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133 It may occur to one that the pulmonary, ophthalmic, and nephrotic syndromes in the case of Mrs. A. B. were each due to a paroxysmal phlebetic attack of unknown xtiology but of the same nature as the phlebetic syndrome in her extremities. REFERENCES Braun-Falco, O. (1953) Derm. Wschr. 127, 506. Calvet, M. J. (1944) Mém. Acad. Chir. 70, 445. Daniels, W. B. (1932) Amer. J. med. Sci. 183, 398. Léger, L. (1947) Pr. méd. Dec. 20, p. 489. Le Lorier, V. (1937) Rev. franç. Gynéc. 32, 325. Mazingarbe, M. A. (1947) Mém. Acad. Chir. 73, 255. Mondor, M. H. (1939) Ibid, 65, 1271. — (1944) Ibid, 70, 96. Nylander, P. E. A. (1941) Wien. med. Wschr. 47, 955. Perrin, M. E. (1947) Mém. Acad. Chir. 73, 94. Tidy, Sir Henry L. (1939) Synopsis of Medicine. London and Bristol ; p. 858. Weber, F. P. (1939) Brit. J. Derm. 51, 132. — (1946) Rare Diseases and Some Debatable Subjects. London; p. 17. — Schwarz, E. (1932) Practitioner, 128, 421. MÉNIÈRE’S DISEASE SUCCESSFUL TREATMENT BY MINOR SURGER SAMUEL ROSEN M.D. OTOLOGIST, MOUNT SINAI HOSPITAL, NEW YORK CITY THE clinical entity of vertigo, tinnitus, and deafness first described by Prosper )11énière in 1861 was thought by him to arise from the internal ear. In 1938 Hallpike and Cairns demonstrated post mortem the first histo- logical findings in 2 cases of Meniere’s disease-a dilatation of the membranous labyrinth. After medical treatment has failed to control the attacks of vertigo, the patient is finally faced with surgery. The two operations most widely used for the vertigo are intracranial section of the auditory nerve and labyrinthotomy. The purpose of these operations is to break the connection between the end-organ (labyrinth) and the central connections in the brain. When the vestibular nerve alone is sectioned for the vertigo, in order to preserve the hearing, the vertigo often persists ; and this may also happen even when the labyrinth is destroyed. Since the internal ear is regarded as the sole anatomical structure from which the vertigo, vomiting, tinnitus, and deafness derive, treatment is naturally directed to the internal ear. The accepted otological operation today for unilateral Meniere’s disease is labyrinthotomy- complete destruction of the entire internal ear-which produces total deafness and total loss of vestibular function. All the operations for M6iii6re’s disease are based on the premise that the disease is unilateral. But what if both ears are or become affected ? Destruction of both labyrinths in such cases would be unthinkable ; yet in 10-15% of patients with Meniere’s disease the affliction is already present or subsequently develops on the supposedly healthy side. Some questions come to mind. Is the internal ear the exclusive anatomical basis of Meniere’s disease ? May neurogenic impulses other than those arising from the vestibular end-organ traverse altogether different pathways and ultimately influence the central vestibular mechanism and thus mediate the attacks of vertigo, nausea, and vomiting 1 Can severe attacks of Meniere’s disease (vertigo, tinnitus, nausea, vomiting, and falling) originate on the side of an already dead labyrinth where deafness and loss of vestibular function are already complete (Rosen 1953a) ? z? A few clinical observations may shed some light on these questions. The ideal operation for Meniere’s disease should be a minor one which, easily and quickly done under local anaesthesia, would abolish the vertigo and preserve the hearing and the static labyrinth. Such an operation could then be safely done even on both sides in bilateral cases. Possibly such an operation may now be at hand in the simple section of the chorda tympani through an ear speculum in the external auditory meatus, as described below. That the chorda tympani might serve sonic and equilibratory functions as well as taste first suggested itself in 1947, when the chorda tympani was used to cover the fenestra in the fenestration operation for otosclerosis (Rosen 1948-52a). In these cases the post- operative vertigo was strikingly reduced in degree and duration, and the hearing returned sooner than when the chorda tympani was not used to cover the fenestra. In view of these findings and after various trials with other neighbouring structures in the cat, the monkey, and man, section of the chorda tympani alone was finally used in the severe paroxysmal vertigo of -Al6ni6re’s disease. I report here the results of section of the chorda tympani in 50 consecutive cases of yleniere’s disease. Technique Section of the chorda tympani is done through an ordinary ear speculum in the external auditory meatus under local anaesthesia (skin injection of 1% procaine hydrochloride 1 ml. with 3 drops of 1-1000 adrenaline, or lignocaine 2% (’ Xylocaine’) 1 ml. with 3 drops of adrenaline chloride 1-1000). In half the cases pre- operative medication consisted of pentobarbitone sodium (’ Nembutal’) gr. 3 two hours before operation and subcutaneous pethidine hydrochloride (’ Demerol’ ) 100 mg. an hour before operation, and the other patients were not given any preoperative medication whatever. An incision is made from 3 o’clock to 9 o’clock through the skin of the posterior wall, floor, and anterior wall of the meatus about 6-7 mm. external to the drum. The skin is then reflected as far as the drum. The. lower half of the drum is lifted out of its sulcus and folded upwards upon itself, thus exposing the chorda-tympani nerve (fig. 1), which is sectioned. The intact drum is then returned to its natural position. This operation takes about ten minutes. The skin of the meatus heals completely in a few days. The patient is ambulatory on the day of operation, is on full diet, leaves the hospital next day and is usually back at work in a few days. Stimulation of Chorda Tympani This exposure of the chorda tympani afforded an unusual opportunity for studying the responses to its Fig. I-Drum membrane lifted out of its sulcus and folded upwards, exposing chorda tympani, shown resting on bipolar electrodes during electrical stimulation. Chorda tympani is next sectioned.

Transcript of MÉNIÈRE'S DISEASE

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It may occur to one that the pulmonary, ophthalmic,and nephrotic syndromes in the case of Mrs. A. B. wereeach due to a paroxysmal phlebetic attack of unknownxtiology but of the same nature as the phlebeticsyndrome in her extremities.

REFERENCES

Braun-Falco, O. (1953) Derm. Wschr. 127, 506.Calvet, M. J. (1944) Mém. Acad. Chir. 70, 445.Daniels, W. B. (1932) Amer. J. med. Sci. 183, 398.Léger, L. (1947) Pr. méd. Dec. 20, p. 489.Le Lorier, V. (1937) Rev. franç. Gynéc. 32, 325.Mazingarbe, M. A. (1947) Mém. Acad. Chir. 73, 255.Mondor, M. H. (1939) Ibid, 65, 1271.

— (1944) Ibid, 70, 96.Nylander, P. E. A. (1941) Wien. med. Wschr. 47, 955.Perrin, M. E. (1947) Mém. Acad. Chir. 73, 94.Tidy, Sir Henry L. (1939) Synopsis of Medicine. London and

Bristol ; p. 858.Weber, F. P. (1939) Brit. J. Derm. 51, 132.

— (1946) Rare Diseases and Some Debatable Subjects. London;p. 17.

— Schwarz, E. (1932) Practitioner, 128, 421.

MÉNIÈRE’S DISEASESUCCESSFUL TREATMENT BY MINOR SURGER

SAMUEL ROSENM.D.

OTOLOGIST, MOUNT SINAI HOSPITAL, NEW YORK CITY

THE clinical entity of vertigo, tinnitus, and deafnessfirst described by Prosper )11énière in 1861 was thoughtby him to arise from the internal ear. In 1938 Hallpikeand Cairns demonstrated post mortem the first histo-

logical findings in 2 cases of Meniere’s disease-a dilatationof the membranous labyrinth.After medical treatment has failed to control the

attacks of vertigo, the patient is finally faced with

surgery. The two operations most widely used for thevertigo are intracranial section of the auditory nerveand labyrinthotomy. The purpose of these operationsis to break the connection between the end-organ(labyrinth) and the central connections in the brain.When the vestibular nerve alone is sectioned for the

vertigo, in order to preserve the hearing, the vertigooften persists ; and this may also happen even when thelabyrinth is destroyed.

Since the internal ear is regarded as the sole anatomicalstructure from which the vertigo, vomiting, tinnitus, anddeafness derive, treatment is naturally directed to theinternal ear. The accepted otological operation todayfor unilateral Meniere’s disease is labyrinthotomy-complete destruction of the entire internal ear-whichproduces total deafness and total loss of vestibularfunction. All the operations for M6iii6re’s disease arebased on the premise that the disease is unilateral. Butwhat if both ears are or become affected ? Destructionof both labyrinths in such cases would be unthinkable ;yet in 10-15% of patients with Meniere’s disease theaffliction is already present or subsequently develops onthe supposedly healthy side.Some questions come to mind. Is the internal ear

the exclusive anatomical basis of Meniere’s disease ?

May neurogenic impulses other than those arising fromthe vestibular end-organ traverse altogether differentpathways and ultimately influence the central vestibularmechanism and thus mediate the attacks of vertigo,nausea, and vomiting 1 Can severe attacks of Meniere’sdisease (vertigo, tinnitus, nausea, vomiting, and falling)originate on the side of an already dead labyrinth wheredeafness and loss of vestibular function are alreadycomplete (Rosen 1953a) ? z? A few clinical observationsmay shed some light on these questions.The ideal operation for Meniere’s disease should be a

minor one which, easily and quickly done under localanaesthesia, would abolish the vertigo and preserve the

hearing and the static labyrinth. Such an operationcould then be safely done even on both sides in bilateralcases. Possibly such an operation may now be at handin the simple section of the chorda tympani through anear speculum in the external auditory meatus, as

described below.That the chorda tympani might serve sonic and

equilibratory functions as well as taste first suggesteditself in 1947, when the chorda tympani was used tocover the fenestra in the fenestration operation forotosclerosis (Rosen 1948-52a). In these cases the post-operative vertigo was strikingly reduced in degree andduration, and the hearing returned sooner than whenthe chorda tympani was not used to cover the fenestra.In view of these findings and after various trials withother neighbouring structures in the cat, the monkey,and man, section of the chorda tympani alone was

finally used in the severe paroxysmal vertigo of -Al6ni6re’sdisease. I report here the results of section of thechorda tympani in 50 consecutive cases of yleniere’sdisease.

TechniqueSection of the chorda tympani is done through an

ordinary ear speculum in the external auditory meatusunder local anaesthesia (skin injection of 1% procainehydrochloride 1 ml. with 3 drops of 1-1000 adrenaline,or lignocaine 2% (’ Xylocaine’) 1 ml. with 3 drops ofadrenaline chloride 1-1000). In half the cases pre-operative medication consisted of pentobarbitone sodium(’ Nembutal’) gr. 3 two hours before operation andsubcutaneous pethidine hydrochloride (’ Demerol’ ) 100

mg. an hour before operation, and the other patientswere not given any preoperative medication whatever.An incision is made from 3 o’clock to 9 o’clock through

the skin of the posterior wall, floor, and anterior wallof the meatus about 6-7 mm. external to the drum.The skin is then reflected as far as the drum. The. lowerhalf of the drum is lifted out of its sulcus and folded

upwards upon itself, thus exposing the chorda-tympaninerve (fig. 1), which is sectioned. The intact drum isthen returned to its natural position. This operationtakes about ten minutes. The skin of the meatus heals

completely in a few days. The patient is ambulatoryon the day of operation, is on full diet, leaves thehospital next day and is usually back at work in a fewdays.

Stimulation of Chorda TympaniThis exposure of the chorda tympani afforded an

unusual opportunity for studying the responses to its

Fig. I-Drum membrane lifted out of its sulcus and folded upwards,exposing chorda tympani, shown resting on bipolar electrodes duringelectrical stimulation. Chorda tympani is next sectioned.

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electrical stimulation (fig. 1) with a view to defining itsfunction (Rosen 1952b).

Stimulation was of three kinds : (1) a vibrationstimulator giving sharp pulses at 120 per sec. ; (2) athyrotron stimulator capable of giving pulses from verylow freqeuncies to about 500 per sec. ; and (3) a square-pulse stimulator capable of independent variation of

pulse width and strength. This was used with pulsesof 0-3 millisec. at frequencies of 50 cycles and 100 cyclesper sec., with pulses of 2 and 3 millisec. at frequenciesof 10, 25, and 50 cycles per sec. The purpose of usingthese various kinds of stimulation was to see whetheror not it was possible to produce different sensations bydifferent means and to eliminate as much as possiblethe spread of stimulation.

The results of experimental stimulation of the chorda-tympani nerve in 50 cases were as follows :

ResponseVertigo aloneVertigo and tinnitusTinnitus aloneTaste:

SpontaneousElicited..

Pain ..Facial contraction

In 40 of the cases tinnitus alone occurred with eachstimulation of the chorda tympani nerve. In the 5 caseswith vertigo it was described as identical with the

vertigo experienced during the attacks. The patientsreported hearing with each stimulation, sounds of hissing,drilling, buzzing, roaring, ringing, or bubbling. Painwas always localised deep in the ear and sometimesreferred to the back teeth (Rosen 1953b). The facialmuscles contracted with each stimulation of the chorda.The absence of taste response on stimulation, in caseswith and without preoperative medication, is unexplainedand adds to the complexity of the physiology of taste.

The Patients and the Results

Section of the chorda tympani was done in 50 con-secutive cases of Meniere’s disease in which medicaltreatment had failed. The symptoms and findings inthese cases varied in degree and consisted mainly insevere paroxysmal vertigo, nausea, vomiting, falling,veering, unsteadiness, fluctuating deafness (average45-50 decibels) with lessened bone conduction, variedtinnitus, subjective and objective recruitment, and

diplacusis. The caloric tests gave hypo-active responsesmore often than normal responses. Several patientshad a bitter taste or dryness in the mouth for weeksor months, especially before an impending attack. One

patient when tested had no taste for bitter, sweet, sour,and salt on the anterior two-thirds of the tongue duringthe attack of vertigo, nausea, tinnitus, and deafness,which persisted for two weeks ; as the attack of Meniere’sdisease abated, the taste upon the tongue for bitter, sweet,sour, and salt gradually returned to normal. About 15%of patients with Meniere’s disease have abnormal tastereactions on the anterior two-thirds of the tongue.The Meniere’s symptoms in 48 of the cases were presentfor from one to fourteen years, and in 2 cases for lessthan a year. Both chordae tympani were sectioned forbilateral Meniere’s disease in 5 cases. The results aresummarised in the accompanying table.

It will be seen that after section of the chorda tympani41 (82%) patients are free from major Meniere’s attacksof vertigo, nausea, and vomiting. Minor attacks do notincapacitate the patient or keep him from his dailywork or activities ; they consist mostly in transientmomentary giddiness, usually mild, without the rotatoryvertigo and vomiting which characterised the formerattacks. In all the cases the preoperative hearing waspreserved-often useful hearing. (2 patients have since

RESULTS OF SECTION OF CHORDA TYMPANI IN MENliSBE’SI

DISEASE

82 % of patients are free from major Meniere’s attacks.

been seen with normal hearing.) The tinnitus was Ilessened in about a third of the cases and disappearedaltogether in a few. In some the tinnitus remained rconspicuously annoying. None of the patients seem tohave been made worse in any way by section of thechorda tympani. During the first few weeks after the =

operation attacks of vertigo of various degrees of severity =

occur in about 15% of the cases but they gradually Ipass off. The proper assessment of any treatment is ;difficult in Meniere’s disease, because spontaneous remis. isions are not uncommon and often a large psychological Ielement is present. Psychiatric, medical, and neuro..logical consultations were obtained whenever they =seemed indicated. l

2 cases of severe Meniere’s disease occurred on the side l

of an apparently dead labyrinth :The first patient, a man of 50, had total deafness, constant

hissing, and roaring tinnitus in the right ear as well as severe 0paroxysmal vertigo, nausea, vomiting, and falling. Totaldeafness was determined by testing the affected ear withboth pure-tone and speech audiometry, with the normal =

ear masked at various measured levels of intensity. The istatic labyrinth did not respond at all to stimulation withice-water. This syndrome, which persisted for four months, ;followed a skull fracture through the petrous pyramid on ithe right side. His left ear was normal in all respects. The

right chorda tympani was sectioned two and a half years ago,after which the vertigo and almost all the tinnitus promptlydisappeared and have not recurred.The second patient, a man of 37, had frequent attacks of

severe rotatory vertigo, nausea, vomiting, and constanttinnitus in the left ear for three years following an attackof mumps. There was almost total deafness in the left earand no caloric response. The patient could not recognisesour, bitter, or salt on the anterior two-thirds of his tonguebilaterally, but he recognised these on the posterior third ofhis tongue promptly. The left chorda tympani was sectionedtwenty months ago, after which the vertigo and almostall the tinnitus also promptly disappeared and have notrecurred.

In view of these 2 cases, as well as published reportsof persistent vertigo after total or partial section ofthe auditory nerve and after labyrinthotomy, it seemsthat the internal ear may not be the exclusive anatomicalbasis for Meniere’s disease.

Discussion

The following explanation makes intelligible the dis-appearance of vertigo after section of the chorda tympani.The chorda tympani and the nervus intermedius (thesensory root of the facial nerve) may be regarded func,tionally as one continuous nerve, being connected inthe geniculate ganglion. The nervus intermedius ascendsfrom the geniculate ganglion and, after giving a branchto the vestibular portion of the auditory nerve in theinternal auditory meatus, joins the tractus solitarius toend in the sensory nucleus of the facial nerve in closeassociation with the vestibular and cochlear nuclei(fig. 2). Vertigo could thus be produced by stimulipassing up the chorda tympani and somehow affectingthe vestibular and cochlear nuclei. Since this pathway

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by-passes the internal ear, section of the chorda tympanicould explain the abolition of vertigo and severe tinnitusin the 2 cases with an already dead labyrinth.The introduction of the chorda tympani into the

aetiology of Meniere’s disease requires a reorientationand modification of the traditional concept that theinternal ear is the exclusive structure from which thesyndrome arises. Since the sensory fibres of the chorda

tympani have traditionally been believed to be con-cerned solely with taste, clinical and experimentalevidence presented here suggests that the chorda tympaniis a mixed sensory nerve having sonic-equilibratory,touch, and pain as well as taste functions. Somethingnew has now been added in the treatment of Meniere’sdisease. To follow blindly the old concept of the mecha-nism of Meniere’s disease leads inevitably to destructionof the vital internal ear-a deprivation which can be

Fig. 2-Chorda tympani and nervus intermedius joining tractus soli-tarius to end in facial sensory nucleus in close association withvestibular and cochlear nuclei.

avoided in a large proportion of cases by section of thechorda tympani. To destroy the labyrinth before tryingsection of the chorda tympani is to reverse the rationalorder of procedure.

Section of the chorda tympani is a simple operationrequiring only one day’s stay in hospital. It should bedone whenever labyrinthotomy or section of the auditorynerve is contemplated, in bilateral cases, and in caseswhere the vertigo is severe but not yet quite disablingenough for radical surgery. By this method the greatmajority of patients crippled by Meniere’s disease maybe restored to useful and happier lives.

SummaryA new pathway for Meniere’s disease is suggested

which includes the chorda tympani and the nervusintermedius.

Section of the chorda tympani has eliminated the majorattacks of vertigo and vomiting in most of the patientsso far treated.

REFERENCES

Hallpike, C. S., Cairns, H. (1938) J. Laryng. 53, 625.Ménière, P. (1861) Gaz. méd. Paris, 16, 597.Rosen, S. (1948) Arch. Otolaryng., Chicago, 47, 428.

— (1949) Ibid, 50, 81.— (1951) Ann. Otol., &c., St Louis, 60, 657.— (1952a) Arch. Otolaryng., Chicago, 56, 152.— (1952b) Neurology, 2, 244.— (1953a) N.Y. St. J. Med. 53, 1095.— (1953b) Arch. Neurol. Psychiat., Chicago, 69, 375.

THE SIMPLE GANGLIONA REVIEW OF MODES OF TREATMENT

AND AN EXPLANATION OF THE FREQUENTFAILURES OF SURGERY

B. V. McEVEDYM.A., B.M. Oxfd, F.R.C.S.

RESIDENT SURGICAL OFFICER, ANCOATS HOSPITAL, MANCHESTER

A PATIENT with a ganglion presents primarily becauseof the unsightly swelling, and secondarily because of theminor weakness at the site. Treatment is thereforeaimed at improving the cosmetic appearance, and at thesame time curing the weakness.

Modes of Treatment

Many forms of treatment have been used, of which themost important are (1) expectant treatment ; (2) surgicalremoval; and (3) injection therapy.

This paper records the late results in 79 cases treatedabout thirteen years ago.

Expectant TreatmentIn 21 cases, 19 of them in young women, no treatment

was given. Of these 19, 11 still complain of an unsightlylump and slight weakness after more than ten years.Thus conservative treatment is obviously unsatisfactoryin a condition which is of sufficient annoyance to the

patients to bring them to hospital.Surgical Removal

This is by no means a simple operation, in view ofthe risk of damage to the important structures nearwhich the ganglion usually lies.

In the present series, of 15 patients submitted tooperation on an average eleven years ago, 9 have hadno recurrence of their swelling, but 4 of these still com-plain of weakness at the site ; of the remaining 6 thatrecurred 2 had no further treatment and their cystsremain after 10 years ; and 4 had a further operation,curing 2 of them, but followed by recurrence in theother 2. We therefore see that to cure 11 patients outof 15 nineteen operations were required, and 4 patientswere left not only with their original swelling and weak-ness but also with a large, stretched, unsightly scar.

Besides this, 8 of the patients were treated as inpatientsand spent an average of five days in hospital.Thus the disadvantages of surgical removal are :(1) The high recurrence-rate (40 % in this series).(2) The production of a scar in the cure of a largely cosmetic

complaint.(3) Failure in many cases to relieve the weakness.(4) The occupation of valuable hospital beds.(5) Risks to important structures in operations done by

inexperienced surgeons or, alternatively, taking up the timeof competent surgeons who might be more usefully engaged.

(6) The unsightly results of failure.

Injection TherapyHandfield-Jones (1948) has said that " no words can

be too strong to condemn this practice" in view of thepossibility of the ganglion being connected with jointsand tendon sheaths which might be damaged by theinjected material.At Ancoats Hospital injection of ganglions has been

the usual form of treatment since 1930 (McEvedy 1930)and has beenperformed in several hundred cases. Therehas been no evidence of any damage to joint or tendon,and it has never been complicated by anything moreserious than moderate pain responding to mild analgesics,or local oedema settling with rest. All the patients areinjected as outpatients, usually on the Saturday, andfew are unfit for work on the following Monday.

Of the present series of 43 patients treated by injectionabout thirteen years ago only 8 still have a swelling.In their treatment sixty-two injections were required,averaging about one and a half for each patient. Sodium