cerebellopontine angle tumor, however, the mechanism of sudden deafness in these cases has been obscure. In this case, sudden deafness presumably resulted from a vascular occlusion due to pressure of the tumor on the VIII cranial nerve and the labyrinthine artery. Received for publication August 25, 1973 109
110
M. NOZUE, H. WATANABE, and F. YAMADA
Several causative factors of sudden deafness have been suggested, however, the pathological processes have not as yet been fully clarified. This report concerns the case of a patient with a jugular foramen tumor and the initial symptom presented by this patient was the onset of sudden deafness. The following is a detailed account of the neurotological findings in this case. METHODS
The following examinations were performed: oto-rhino-laryngological examination, hearing tests, the caloric test, balance tests (Romberg test, standing test on one foot and stepping test), observation of nystagmus (gaze nystagmus, positional and positioning nystagmus), test of optokinetic nystagmus (OKN) and the neurological examination. Gaze nystagmus was observed during forward, right, left, upward and downward gaze. The amount of eye deviation to all gaze positions was about 30°. Positional nystagmus was observed by using Frenzel glasses. The patient was first placed in the supine position with the head held straight, then the patient's head was slowly rotated to the right and then to the left. The same procedure was repeated with the patient in the head-hanging position. Positioning nystagmus was also observed with Frenzel glasses. In this test, the patient was placed in a sitting position initially and then he was quickly brought to the supine position with the head hanging straight. This position was maintained for a moment and then the sitting position was resumed. Optokinetic stimuli were delivered by an electrically controlled rotating drum with the patient sitting inside the drum at the center. The dimensions of the drum were 1.5 m in diameter and 1.2 m in height. There were 12 black vertical stripes, each 3 em wide, drawn on the inside surface. The optokinetic pattern test shown in Fig. 2 is the result of a drum acceleration from 0° to 180° sec-I, with an angular acceleration of 4° sec- 2 • The drum was immediately decelerated to zero with an identical but negative acceleration. Nystagmus induced by optokinetic stimulation was recorded by electronystagmograph (ENG) at a paper speed of 0.1 em sec-I. Pen deflection upward is nystagmus to the right and downward pen deflection is nystagmus to the left. CASE
Case. T.K., a 23-year old chauffer, noticed sudden hearing loss in his left ear beginning in early October of 1969. He had had no previous episodes of hearing loss, tinnitus nor vertigo. One and a half months after the onset of his sudden deafness the patient noticed a slight dizziness and headache. On November 21, 1969 the patient visited the ENT clinic of Tokyo University and was diagnosed as a case of sudden deafness. At that time he was advised to undergo more detailed
SUDDEN DEAFNESS DUE TO JUGULAR FORAMEN TUMOR
111
examination as his initial neurotological examination indicated a possible central lesion. However, in that the patient's only complaint was a hearing loss in his left ear, he declined any further examination and continued with his employment. In January, 1971 he noticed that he had begun to occasionally choke on his food while eating, but this symptom caused him little concern. In the middle of November of 1971 the patient began to feel a disturbance with his gait, and as a result he visited the clinic of neurosurgery and ENT at Tokyo Toritsu Bokuto Hospital. He was suspected of suffering from a cerebellopontine angle tumor. Nov. 21, 1969
Dec. 14, 1971
Gaze Nystagmus
Posit ional Nystagmus head hanging
right side dawn
0 0
0 <----
"----'"
n n
~
n
<----
---+
<----
<----
left side down
supine
Posit ion ing Nystag mils head hanging straight
[Ql3-4X ~5_6X
OJ
2x
[ [ ] 5-6x
sitting with head straight
Fig. 1. On the left side of this figure are shown the nystagmus findings during the patient first visit to the clinic. Gaze nystagmus to the right and to the left was observed. On the right side of the figure are shown the nystagmus findings approximately two years after the first examination. Gaze nystagmus to the right and to the left was noted. Also, direction changing nystagmus in the positional nystagmus test and vertical nystagmus in the positioning nystagmus test were noted. Arrows indicate the direction of the quick phase of nystagmus, circles indicate no nystagmus and a circle around the arrows indicates vertiginous sensation. The numbers in the positioning nystagmus test are the number of nystagmic beats seen during the test.
M. NOZUE, H. WATANABE, and F. YAMADA
112
Results of neurotological examination
On November 21, 1969, during his first examination, it was found that the hearing in his left ear was totally abolished. The Romberg test and the standing test on one foot with the eyes closed indicated a slight falling tendency. During the stepping test staggering was noticed. The test for gaze nystagmus showed combined horizontal and rotatory nystagmus beating toward the side of lateral gaze. Positional and positioning nystagmus was seen, either of purely rotatory type or combined rotatory and horizontal nystagmus as illustrated in Fig. 1. Caloric responses were preserved equally in both ears. Optokinetic nystagmus (OKN) to the right was fairly normal, however OKN to the left seemed to be slightly inhibited as shown in Fig. 2. Neurological examination revealed a disturbance in movement of the left soft palate. Disdiadochokinesis was noticed on the left hand and the finger-to finger test was also poorly executed. Nov. 21 , 1969
Dec. 14, 1971 .f.".~J"l"l.n .. tLl.il.iilii'htWi .. iHi.L"lIIiill.hMili",,' ....:!) .• I ...
10'
R-DIN
L-Oll
-m________~,"--~,~II--------_,~. 10 sec.
10'
R-DIN
L-DIN
. . . . . . . .~I,----·_mIl......~~,P 10 sec.
~~ I
Fig. 2. The left side of this figure shows the patient's OKN during his first visit to the clinic. OKN to the left is slightly reduced. The right side of the figure show the OKN approximately two years after the first examination. OKN to the right is infrequent and irregular and OKN to the left is strongly inhibited. Four traces are shown in the figure: the top trace is the paper speed; the second shows the direct ENG recording (time constant 3 sec); third is the differenciated recording (time constant 0.03 sec); bottom tracing is the recording of photo cell response indicating the movement of the stripes on the OKN drum. R-OKN indicates drum rotation to the left inducing nystagmus to the right. L-OKN indicates drum rotation to the right inducing nystamus to the left.
On December 14, 1971, during the second examination, it was found that the patient's hearing had remained unchanged. The Romberg test was positive and the standing test on one foot showed stronger falling tendency than during the first examination. Also, in the stepping test greater degree of staggering was noticed. The test for gaze nystagmus revealed large amplitude, low frequency horizontal nystagmus to the left on left lateral gaze and small amplitude, high
SUDDEN DEAFNESS DUE TO JUGULAR FORAMEN TUMOR
113
frequency nystagmus to the right on right lateral gaze (Fig. 1). Positional testing showed direction changing nystagmus and the positioning test showed vertical nystagmus (Fig. 1). Ice water caloric irrigations were performed bilaterally; the reaction on the right ear was preserved and the left ear gave no response. The OKN test gave infrequent and irregular responses, and especially the OKN to the left was strongly inhibited as shown in Fig. 2. Neurological examinations revealed distrubances of left facial sensation, corneal reflex and taste sensation. Movement of the left soft palate was completely paralysed and the gag reflex was abolished. Left disdiadochokinesis was again noticed and the finger-to-finger test was poorly executed. The skull X-rays were unremarkable. Prior to surgery, complete radiological examinations, such as angiogram and pneumoencephalogram, were performed. Surgery
Left suboccipital craniectomy was performed and a large cerebellopontine angle tumor, pushing upon nerves VII, VIII and some lower cranial nerves, was found as illustrated in Fig. 3. Following the removal of this tumor, it was ascertained that this tumor originated from the jugular foramen, presumably at cranial nerve IX, and that the internal auditory canal was intact (Fig. 4). Pathological examination of the tumor identified it as a neurinoma Antoni A type.
Fig. 3. This figure is a schematic illustration of the exposed tumor just prior to surgical removal. The tumor is pushing upon VII, VIII, IX and some lower cranial nerves.
114
M. NOZUE, H. WATANABE, and F. YAMADA
Fig. 4. This figure is a schematic illustration of the surgical field after removal of the tumor. The internal acoustic meatus is intact and the remains of the tumor can be seen lying within the jugular foramen.
COMMENT
Several possible factors have been suggested concerning the etiology of sudden deafness, i.e.: 1) vascular lesions such as hemorrhage, thrombosis or spasm; 2) viral infections; 3) allergy and others. Sudden deafness resulting from a cere bellopontine angle tumor is rare. CUSHING (1917) mentioned two cases of sudden deafness which resulted from cerebellopontine angle tumors. MEYER (1941), EDWARDS and PATERSON (1951) and HALLBERG et al. (1959) also described cases of cerebellopontine angle tumors in which the loss of hearing appeared abruptly. In fact, the mechanism of sudden deafness in these cases has as yet to be fully clarified, however vascular occlusion due to pressure of the tumor on the internal auditory canal can be suggested. In this particular case, during the patient's first examination consideration was given to the presence of a central lesion. This consideration was based on the findings of staggering on the stepping test, gaze nystagmus to the right and left, OKN inhibition to the left, disturbance of movement of the left soft palate and cerebellar symptoms. However, because the patient complained only of hearing loss in his left ear, he did not return for the second examination until about two years after the first examination. In the second examination stronger ataxia and staggering were noticed on the Romberg test, standing test on one foot and
SUDDEN DEAFNESS DUE TO JUGULAR FORAMEN TUMOR
115
on the stepping test. Furthermore, the patient showed gaze nystagmus to the right and to the left, no caloric response on the left ear, strong inhibition on the OKN test, disturbances of the left V, VII, VIII and IX cranial nerves and cerebellar symptoms. These findings indicated the presence of a cerebellopontine angle tumor, specifically an acoustic neurinoma. In surgery, a large jugular foramen tumor was found as mentioned above. This was an unusual case because this tumor did not produce the usual jugular foramen syndrome, but rather showed the symptoms of an acoustic neurinoma. ARENBERG and McCREARY (1971) mentioned in their paper that a neurilemmoma of the jugular foramen could be mistaken clinically for an acoustic neurilemmoma, in the abscence of a jugular foramen-like syndrome. In this case also, the signs and symptoms of V, VII and VIII cranial nerves and the cerebellar symptoms were manifest and the jugular foramen syndrome was rather poorly developed. These findings were consistent with the pre-operative diagnosis of a cerebellopontine angle tumor. It is interesting to note that these signs and symptoms seemed to be the result of compression by the tumor, and sudden deafness was the initial symptom from this patient. Sudden deafness, in this case, presumably might have come from vascular occlusion due to pressure on the VIII cranial nerve and the labyrinthine artery. Postoperative examination of this patient showed no recovery of hearing nor caloric response in the left ear, however his staggering and other symptoms have greatly diminished. SUMMARY
A case of sudden deafness due to a jugular foramen tumor has been documented with detailed neurotological findings. From neurotological findings the presence of a cerebellopontine angle tumor, specifically an acoustic neurinoma was suggested. In surgery, a large cerebellopontine angle tumor was found and this tumor pushed upon cranial nerves VII and VIII and some of the lower cranial nerves. Following the removal of this tumor, it was ascertained that its origin was from the jugular foramen and that the internal auditory canal was intact. Generally, the mechanism of sudden deafness due to a cerebellopontine angle tumor has been obscure. However, in this case, sudden deafness might presumably have originated from vascular occlusion due to pressure on the VIII cranial nerve and the labyrinthine artery. We wish to thank Professor Yasuo Sato, Head of the Department of Tokyo University, neurosurgeons of Tokyo Toritsu Bokuto Hospital for their assistance and Mr. Dominic W. Hughes for his help in English compilation.
116
M. NOZUE, H. WATANABE, and F. YAMADA REFERENCES
ARENBERG, I. K. and MCCREARY, H. S. : Neurilemmoma of the jugular foramen. Laryngoscope 4: 544-557, 1971. CUSHING, H. W.: Tumors of Nervus Acusticus and the Syndrome of the Cerebellopontine Angle. Philadelphia, W.B. Saunders Company, 1917. EDWARDS, C. H. and PATERSON, J. H.: Review of symptoms and signs of acoustic neurofibromata. Brain. 74: 144-190, 1951. HALLBERG, O. E., VIHLEIN, A. and SIEKERT, R. G . : Sudden deafness due to cerebellopontineangle tumor. Arch. Otolaryng. 69: 160-162, 1959. MEYER, B. C. : Neoplasm of posterior fossa stimulating cerebral vascular disease: Report of 5 cases with reference to role of medulla in production of arterial hypertension. Arch. Neurol. Psychiat. 45: 468-480, 1941. Request reprints from: Dr. M. NOZUE, Department of Otolaryngology, Faculty of Medicine, University of Tokyo, Hongo, Bunkyo-ku, Tokyo, Japan.