Ann 0101 84: 1975
GRAND ROUNDS PADOVA OTORHINOLARYNGOLOGICAL CLINIC UNIVERSITY OF PADUA, ITALY MICHELE ARSLAN, M.D., Professor and Chairman PADUA, ITALY PARTICIPANTS:
GIORGIO MOLINARI, M.D., Associate Professor, Audiology DAVIDE MEGIGHIAN, M.D., Associate Professor, Otoneurology WALTER Mazzo, M.D., Audiologist MARIO CENZI, M.D., Resident GIANNI RUFFINI, M.D., Resident
Dr. Davide Megighian: A 55-year-old woman consulted me in December 1972 complaining of vertiginous attacks and postural disturbances. She had no previous history of serious illness, nor did her way of life and diet show any signs of the use of toxic agents or of excessive physical effort. In 1971, during a checkup performed in the Department of Internal Medicine, it was noted after taking several periodic readings that she had very low blood pressure (80 minimum, 115 maximum). The patient was questioned as to the features of her disturbances. It was learned that vertigo was experienced primarily in the morning, beginning quite suddenly, and she always had the feeling that her body was rotating toward the right. Generally, the attack was of short duration. During the attack the patient had to close her eyes, sit down, or lie down. Movement of the head often made the attack worse. Once the attack was over, there was no disturbance except weakness for an hour or two. There were no premonitory disturbances; the Lermoyez sign was absent. In 1971 she was examined by an otologist and underwent nasopharyn-
geal, audiometric, and vestibulometric tests, after which a diagnosis of Meniere's disease involving the right ear was made. Antivertiginous drugs were prescribed. Some improvement occurred, but the disturbances soon returned. As regards the postural disturbances, the patient stated that she often felt a tendency to move toward the right, although she did not fall. Finally, she said she had never suffered from earache, nor otorrhea. She reported that hearing was normal in the left ear, with a slight loss of hearing in the right. The otorhinolaryngological and cochleovestibular examination showed the following: right tympanum membrane opaque and slightly irregular; left normal. The upper airways were normal. The audiometric evaluation revealed a slight hypoacusia on the right, prevalent in the low frequencies (threshold of 50 dB for 250 Hz, 40 dB for 500 Hz, 30 dB for 1 kHz and 2 kHz). A normal audiogram was obtained on the left. It must be noted that the patient did not spontaneously complain of hypoacusia; this was restricted to the right ear while the left ear functioned well. We were somewhat
256
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GRAND ROUNDS
surprised by the bone conduction threshold: on the right it had fallen slightly, but there was, nevertheless, a gap of 25 dB. The vestibular examination showed the following: no sign of spontaneous lack of balance (there was no spontaneous nystagmus nor deviation of the index fingers nor of the trunk; there was no positional nystagmus in the De Kleyn position); no vestibular reactivity, either on hot or cold thermic stimulation (Veits' method). On acceleratory stimulation ( BuysFischer-Arslan method, with subliminal angular acceleration and angular velocity of 120 there occurred slight hyporeactivity on the right with quantitative nystagmus values about half those obtained on the left. The slight rightsided hyporeactive syndrome may be defined as a deficient harmonic vestibular syndrome, that is to say, all the nystagmic oscillations provoked by the various tests responded with equivalent values. The electronystagmogram confirmed vestibular hyporeactivity on the right. There was a slight difference in form between the nystagmus provided on the right and that provided on the left. I diagnosed right-side tympanosclerosis, probably caused by otitis in infancy (which the patient does not remember), which evolved over the years into labyrinthosis of the Meniere type. I prescribed two types of treatment: 1) periodic ventilation of the Eustachian tube on the right, and 2) histamine, peripheral vasodilators, and diuretics. The patient has returned today for a further consultation. I would like to discuss this unusual case with my colleagues. 0
)
Dr. Michele Arslan: I, too, am rather perplexed about this diagnosis, especially because while the right-sided tympanic objectivity clearly points to tympanosclerosis, the Meniere syndrome does not seem very typical. Dr. Giorgio Molinari: Is Dr. Megighian sure that the disturbances of which the patient complains are not neurotic disturbances or the result of some other nervous cause? If this were so, there would not be the diagnostic doubt now
257
under discussion. Dr. Megighian: I have checked on the possible psychogenic origin of the vertigo. The patient impressed me as being perfectly balanced from the psychic point of view, and also, the neurologist to whom I referred the patient told me that according to his examination she was normal. Dr. Walter Mozzo: The diagnostic problem could be solved by submitting the patient to impedance measurement to see whether there is tympanic and/or ossicle mobility. Furthermore, I would perform an audiogram with Bekesy's technique. It might, in fact, be possible that the tympanosclerosis is accompanied by stapes and footplate fixation: therefore, the alteration in the peri- and endolymphatic circulation would be the cause of vertigo, and it would not be a case of Meniere's disease. Dr. Mario Cenzi: An important point in the problem concerning this patient is that the tympanosclerosis is certainly postinHammatory: it is, in fact, unilateral and is easily seen otoscopically. The only means of ascertaining whether the tympanoscl-rosis is an aftereffect of otitis in childhood is to perform an x-ray of the mastoid. We know that, according to Wittmaack's theory which is still valid, every inflammation (however brief) of the middle ear in childhood disturbs the pneumatization process of the mastoid, which remains just as it is for the rest of life, with areas of density and opaqueness. The inflammation interrupts the osteoclastic pneumatizing process of the first years of life. Dr. Arslan: Dr. Cenzi's observation is correct, and we will immediately perform an x-ray of this patient's mastoids. Dr. Molinari: In this case we must also consider another aspect of the clinical picture, precisely the features of the disturbances experienced by the patient. The patient told Dr. Megighian that her vertiginous attacks are not accompanied by illusions of rotation of the outside world, that they are never accompanied by vomiting, or even
Downloaded from aor.sagepub.com at MCMASTER UNIV LIBRARY on July 1, 2015
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ARSLAN ET AL.
nausea, that they are not preceded by increased tinnitus (namely, there is an absence of that very important diagnostic sign of true Meniere's disease. the Lermoyez sign), that they are of short duration, and. above all, that they are not aggravated by movements of the head. I wonder if this vertigo could have a central origin, precisely from some problem in the vestibular nuclei. Could it not be a vertiginous syndrome from cervical arthrosis (Barre's syndrome)? Dr. Gianni Ruffini: I think Dr. Molinari's hypothesis is verv well founded. In fact, an indication of the central origin of the syndrome could be seen in the variations in the eIectronystagmographic form of the nystagmus, observed in all the recordings obtained on the affected side. We know that these "qualitative" alterations of the nystagmus (prevalence of the slow phase, "dome-shaped" appearance of the nystagmus, etc.) depend on the vestibular nuclei and on the reticular formation in which the nystagmus is elaborated. Dr. Arslan: Dr. Molinari's hypothesis is very suggestive. I would advise referring the patient to a neuroradiolozist and asking him to obtain x-rays of her cervical vertebrae and arteriography of the vertebral arteries. FOLLOW-UP CONFERENCE
Dr. Arslan: We shall continue the talk we had yesterday about the unusual patient brought to our notice by Dr. Megighian. Xvravs were obtained of the mastoids and of the cervical vertebrae with bilateral visualization of the vertebral arteries, as well as the requested audiometric tests. The results of these tests were as follows: 1) The right mastoid is less pneumatized than the left and reveals a zone of limited bone sclerosis; 2) There is extensive arthrosis of the cervical vertebrae with an initial fusion of C5 and C6, and some osteophytes can be seen on the right. The patient was questioned about this and stated that for some years she had, in fact, suffered from nuchal pains, which at times spread out toward the
shoulders; and 3) Arteriography of the vertebral arteries shows two small arteries, without data as to their pathologic form or course. Special audiometric tests (impedance measurement, etc.) do not reveal tvrnpanic or bone rigidity. There are a few slight signs of lesions of the cochlear receptor. Dr. Ruffini: I would like to ask: what is the nature of the changes in the vestibular nuclei? Dr. Molinari: Much can be said about this. In the literature we can find various diagnoses: the vestibular nuclei can be affected by toxic agents, allergy, limited encephalitic processes, etc. Nor must we forget Arslan's nucleo-reticular syndrome or Hallpike's vestibular n-uronitis, which refer in substance to the same clinical picture, now accepted by all otologists as a nosological fact. In the patient under discussion, the nuclear change appears to be an ischemia of very short duration. caused by compression of the homolateral vertebral artery, which manifests itself on rotation of the head; this compression is due to the arthritic osteophytes at the level of the intertransverse vertebral aperture. That is, it is Barre's syndrome, as formulated over thirty years ago. in which Barre drew up a pathogenic hvpothesis (compression of the vertebral nerve), which was later proved to be completely mistaken. Dr. AsIan: I think Dr. Molinari's statement is correct. The compression of a vertebral artery for one or two seconds can provoke a fleeting ischemia of the homolateral vestibular nucleus; this ischemia is immediately compensated by the opposite vertebral artery in order to normalize with extreme rapidity the flux of the basilar trunk, which supplies blood to the vestibular nuclei. It is a picture well known to neurologists who call it "intermittent insufficiency of the vertebroarterial connection." Let me remind you that some years ago in our Institute, Dr. Carbone, in collaboration with Dr. Molinari, carried out experimental research on the effects of ligation of the vertebral artery: he noted
Downloaded from aor.sagepub.com at MCMASTER UNIV LIBRARY on July 1, 2015
259
GRAND ROUNDS
the appearance of an irritative nystagmus (that is, with the rapid phase on the side of the ligation), with a duration of a few minutes. accompanied by deviation of the trunk toward the opposite direction, etc. Dr. Megighian: I think this new diagnosis gives a clearer interpretation of the clinical picture presented by this patient. Tympanosclerosis is not, therefore, included among the factors that caused the suspected Meniere's disease. However, the patient does present a hearing loss which still indicates a perceptive peripheral lesion. How can this fact be explained? Dr. Arslan: The relationships between Meniere's disease and Barre's syndrome are not really as simple as they seem: the two conditions are not autonomous. We must not forget that the internal acoustic artery also emerges from the basilar trunk; therefore, it can certainly
be expected that repeated attacks of intermittent insufficiency of the vertebrobasilar system will provoke not only a fleeting ischemia of the homolateral nucleus, but also a fleeting ischemia of the inner ear, with consequent damage to the cochlear and vestibular neuroepithelia, which adds to or aggravates the effect of the nuclear ischemia. At this point, I wish to recall another clinical fact which is very important: the patient presents a very pronounced arterial hypotension (this can be seen in a great many people suffering from vertigo, especially with Meniere's disease). It is obvious that this favors the harmful consequences of the intermittence of the vertebrobasilar system. I would advise that the patient undergo therapy for arthritis and physiotherapy. We thank Dr. Megighian for his presentation and for the stimulus he has provided for this discussion.
AMERICAN COUNCIL WORKSHOPS The 1975 series of Practice Management and Productivity Workshops sponsored by the American Council of Otolaryngology will be held at the dates and locations listed below. These Workshops, designed for the practicing otolaryngologist, are carefully structured to otolaryngology management and practice problems found in a cross section of otolaryngology offices across the country. A 5th hour program is included for the physician's aide and is given concurrently on the first day of the Workshop. Course fee (including instructional materials) is $345.
Dates of Workshops -1975
Location
Dates of Workshops - 1975
Location
May 16-17
Atlanta
June 24 - 25
New York
June 10 - 11
Philadelphia
July 11 - 12
Dallas
June 17 - 18
San Francisco
July 18 - 19
Las Vegas
June 20 - 21
Los Angeles
July 22 - 23
Chicago
Make application to: The American Council of Otolaryngology, 1100 - 17th St., N.W., Suite 602: Wa.shington, D.C. 20036 or call toll free to Conomikes Associates, Inc., (800) 4216512; California residents should call collect (213) 823-4661.
Downloaded from aor.sagepub.com at MCMASTER UNIV LIBRARY on July 1, 2015
GRAND ROUNDS PADOVA OTORHINOLARYNGOLOGICAL CLINIC UNIVERSITY OF PADUA, ITALY MICHELE ARSLAN, M.D., Professor and Chairman PADUA, ITALY PARTICIPANTS:
GIORGIO MOLINARI, M.D., Associate Professor, Audiology DAVIDE MEGIGHIAN, M.D., Associate Professor, Otoneurology WALTER Mazzo, M.D., Audiologist MARIO CENZI, M.D., Resident GIANNI RUFFINI, M.D., Resident
Dr. Davide Megighian: A 55-year-old woman consulted me in December 1972 complaining of vertiginous attacks and postural disturbances. She had no previous history of serious illness, nor did her way of life and diet show any signs of the use of toxic agents or of excessive physical effort. In 1971, during a checkup performed in the Department of Internal Medicine, it was noted after taking several periodic readings that she had very low blood pressure (80 minimum, 115 maximum). The patient was questioned as to the features of her disturbances. It was learned that vertigo was experienced primarily in the morning, beginning quite suddenly, and she always had the feeling that her body was rotating toward the right. Generally, the attack was of short duration. During the attack the patient had to close her eyes, sit down, or lie down. Movement of the head often made the attack worse. Once the attack was over, there was no disturbance except weakness for an hour or two. There were no premonitory disturbances; the Lermoyez sign was absent. In 1971 she was examined by an otologist and underwent nasopharyn-
geal, audiometric, and vestibulometric tests, after which a diagnosis of Meniere's disease involving the right ear was made. Antivertiginous drugs were prescribed. Some improvement occurred, but the disturbances soon returned. As regards the postural disturbances, the patient stated that she often felt a tendency to move toward the right, although she did not fall. Finally, she said she had never suffered from earache, nor otorrhea. She reported that hearing was normal in the left ear, with a slight loss of hearing in the right. The otorhinolaryngological and cochleovestibular examination showed the following: right tympanum membrane opaque and slightly irregular; left normal. The upper airways were normal. The audiometric evaluation revealed a slight hypoacusia on the right, prevalent in the low frequencies (threshold of 50 dB for 250 Hz, 40 dB for 500 Hz, 30 dB for 1 kHz and 2 kHz). A normal audiogram was obtained on the left. It must be noted that the patient did not spontaneously complain of hypoacusia; this was restricted to the right ear while the left ear functioned well. We were somewhat
256
Downloaded from aor.sagepub.com at MCMASTER UNIV LIBRARY on July 1, 2015
GRAND ROUNDS
surprised by the bone conduction threshold: on the right it had fallen slightly, but there was, nevertheless, a gap of 25 dB. The vestibular examination showed the following: no sign of spontaneous lack of balance (there was no spontaneous nystagmus nor deviation of the index fingers nor of the trunk; there was no positional nystagmus in the De Kleyn position); no vestibular reactivity, either on hot or cold thermic stimulation (Veits' method). On acceleratory stimulation ( BuysFischer-Arslan method, with subliminal angular acceleration and angular velocity of 120 there occurred slight hyporeactivity on the right with quantitative nystagmus values about half those obtained on the left. The slight rightsided hyporeactive syndrome may be defined as a deficient harmonic vestibular syndrome, that is to say, all the nystagmic oscillations provoked by the various tests responded with equivalent values. The electronystagmogram confirmed vestibular hyporeactivity on the right. There was a slight difference in form between the nystagmus provided on the right and that provided on the left. I diagnosed right-side tympanosclerosis, probably caused by otitis in infancy (which the patient does not remember), which evolved over the years into labyrinthosis of the Meniere type. I prescribed two types of treatment: 1) periodic ventilation of the Eustachian tube on the right, and 2) histamine, peripheral vasodilators, and diuretics. The patient has returned today for a further consultation. I would like to discuss this unusual case with my colleagues. 0
)
Dr. Michele Arslan: I, too, am rather perplexed about this diagnosis, especially because while the right-sided tympanic objectivity clearly points to tympanosclerosis, the Meniere syndrome does not seem very typical. Dr. Giorgio Molinari: Is Dr. Megighian sure that the disturbances of which the patient complains are not neurotic disturbances or the result of some other nervous cause? If this were so, there would not be the diagnostic doubt now
257
under discussion. Dr. Megighian: I have checked on the possible psychogenic origin of the vertigo. The patient impressed me as being perfectly balanced from the psychic point of view, and also, the neurologist to whom I referred the patient told me that according to his examination she was normal. Dr. Walter Mozzo: The diagnostic problem could be solved by submitting the patient to impedance measurement to see whether there is tympanic and/or ossicle mobility. Furthermore, I would perform an audiogram with Bekesy's technique. It might, in fact, be possible that the tympanosclerosis is accompanied by stapes and footplate fixation: therefore, the alteration in the peri- and endolymphatic circulation would be the cause of vertigo, and it would not be a case of Meniere's disease. Dr. Mario Cenzi: An important point in the problem concerning this patient is that the tympanosclerosis is certainly postinHammatory: it is, in fact, unilateral and is easily seen otoscopically. The only means of ascertaining whether the tympanoscl-rosis is an aftereffect of otitis in childhood is to perform an x-ray of the mastoid. We know that, according to Wittmaack's theory which is still valid, every inflammation (however brief) of the middle ear in childhood disturbs the pneumatization process of the mastoid, which remains just as it is for the rest of life, with areas of density and opaqueness. The inflammation interrupts the osteoclastic pneumatizing process of the first years of life. Dr. Arslan: Dr. Cenzi's observation is correct, and we will immediately perform an x-ray of this patient's mastoids. Dr. Molinari: In this case we must also consider another aspect of the clinical picture, precisely the features of the disturbances experienced by the patient. The patient told Dr. Megighian that her vertiginous attacks are not accompanied by illusions of rotation of the outside world, that they are never accompanied by vomiting, or even
Downloaded from aor.sagepub.com at MCMASTER UNIV LIBRARY on July 1, 2015
258
ARSLAN ET AL.
nausea, that they are not preceded by increased tinnitus (namely, there is an absence of that very important diagnostic sign of true Meniere's disease. the Lermoyez sign), that they are of short duration, and. above all, that they are not aggravated by movements of the head. I wonder if this vertigo could have a central origin, precisely from some problem in the vestibular nuclei. Could it not be a vertiginous syndrome from cervical arthrosis (Barre's syndrome)? Dr. Gianni Ruffini: I think Dr. Molinari's hypothesis is verv well founded. In fact, an indication of the central origin of the syndrome could be seen in the variations in the eIectronystagmographic form of the nystagmus, observed in all the recordings obtained on the affected side. We know that these "qualitative" alterations of the nystagmus (prevalence of the slow phase, "dome-shaped" appearance of the nystagmus, etc.) depend on the vestibular nuclei and on the reticular formation in which the nystagmus is elaborated. Dr. Arslan: Dr. Molinari's hypothesis is very suggestive. I would advise referring the patient to a neuroradiolozist and asking him to obtain x-rays of her cervical vertebrae and arteriography of the vertebral arteries. FOLLOW-UP CONFERENCE
Dr. Arslan: We shall continue the talk we had yesterday about the unusual patient brought to our notice by Dr. Megighian. Xvravs were obtained of the mastoids and of the cervical vertebrae with bilateral visualization of the vertebral arteries, as well as the requested audiometric tests. The results of these tests were as follows: 1) The right mastoid is less pneumatized than the left and reveals a zone of limited bone sclerosis; 2) There is extensive arthrosis of the cervical vertebrae with an initial fusion of C5 and C6, and some osteophytes can be seen on the right. The patient was questioned about this and stated that for some years she had, in fact, suffered from nuchal pains, which at times spread out toward the
shoulders; and 3) Arteriography of the vertebral arteries shows two small arteries, without data as to their pathologic form or course. Special audiometric tests (impedance measurement, etc.) do not reveal tvrnpanic or bone rigidity. There are a few slight signs of lesions of the cochlear receptor. Dr. Ruffini: I would like to ask: what is the nature of the changes in the vestibular nuclei? Dr. Molinari: Much can be said about this. In the literature we can find various diagnoses: the vestibular nuclei can be affected by toxic agents, allergy, limited encephalitic processes, etc. Nor must we forget Arslan's nucleo-reticular syndrome or Hallpike's vestibular n-uronitis, which refer in substance to the same clinical picture, now accepted by all otologists as a nosological fact. In the patient under discussion, the nuclear change appears to be an ischemia of very short duration. caused by compression of the homolateral vertebral artery, which manifests itself on rotation of the head; this compression is due to the arthritic osteophytes at the level of the intertransverse vertebral aperture. That is, it is Barre's syndrome, as formulated over thirty years ago. in which Barre drew up a pathogenic hvpothesis (compression of the vertebral nerve), which was later proved to be completely mistaken. Dr. AsIan: I think Dr. Molinari's statement is correct. The compression of a vertebral artery for one or two seconds can provoke a fleeting ischemia of the homolateral vestibular nucleus; this ischemia is immediately compensated by the opposite vertebral artery in order to normalize with extreme rapidity the flux of the basilar trunk, which supplies blood to the vestibular nuclei. It is a picture well known to neurologists who call it "intermittent insufficiency of the vertebroarterial connection." Let me remind you that some years ago in our Institute, Dr. Carbone, in collaboration with Dr. Molinari, carried out experimental research on the effects of ligation of the vertebral artery: he noted
Downloaded from aor.sagepub.com at MCMASTER UNIV LIBRARY on July 1, 2015
259
GRAND ROUNDS
the appearance of an irritative nystagmus (that is, with the rapid phase on the side of the ligation), with a duration of a few minutes. accompanied by deviation of the trunk toward the opposite direction, etc. Dr. Megighian: I think this new diagnosis gives a clearer interpretation of the clinical picture presented by this patient. Tympanosclerosis is not, therefore, included among the factors that caused the suspected Meniere's disease. However, the patient does present a hearing loss which still indicates a perceptive peripheral lesion. How can this fact be explained? Dr. Arslan: The relationships between Meniere's disease and Barre's syndrome are not really as simple as they seem: the two conditions are not autonomous. We must not forget that the internal acoustic artery also emerges from the basilar trunk; therefore, it can certainly
be expected that repeated attacks of intermittent insufficiency of the vertebrobasilar system will provoke not only a fleeting ischemia of the homolateral nucleus, but also a fleeting ischemia of the inner ear, with consequent damage to the cochlear and vestibular neuroepithelia, which adds to or aggravates the effect of the nuclear ischemia. At this point, I wish to recall another clinical fact which is very important: the patient presents a very pronounced arterial hypotension (this can be seen in a great many people suffering from vertigo, especially with Meniere's disease). It is obvious that this favors the harmful consequences of the intermittence of the vertebrobasilar system. I would advise that the patient undergo therapy for arthritis and physiotherapy. We thank Dr. Megighian for his presentation and for the stimulus he has provided for this discussion.
AMERICAN COUNCIL WORKSHOPS The 1975 series of Practice Management and Productivity Workshops sponsored by the American Council of Otolaryngology will be held at the dates and locations listed below. These Workshops, designed for the practicing otolaryngologist, are carefully structured to otolaryngology management and practice problems found in a cross section of otolaryngology offices across the country. A 5th hour program is included for the physician's aide and is given concurrently on the first day of the Workshop. Course fee (including instructional materials) is $345.
Dates of Workshops -1975
Location
Dates of Workshops - 1975
Location
May 16-17
Atlanta
June 24 - 25
New York
June 10 - 11
Philadelphia
July 11 - 12
Dallas
June 17 - 18
San Francisco
July 18 - 19
Las Vegas
June 20 - 21
Los Angeles
July 22 - 23
Chicago
Make application to: The American Council of Otolaryngology, 1100 - 17th St., N.W., Suite 602: Wa.shington, D.C. 20036 or call toll free to Conomikes Associates, Inc., (800) 4216512; California residents should call collect (213) 823-4661.
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