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Polyarteritis nodosa as a cause of sudden deafness
Polyarteritis Nodosa as a Cause of Sudden Deafness A Human Temporal Bone Study HERMAN A. JENKINS, M.D.,* ANITA M. POLLAK,M.D.,~" AND UGO FISCH, M.D.:~
Pathologic changes in the temporal bone are described in a case of polyarteritis nodosa in a 48 year old man in whom the onset of sudden unilateral deafness and vertigo occurred seven months prior to death as one of the early manifestations of the disease. The patient had received only a seven week course of prednisolone and, earlier, a two week course of anti-inflammatory agents. Autopsy revealed involvement of the arteries supplying the kidneys, testes, and pancreas. Changes within the temporal bone were seen bilaterally and there was thickening of the mucosa of the middle ear. Inner ear involvement was m a i n l y limited to the cochlea, the deaf ear showing more pronounced changes. These c h a n g e s included loss of the organ of Corti in the hook portion of the basal coil, absence of t h e tectorial membrane, and atrophy of the stria vascularis. The scala tympani was obliterated by fibrosis and new bone formation. The scala media showed hydrops, and a marked decrease in the spiral ganglion cells and nerve fibers supplying this portion of the cochlea was evident. Focal changes were seen t h r o u g h o u t the remainder of the cochlea. The vestibular structures showed no detectable pathologic changes. Small vessel ar~eritis was found in the dural and subarcuate vessels in both temporal bones.
Evidence that polyarteritis nodosa might be a cause of deafness first appeared in the literature in a case report by McNeil e t a l . ~ in 1952 in w h i c h tinnitus and deafness occurred two years after the onset of symptoms. These authors also m a d e reference to a previous work by Foster and M a l a m u d 2 in which two patients from their series of 300 patients with vasculitis had concernRant deafness. Two additional cases of polyarteritis nodosa were presented by Peitersen and Carlsen a in which aural complaints were the pres e n t i n g symptoms of the disease. Only the first case, however, would be considered as polyarteritis nodosa under the presently accepted classification of the disease.
The first pathologic description of temporal bone involvement with polyarteritis nodosa was reported by Gussen "tin a case in w h i c h the hearing loss occurred approximately two years after the appearance of the initial symptoms of the disease. This patient had the typical history of pclyarteritis nodosa, complicated by hypertension and cardiac and renal failure. She had also received treatment with m a n y Lrnmunosuppressive, anti-inflammatory, and diuretic drugs. In this article the pathologic fir~dings in the temporal bones are presented in a patient with polyarteritis nodosa in w h o m the aural complaints were among the initial presenting symptoms, with death ensuing seven months after the
Accepted for publication January 28, 1981. Dr. Jenkins is a Clinical Fellow of the Department of SL~rgery,University af California, Los Angeles, School of Medicine, Los Angeles, California. His work was supported in part by Teacher-Investigator Award 00294 from the Notional Institutes of Health. *Assistant Prufessor of Surgery, University of California, Los Angeles, Medical Center, Los Angeles, California. tReseaz'ch Fellow, ENT Department, University Hospital, Zurich, Switzerland. ~:Professor and Head of ENT Department, University Hospital, Zurich, Switzerland. American Journal of OLalaryngology--Volume 2, Number 2, May 1981
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onset of deafness. The patient received therapeutic dosages of anti-inflammatory agents as a treatment for possible collagenosis only during the seven weeks prior to death. The diagnosis of polyarteritis n o d o s a was not established until necropsy,
CASE
REPORT
A 48 y e a r old w h i t e male f a c t o r y worker s o u g h t initial treatment from his private physician i n June 1962 for a persistent upper respiratory infection of several m o n t h s ' duration. There h a d been no associated fever or other general symptoms, a n d prior to this he had been in excellent health. A few weeks later he developed p h a r y n g i t i s and a p e r i o d o n t a l abscess with spontaneous extrusion of a tooth. In November 1962 he developed severe pain in the left leg along with s u d d e n dizziness, nausea, vomiting, and deafness in the left ear a n d decreased hearing in the right ear. The pain in the legs and the dizziness subsided after two weeks, but the hearing loss persisted. Three months later he developed a low grade fever and arthralgias of nearly all the large and small joints of the body with swelling, erythema, and warmth. These symptoms subsided after a two week course of treatm e n t with p h e n y l b u t a z o n e a n d penicillin. Two weeks later he again developed dizziness with tinnitus and bitemporal headaches. A neurologic evaluation y i e l d e d essentially negative findings, and four m o n t h s after the initial aural s y m p t o m s h a d a p p e a r e d he was referred to the ENT Department of the University of Zurich for further evaluation. Audiometric studies showed complete deafness on the left side w i t h the combination on the right side of a m i l d conductive and a moderate sloping high frequency sensorineural hearing loss. Caloric testing s h o w e d a nonfunctioning labyrinth on the left. Paracentesis of the right m i d d l e ear p r o v i d e d immediate improvement of the conductive c o m p o n e n t of the hearing loss. The secretions of the middle ear were described as yellow and tenacious. No bacteriologic studies were performed. The patient's g e n e r a l s y m p t o m s increased w i t h the appearance of a chronic olecranon bursitis, w h i c h was histologically nonspecific on biopsy. He h a d increasing facial edema, oliguria, and hematuria w i t h acute abdominal pain and required hospitalization. Treatment with prednisoione was begun at another hospital several weeks prior to his transfer to the University Hosi00
pital for further diagnostic work-up and treatment. On admission in May 1963 the patient was normotensive (135/80 ram. Hg). Laboratory evaluation showed a hemoglobin level of 9.8 mg. per 100 ml. and an erythrocyte sedimentation rate of 64 ram. per hr. The white cell count was 12,800 per cu. ram. with a shift to the left and an absolute eosinophilia of 684. The residual nitrogen was 196 rag. per 100 m]., blood urea nitrogen 264 rag. per 100 ml., uric acid 16.8 mg. per 1(]0 ml., and creatinine 12.5 rag. per 100 ml. Urinalysis showed a 0.8 gin. per 1000 ml. protein concentration with macroscopic hematuria and cylindrical casts. Cultures of the urine showed no growth. Serologic testing for syphilis was negative, as was the antiglobulin c o n s u m p t i o n test with cell nuclei. The antistreptolysin titer was not elevated. A muscle biopsy showed no evidence of vasculitis and a renal biopsy specimen was interpreted as s h o w i n g chronic pyelonephritis. The patient underwent peritoneal dialysis on three occasions. He received a total course of seven weeks of prednisolone at a dosage of 40 mg. per day. Before further definitive work-up could proceed, the patient had respiratory failure and died, secondary to uremic pneumonia.
PATHOLOGIC
STUDY
The general pathologic changes were limited to the small arteries supplying the kidneys, pancreas, and testes. These changes were similar to those described in the following section. In addition, the kidneys showed features of advanced membraneous glomerulonephritis with hyalinization of m a n y glomeruli. The testes contained focal areas of acute and chronic hemorrhagic infarction secondary to arteriolar obstruction. The other major organs of the body did not show any pathologic changes.
Temporal Bone LEFT EAR. The left temporal bone was decalcified and embedded in celloidin. It was cut in vertical sections 20 microns in thickness, with orientation at a right angle to the longitudinal axis of the petrous bone. The sections were stained with hematoxylin and eosin and every tenth section was studied. Additional special stains for myelin and elastic fibers were used. Focal perivascular infiltration of inflmnmatory
POLYARTERITISNODOSAAS A CAUSE OF SUDDENDEAFNESS
Volume 2 Number 2 May 1981
Figure 1. Photomicrograph of the skin lining the external auditory canal. Arteritis of the small vessels is prominent.
Figure 2. Photomicrograph of the mucosal lining of the middle ear. Note the hypertrophic changes in the submucosal layer with round cell infiltration, particularly in the perivascular areas.
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Figure 3.
P h o t o m i c r o g r a p h of the r o u n d w i n d o w niche with obliteration by hypertrophic fibrous tissue.
cells was present in the subcutaneous layer of the skin lining the b o n y external auditory canal (Fig. 1). The m i d d l e ear and mastoid air spaces s h o w e d marked thickening of the submucosal layer with a highly cellular connective tissue, infiltrated t h r o u g h o u t with chronic inflammatory cells concentrated mainly in the perivascular areas (Fig. 2). The round w i n d o w niche was filled with a mass of areolar connective tissue that obliterated the entire niche and exhibited pericapillary infiltration with chronic inflammatory cells (Fig. 3). The ossicles of the m i d d l e e a r were normal, including the joint spaces, but were e m b e d d e d in a thick layer of connective tissue, similar to the connective tissue of the s u b m u c o s a l layer. The tensor tympani and stapedius muscles were normal. In the h o o k p o r t i o n of the basal coil of the cochlea there was a t r o p h y of the stria vascularis with absence of the organ of Corti and the tec-
torial membrane (Fig. 4). The ceils of the limbus appeared normal. Reissner's m e m b r a n e was intact but bulged into the scala vestibuli. The entire scala tympani in this area of the cochlea was filled with disorganized connective tissue with areas of n e w bone formation. The spiral ligament was thickened and continuous with the co.nnectire tissue in the scala tympani. There was a marked loss of nerve fibers and spiral ganglion cells in this portion of the cochlea. The remaining cochlea s h o w e d a d h e r e n c e of Reissner's membrane to the limbus and tectorial membrane with the free portion bulging slightly into the scala vestibuli. There was focal atrophy of the stria vascularis and isolated areas of collapse of the organ of Corti throughout the cochlea, particularly in the apical coil. The r e m a i n d e r of the scala t y m p a n i s h o w e d n o c o n n e c t i v e tissue obliteration, and the spiral ganglion cells and nerve fibers appeared normal. There were occa-
Figure 4. A, P h o t o m i c r o g r a p h of the m i d m o d i o l a r view of the cochlea of the left ear. B, Higher power view of the hook portion of the basal coil. Note the fibrosis and n e w bone formation obliterating the scala t y m p a n i w i t h absence of the organ of Corti and tectorial m e m b r a n e and the decrease in the nerve b u n d l e s and spiral ganglion cells to the basal coil.
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Figure 4.
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Figure 5. A, Photomicrograph of the dura with periarterial infiltration and fibrinoid necrosis of the vessel wall. B, Photomicrograph showing similar changes in the subarcuate vessels of the ]eft temporal bone.
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POLYARTERITIS NODOSA AS A CAUSE OF SUDDEN DEAFNESS
sional connective tissue synechiae within the scala vestibuli of the middle coil, adjacent to the hook portion of the basal coil of the cochlea. No detectable pathologic changes were present in the vestibular structures. The nerves within the internal auditory canal were normal, as was the dura. Several small arteries were present in the internal auditary canal without a n y pathologic changes. The dura of the posterior fossa was markedly thickened with a dense, well organized collagenous tissue and" showed perivascular infiltration of the small arterioles with fibrinoid necrosis in the walls and fibrocytic proliferation at the periphery of the vessels (Fig. 5A). The lumen was almost completely obliterated in many of these vessels. Similar changes were found in the vessels of the suba r c u a t e fossa (Fig. 5B). The same c h r o n i c i n f l a m m a t o r y cell i n f i l t r a t i o n was p r e s e n t around the venules without compromise of their lumens. RIGHT EAR. The right ear was cut in horizontal sections 20 microns in thickness. The sections were stained with hemotoxylin and eosin and every tenth section was studied, The pathologic changes in the external auditory canal,
middle ear, mastoid, and tensor t y m p a n i muscle were identical to those described in the left ear. The cochlea showed focal areas of atrophy of the stria vascularis throughout that appeared to be more pronounced in the basal coil. The number of spiral ganglion cells and nerve fibers supplying the hook portion of the basal coil of the cochlea was markedly decreased. The remainder of the cochlear and vestibular structures of the inner ear were normal. The dura of the middle and posterior fossae was normal in thickness but showed an inflammatory perivascular infiltration and fibrinoid necrosis in a few of the arterioles seen. The same vascular changes were found in the subarcuate vessels as described in the left ear (Fig. 6). Only small caliber arteries were present in the internal auditory canal and t h e y w e r e w i t h o u t e v i d e n c e of p a t h o l o g i c change.
Volume 2 Number 2 May 1981
DISCUSSION
Po]yarteritis nodosa is a systemic vasculitis of unknown etiology, involving mainly small and middle caliber arteries. The lesions are s e g m e n -
Figure 6. Photomicrographof subarcuate vessels of the right temporal bone showing perivascular infiltration and fibrinoid necrosis. The lumen of the artery is almost completelyobliterated. HERMANA. JENKINSET AL.
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tal with a predilection for the bifurcations of the vessels. The disease process spreads longitudinally along the arterioles and eventually involves accompanying venules. Fauci 5 called this pattern of spreading of the lesions to the arterioles, venules, and capillaries an "overlap syndrome," which he considered to be a subgroup of classic polyarterifis nodosa. In the acute state the characteristic pathologic change is a polymorphonuclear cellular infiltration of all three layers of the vessel wall and the perivascular area. In the later stages the vascular and perivascular infiltration changes from the polymorphonuclear cells of the acute stage to a chronic lymphoplasmic cellular infiltrate. The vessels undergo fibrinoid necrosis, mainly in the media layer, with loss of elastic fibers in the elastic membrane of the arterial wall. These changes lead to scarring in the involved vessels. In the classic form of polyarteritis all three stages may occur simultaneously. Typically the distribution of the disease in the body spares the small vessels of the lungs and spleen. According to the criteria of Zeek, ~ localization of the arterial lesions in the present case to the kidneys, pancreas, and testes, along with associated microscopic findings described, classifies this patient as a typical case of polyarteritis nodosa. The changes found in the cochlea in this case are similar to those reported by several authors in experimental work with compromise of the arterial supply of the inner ear. Loss of the organ of Corti and strial changes were reported by Kimttra and Perlman 7, Alford et el. s Igarashi et el. 0 and Bernstein and Sflverstein 1~ in cases of occulsion of the anterior inferior cerebellar artery and arteries of the internal auditory canal. Segmental hydrops has also been reported by Alford et al. s and Igarashi et al. s in some of their experimental animals. Gussen 4 first reported hydrops in the human associated with arterial compromise in her case of polyarteritis nodosa of the temporal bone in which the findings were similar to those in the present case. The changes in this temporal bone are not as severe as in the case previously reported. However, the duration of time from definite onset of disease symptoms to death was shorter in this patient, and he received far fewer drugs that may have contributed to the otologic changes in the temporal bone. Absence of arteritis in the vessel walls of the internal auditory artery does not preclude compromise of this vessel. Involvement of the subarcuate fossa vessels in both ears is sufficient to establish small vessel disease of the inner ear. It should be pointed out that polyar-
teritis nodosa is a segmental process in the vessel walls, and uninvolved adjacent areas of the vessel usually appear normal. It is possible that involvement of the internal auditory artery in this patient was more proximal than the portion contained in the tissue block examined or that segments involved were not included in the sections evaluated. Fibrosis of the scala tympani with new bone formation secondary to arterial occlusion has been demonstrated by Kimura and Perlman 7 as early as two months after occlusion of the anterior inferior cerebellar artery and after four months by Igarashi et a12 with embolization of the peripheral terminal branches of the internal auditory artery. Fibrosis of the cochlea and labyrinth was also described b y Gussen '1 in the previous case of polyarteritis nodosa. The fibrosis and new bone formation resemble those shown by Suga and Lindsay '1 in a study in which these changes were localized to the scala tympani in the beginning portion of the basal coil of the cochlea in response to chronic suppurative otitis media. In this respect the work of Sargent and Christian '2 must be mentioned in relating vasculitis and acute secretory otitis media. They reported seven cases in which the onset of widespread necrotizing vasculitis occurred following acute secretory otitis media. Six of these seven patients had positive rheumatoid factor tests and four had episcleritis. In addition, several of the patients presented with gudden hearing loss, presumably of the sensorineural type. No temporal bone histologic study was presented. Their speculation that this form of disseminated vasculitis resulted from a hypersensitivity reaction to an as yet unknown etiologic agent causing the middle ear inflammation does not seem plausible, since the vascular changes in the temporal bone are identical to the findings of collagenosis involving the general body organs. This case presents additional evidence in the human that sudden hearing loss may result from vascular compromise of the inner ear. In cases of sudden deafness, particularly in association with other constitutional symptoms, the diagnosis of vasculitis should be taken into consideration as part of the differential diagnosis.
References 1. McNeil, N. F., Berke, M., and Reingold, I. M.: Polyarteriris nodosa causing deafness in an adult. Ann, Int, Med., 37:1253-1267, 1952. 2. Foster, D. B., and Malamud, N.: Periarteritis nodosa. A clinicopathologic report with special reference to the
POLYARTERITIS N O D O S A AS A CAUSE OF S U D D E N DEAFNESS
3. 4. 5. 6. 7. 6.
central nervous system. A preliminary report, Univ. Hosp, Bull. (Ann Arbor), 7:102-104, 1941. Peitersen, E., and Carlsen, B. H.: Hearing impairment as the initial sign of polyarteritis nodosa. Acts Otolaryngol., 61:189-195, 1965. Gussen, R.: Polyarteritis nodosa and deafness. A human t e m p o r a l bone study. Arch. Otorhinolaryngol., 2:[7:263-271, 1977, Fauci, A. S.: The spectrum of vasculitis. Clinical, pathologic, immunologic, and therapeutic considerations. Ann. Int. Med., 89:660-676, 1978, Zeek, P. M.: Periarteritis nodosa: a critical review. Am. J. Clin. Patho]., 22',777-790, 1952. Kimnra, R., and Perlman, H. B.: Arterial obstruction of the labyrinth. Part I. Cochlear changes. Ann. Otol. Rhiaol. Laryngol,, 67:5-24, 1958, Alford, B. R., Shaver, E. F., Rosenberg, J. J., and Guilford, F. R.: Physiologic and histopathologic effects of microembolism of the internal auditory artery. Ann. Otol. Rhinol. Laryn~ol., 74:728-748, 1965.
HERMAN A. JENKINS ET AL.
g. Igarashi, M., Alford, B. R., Konishi, S., Shaver, E. F., and Guilford, F. R.:Functional and histological correlation after microembolism of the peripheral labyrinthine artery in the dog, Laryngoscope, 79:603-823, 1969. 10. Berstein, ]. M., and Silverstein, H.: Anterior cerebella' and labyrinthine arteries. Arch. Otolsryngol., 83:422-435, ]966. hi. Suga, F., and Lindsay, J. R.: Labyrinthitis ossificans due to chronic otitis media, Ann. Otol. Rhinol. Laryngol., 84:37-44, 1975. 12. Sergent, J. S., and Christian, C. L.: Necrotizing vasculitis after acute serous otitis media, Ann. Int. Med., 81:195-199, 1974.
Volume 2 Number 2 May 1981
ENT Department University Hospital 80gl Zurich Switzerland (Dr. Flsch)
10 7
Pathologic changes in the temporal bone are described in a case of polyarteritis nodosa in a 48 year old man in whom the onset of sudden unilateral deafness and vertigo occurred seven months prior to death as one of the early manifestations of the disease. The patient had received only a seven week course of prednisolone and, earlier, a two week course of anti-inflammatory agents. Autopsy revealed involvement of the arteries supplying the kidneys, testes, and pancreas. Changes within the temporal bone were seen bilaterally and there was thickening of the mucosa of the middle ear. Inner ear involvement was m a i n l y limited to the cochlea, the deaf ear showing more pronounced changes. These c h a n g e s included loss of the organ of Corti in the hook portion of the basal coil, absence of t h e tectorial membrane, and atrophy of the stria vascularis. The scala tympani was obliterated by fibrosis and new bone formation. The scala media showed hydrops, and a marked decrease in the spiral ganglion cells and nerve fibers supplying this portion of the cochlea was evident. Focal changes were seen t h r o u g h o u t the remainder of the cochlea. The vestibular structures showed no detectable pathologic changes. Small vessel ar~eritis was found in the dural and subarcuate vessels in both temporal bones.
Evidence that polyarteritis nodosa might be a cause of deafness first appeared in the literature in a case report by McNeil e t a l . ~ in 1952 in w h i c h tinnitus and deafness occurred two years after the onset of symptoms. These authors also m a d e reference to a previous work by Foster and M a l a m u d 2 in which two patients from their series of 300 patients with vasculitis had concernRant deafness. Two additional cases of polyarteritis nodosa were presented by Peitersen and Carlsen a in which aural complaints were the pres e n t i n g symptoms of the disease. Only the first case, however, would be considered as polyarteritis nodosa under the presently accepted classification of the disease.
The first pathologic description of temporal bone involvement with polyarteritis nodosa was reported by Gussen "tin a case in w h i c h the hearing loss occurred approximately two years after the appearance of the initial symptoms of the disease. This patient had the typical history of pclyarteritis nodosa, complicated by hypertension and cardiac and renal failure. She had also received treatment with m a n y Lrnmunosuppressive, anti-inflammatory, and diuretic drugs. In this article the pathologic fir~dings in the temporal bones are presented in a patient with polyarteritis nodosa in w h o m the aural complaints were among the initial presenting symptoms, with death ensuing seven months after the
Accepted for publication January 28, 1981. Dr. Jenkins is a Clinical Fellow of the Department of SL~rgery,University af California, Los Angeles, School of Medicine, Los Angeles, California. His work was supported in part by Teacher-Investigator Award 00294 from the Notional Institutes of Health. *Assistant Prufessor of Surgery, University of California, Los Angeles, Medical Center, Los Angeles, California. tReseaz'ch Fellow, ENT Department, University Hospital, Zurich, Switzerland. ~:Professor and Head of ENT Department, University Hospital, Zurich, Switzerland. American Journal of OLalaryngology--Volume 2, Number 2, May 1981
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onset of deafness. The patient received therapeutic dosages of anti-inflammatory agents as a treatment for possible collagenosis only during the seven weeks prior to death. The diagnosis of polyarteritis n o d o s a was not established until necropsy,
CASE
REPORT
A 48 y e a r old w h i t e male f a c t o r y worker s o u g h t initial treatment from his private physician i n June 1962 for a persistent upper respiratory infection of several m o n t h s ' duration. There h a d been no associated fever or other general symptoms, a n d prior to this he had been in excellent health. A few weeks later he developed p h a r y n g i t i s and a p e r i o d o n t a l abscess with spontaneous extrusion of a tooth. In November 1962 he developed severe pain in the left leg along with s u d d e n dizziness, nausea, vomiting, and deafness in the left ear a n d decreased hearing in the right ear. The pain in the legs and the dizziness subsided after two weeks, but the hearing loss persisted. Three months later he developed a low grade fever and arthralgias of nearly all the large and small joints of the body with swelling, erythema, and warmth. These symptoms subsided after a two week course of treatm e n t with p h e n y l b u t a z o n e a n d penicillin. Two weeks later he again developed dizziness with tinnitus and bitemporal headaches. A neurologic evaluation y i e l d e d essentially negative findings, and four m o n t h s after the initial aural s y m p t o m s h a d a p p e a r e d he was referred to the ENT Department of the University of Zurich for further evaluation. Audiometric studies showed complete deafness on the left side w i t h the combination on the right side of a m i l d conductive and a moderate sloping high frequency sensorineural hearing loss. Caloric testing s h o w e d a nonfunctioning labyrinth on the left. Paracentesis of the right m i d d l e ear p r o v i d e d immediate improvement of the conductive c o m p o n e n t of the hearing loss. The secretions of the middle ear were described as yellow and tenacious. No bacteriologic studies were performed. The patient's g e n e r a l s y m p t o m s increased w i t h the appearance of a chronic olecranon bursitis, w h i c h was histologically nonspecific on biopsy. He h a d increasing facial edema, oliguria, and hematuria w i t h acute abdominal pain and required hospitalization. Treatment with prednisoione was begun at another hospital several weeks prior to his transfer to the University Hosi00
pital for further diagnostic work-up and treatment. On admission in May 1963 the patient was normotensive (135/80 ram. Hg). Laboratory evaluation showed a hemoglobin level of 9.8 mg. per 100 ml. and an erythrocyte sedimentation rate of 64 ram. per hr. The white cell count was 12,800 per cu. ram. with a shift to the left and an absolute eosinophilia of 684. The residual nitrogen was 196 rag. per 100 m]., blood urea nitrogen 264 rag. per 100 ml., uric acid 16.8 mg. per 1(]0 ml., and creatinine 12.5 rag. per 100 ml. Urinalysis showed a 0.8 gin. per 1000 ml. protein concentration with macroscopic hematuria and cylindrical casts. Cultures of the urine showed no growth. Serologic testing for syphilis was negative, as was the antiglobulin c o n s u m p t i o n test with cell nuclei. The antistreptolysin titer was not elevated. A muscle biopsy showed no evidence of vasculitis and a renal biopsy specimen was interpreted as s h o w i n g chronic pyelonephritis. The patient underwent peritoneal dialysis on three occasions. He received a total course of seven weeks of prednisolone at a dosage of 40 mg. per day. Before further definitive work-up could proceed, the patient had respiratory failure and died, secondary to uremic pneumonia.
PATHOLOGIC
STUDY
The general pathologic changes were limited to the small arteries supplying the kidneys, pancreas, and testes. These changes were similar to those described in the following section. In addition, the kidneys showed features of advanced membraneous glomerulonephritis with hyalinization of m a n y glomeruli. The testes contained focal areas of acute and chronic hemorrhagic infarction secondary to arteriolar obstruction. The other major organs of the body did not show any pathologic changes.
Temporal Bone LEFT EAR. The left temporal bone was decalcified and embedded in celloidin. It was cut in vertical sections 20 microns in thickness, with orientation at a right angle to the longitudinal axis of the petrous bone. The sections were stained with hematoxylin and eosin and every tenth section was studied. Additional special stains for myelin and elastic fibers were used. Focal perivascular infiltration of inflmnmatory
POLYARTERITISNODOSAAS A CAUSE OF SUDDENDEAFNESS
Volume 2 Number 2 May 1981
Figure 1. Photomicrograph of the skin lining the external auditory canal. Arteritis of the small vessels is prominent.
Figure 2. Photomicrograph of the mucosal lining of the middle ear. Note the hypertrophic changes in the submucosal layer with round cell infiltration, particularly in the perivascular areas.
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Figure 3.
P h o t o m i c r o g r a p h of the r o u n d w i n d o w niche with obliteration by hypertrophic fibrous tissue.
cells was present in the subcutaneous layer of the skin lining the b o n y external auditory canal (Fig. 1). The m i d d l e ear and mastoid air spaces s h o w e d marked thickening of the submucosal layer with a highly cellular connective tissue, infiltrated t h r o u g h o u t with chronic inflammatory cells concentrated mainly in the perivascular areas (Fig. 2). The round w i n d o w niche was filled with a mass of areolar connective tissue that obliterated the entire niche and exhibited pericapillary infiltration with chronic inflammatory cells (Fig. 3). The ossicles of the m i d d l e e a r were normal, including the joint spaces, but were e m b e d d e d in a thick layer of connective tissue, similar to the connective tissue of the s u b m u c o s a l layer. The tensor tympani and stapedius muscles were normal. In the h o o k p o r t i o n of the basal coil of the cochlea there was a t r o p h y of the stria vascularis with absence of the organ of Corti and the tec-
torial membrane (Fig. 4). The ceils of the limbus appeared normal. Reissner's m e m b r a n e was intact but bulged into the scala vestibuli. The entire scala tympani in this area of the cochlea was filled with disorganized connective tissue with areas of n e w bone formation. The spiral ligament was thickened and continuous with the co.nnectire tissue in the scala tympani. There was a marked loss of nerve fibers and spiral ganglion cells in this portion of the cochlea. The remaining cochlea s h o w e d a d h e r e n c e of Reissner's membrane to the limbus and tectorial membrane with the free portion bulging slightly into the scala vestibuli. There was focal atrophy of the stria vascularis and isolated areas of collapse of the organ of Corti throughout the cochlea, particularly in the apical coil. The r e m a i n d e r of the scala t y m p a n i s h o w e d n o c o n n e c t i v e tissue obliteration, and the spiral ganglion cells and nerve fibers appeared normal. There were occa-
Figure 4. A, P h o t o m i c r o g r a p h of the m i d m o d i o l a r view of the cochlea of the left ear. B, Higher power view of the hook portion of the basal coil. Note the fibrosis and n e w bone formation obliterating the scala t y m p a n i w i t h absence of the organ of Corti and tectorial m e m b r a n e and the decrease in the nerve b u n d l e s and spiral ganglion cells to the basal coil.
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Figure 4.
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Figure 5. A, Photomicrograph of the dura with periarterial infiltration and fibrinoid necrosis of the vessel wall. B, Photomicrograph showing similar changes in the subarcuate vessels of the ]eft temporal bone.
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POLYARTERITIS NODOSA AS A CAUSE OF SUDDEN DEAFNESS
sional connective tissue synechiae within the scala vestibuli of the middle coil, adjacent to the hook portion of the basal coil of the cochlea. No detectable pathologic changes were present in the vestibular structures. The nerves within the internal auditory canal were normal, as was the dura. Several small arteries were present in the internal auditary canal without a n y pathologic changes. The dura of the posterior fossa was markedly thickened with a dense, well organized collagenous tissue and" showed perivascular infiltration of the small arterioles with fibrinoid necrosis in the walls and fibrocytic proliferation at the periphery of the vessels (Fig. 5A). The lumen was almost completely obliterated in many of these vessels. Similar changes were found in the vessels of the suba r c u a t e fossa (Fig. 5B). The same c h r o n i c i n f l a m m a t o r y cell i n f i l t r a t i o n was p r e s e n t around the venules without compromise of their lumens. RIGHT EAR. The right ear was cut in horizontal sections 20 microns in thickness. The sections were stained with hemotoxylin and eosin and every tenth section was studied, The pathologic changes in the external auditory canal,
middle ear, mastoid, and tensor t y m p a n i muscle were identical to those described in the left ear. The cochlea showed focal areas of atrophy of the stria vascularis throughout that appeared to be more pronounced in the basal coil. The number of spiral ganglion cells and nerve fibers supplying the hook portion of the basal coil of the cochlea was markedly decreased. The remainder of the cochlear and vestibular structures of the inner ear were normal. The dura of the middle and posterior fossae was normal in thickness but showed an inflammatory perivascular infiltration and fibrinoid necrosis in a few of the arterioles seen. The same vascular changes were found in the subarcuate vessels as described in the left ear (Fig. 6). Only small caliber arteries were present in the internal auditory canal and t h e y w e r e w i t h o u t e v i d e n c e of p a t h o l o g i c change.
Volume 2 Number 2 May 1981
DISCUSSION
Po]yarteritis nodosa is a systemic vasculitis of unknown etiology, involving mainly small and middle caliber arteries. The lesions are s e g m e n -
Figure 6. Photomicrographof subarcuate vessels of the right temporal bone showing perivascular infiltration and fibrinoid necrosis. The lumen of the artery is almost completelyobliterated. HERMANA. JENKINSET AL.
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tal with a predilection for the bifurcations of the vessels. The disease process spreads longitudinally along the arterioles and eventually involves accompanying venules. Fauci 5 called this pattern of spreading of the lesions to the arterioles, venules, and capillaries an "overlap syndrome," which he considered to be a subgroup of classic polyarterifis nodosa. In the acute state the characteristic pathologic change is a polymorphonuclear cellular infiltration of all three layers of the vessel wall and the perivascular area. In the later stages the vascular and perivascular infiltration changes from the polymorphonuclear cells of the acute stage to a chronic lymphoplasmic cellular infiltrate. The vessels undergo fibrinoid necrosis, mainly in the media layer, with loss of elastic fibers in the elastic membrane of the arterial wall. These changes lead to scarring in the involved vessels. In the classic form of polyarteritis all three stages may occur simultaneously. Typically the distribution of the disease in the body spares the small vessels of the lungs and spleen. According to the criteria of Zeek, ~ localization of the arterial lesions in the present case to the kidneys, pancreas, and testes, along with associated microscopic findings described, classifies this patient as a typical case of polyarteritis nodosa. The changes found in the cochlea in this case are similar to those reported by several authors in experimental work with compromise of the arterial supply of the inner ear. Loss of the organ of Corti and strial changes were reported by Kimttra and Perlman 7, Alford et el. s Igarashi et el. 0 and Bernstein and Sflverstein 1~ in cases of occulsion of the anterior inferior cerebellar artery and arteries of the internal auditory canal. Segmental hydrops has also been reported by Alford et al. s and Igarashi et al. s in some of their experimental animals. Gussen 4 first reported hydrops in the human associated with arterial compromise in her case of polyarteritis nodosa of the temporal bone in which the findings were similar to those in the present case. The changes in this temporal bone are not as severe as in the case previously reported. However, the duration of time from definite onset of disease symptoms to death was shorter in this patient, and he received far fewer drugs that may have contributed to the otologic changes in the temporal bone. Absence of arteritis in the vessel walls of the internal auditory artery does not preclude compromise of this vessel. Involvement of the subarcuate fossa vessels in both ears is sufficient to establish small vessel disease of the inner ear. It should be pointed out that polyar-
teritis nodosa is a segmental process in the vessel walls, and uninvolved adjacent areas of the vessel usually appear normal. It is possible that involvement of the internal auditory artery in this patient was more proximal than the portion contained in the tissue block examined or that segments involved were not included in the sections evaluated. Fibrosis of the scala tympani with new bone formation secondary to arterial occlusion has been demonstrated by Kimura and Perlman 7 as early as two months after occlusion of the anterior inferior cerebellar artery and after four months by Igarashi et a12 with embolization of the peripheral terminal branches of the internal auditory artery. Fibrosis of the cochlea and labyrinth was also described b y Gussen '1 in the previous case of polyarteritis nodosa. The fibrosis and new bone formation resemble those shown by Suga and Lindsay '1 in a study in which these changes were localized to the scala tympani in the beginning portion of the basal coil of the cochlea in response to chronic suppurative otitis media. In this respect the work of Sargent and Christian '2 must be mentioned in relating vasculitis and acute secretory otitis media. They reported seven cases in which the onset of widespread necrotizing vasculitis occurred following acute secretory otitis media. Six of these seven patients had positive rheumatoid factor tests and four had episcleritis. In addition, several of the patients presented with gudden hearing loss, presumably of the sensorineural type. No temporal bone histologic study was presented. Their speculation that this form of disseminated vasculitis resulted from a hypersensitivity reaction to an as yet unknown etiologic agent causing the middle ear inflammation does not seem plausible, since the vascular changes in the temporal bone are identical to the findings of collagenosis involving the general body organs. This case presents additional evidence in the human that sudden hearing loss may result from vascular compromise of the inner ear. In cases of sudden deafness, particularly in association with other constitutional symptoms, the diagnosis of vasculitis should be taken into consideration as part of the differential diagnosis.
References 1. McNeil, N. F., Berke, M., and Reingold, I. M.: Polyarteriris nodosa causing deafness in an adult. Ann, Int, Med., 37:1253-1267, 1952. 2. Foster, D. B., and Malamud, N.: Periarteritis nodosa. A clinicopathologic report with special reference to the
POLYARTERITIS N O D O S A AS A CAUSE OF S U D D E N DEAFNESS
3. 4. 5. 6. 7. 6.
central nervous system. A preliminary report, Univ. Hosp, Bull. (Ann Arbor), 7:102-104, 1941. Peitersen, E., and Carlsen, B. H.: Hearing impairment as the initial sign of polyarteritis nodosa. Acts Otolaryngol., 61:189-195, 1965. Gussen, R.: Polyarteritis nodosa and deafness. A human t e m p o r a l bone study. Arch. Otorhinolaryngol., 2:[7:263-271, 1977, Fauci, A. S.: The spectrum of vasculitis. Clinical, pathologic, immunologic, and therapeutic considerations. Ann. Int. Med., 89:660-676, 1978, Zeek, P. M.: Periarteritis nodosa: a critical review. Am. J. Clin. Patho]., 22',777-790, 1952. Kimnra, R., and Perlman, H. B.: Arterial obstruction of the labyrinth. Part I. Cochlear changes. Ann. Otol. Rhiaol. Laryngol,, 67:5-24, 1958, Alford, B. R., Shaver, E. F., Rosenberg, J. J., and Guilford, F. R.: Physiologic and histopathologic effects of microembolism of the internal auditory artery. Ann. Otol. Rhinol. Laryn~ol., 74:728-748, 1965.
HERMAN A. JENKINS ET AL.
g. Igarashi, M., Alford, B. R., Konishi, S., Shaver, E. F., and Guilford, F. R.:Functional and histological correlation after microembolism of the peripheral labyrinthine artery in the dog, Laryngoscope, 79:603-823, 1969. 10. Berstein, ]. M., and Silverstein, H.: Anterior cerebella' and labyrinthine arteries. Arch. Otolsryngol., 83:422-435, ]966. hi. Suga, F., and Lindsay, J. R.: Labyrinthitis ossificans due to chronic otitis media, Ann. Otol. Rhinol. Laryngol., 84:37-44, 1975. 12. Sergent, J. S., and Christian, C. L.: Necrotizing vasculitis after acute serous otitis media, Ann. Int. Med., 81:195-199, 1974.
Volume 2 Number 2 May 1981
ENT Department University Hospital 80gl Zurich Switzerland (Dr. Flsch)
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