Ménière's disease: Diagnosis and treatment

Ml?NIfiRE’S

DIAGNOSIS AND TREATMENT

DISEASE:

REPORT OF THIRTY CASES WALTER

E. DANDY,

M.D.,

BALTIMORE,

MD.

F.A.C.S.

MIGHT preface my remarks by two genera1 statements concerning MCni&re’s disease: T J- I. It is easy to diagnose and the diagnosis is unmistakabIe. 2. It can be permanentjy cured and at practicaIIy no risk to Iife or function. In 1928 a preIiminary report was made upon the operative resuIts on g patients afllicted with M&&-e’s disease. A tota of 30 patients who have been operated upon forms the basis of this report. In 1861 M&&e, a Frenchman, described a syndrome which remains practicaIIy unchanged today. The story of M&i&e’s disease is much Iike this: A patient who is perfectIy we11 in every respect, suddenIy, without warning and without apparent cause, is seized with a terrific dizzy speI1, Iasting for many minutes, sometimes hours or even days. The dizzy speI1 passes off and the patient is again perfectIy normaI unti1 at a Iater time a simiIar attack deveIops, and again without warning. From that time on, Iife is a succession of just such attacks occurring at varying intervaIs of time. Associated with the attacks there is usuaIIy vomiting and nausea. And most important of a11 there is aImost aIways tin&us and aIways subtota1 deafness, and both are in the same ear. M&%-e’s disease, therefore, is a series of attacks of terrific dizziness, nausea, vomiting and tinnitus, grafted upon uniIateraI defective hearing. The deafness is progressive. A mistake in our conception about M&&-e’s description of the pathoIogy of M&x-e’s disease shouId be noted. As a matter of fact he had never seen an autopsy of a case of true 693

694

WALTER

E. DANDY

M&&-e’s disease. He did make a pathoIogica1 report of a very dissimiIar cIinica1 condition, nameIy, acute Iabyrinthitis; and he described, as one might expect, the course of puruIent and

FIG. I. Scar of author’s operation

for MkniZxe’s disease.

hemorrhagic exudate in the Iabyrinth. That, of course, was before the deveIopment of bacterioIogy. At the present time most textbooks carry the statement that M&&e’s disease is due to a hemorrhage in the interna ear. But, as I shaI1 expIain, M&&e’s disease has probabIy nothing to do with the inner ear. It is mereIy an expIosion of the function of the inner ear and is due to a Iesion of the auditory nerve. There are two conditions which may be confused with M&3-e’s disease in differentia1 diagnosis : one, pseudoM&&-e’s attacks, perhaps quite seriousIy; and the other, cerebeIIopontine tumor, onIy supe&ciaIIy. In the former the symptoms are much alike but the signs dissimiIar; in the Iatter the signs are quite simiIar but the symptoms are never the same. In pseudo-M&&-e’s disease the attacks are exactIy Iike those of M&&e’s disease, but there is never uniIatera1

MCNICRE’S

DISEASE

695

deafness. These attacks come on without any change in hearing and upon this distinction aIone one can make the differentia1 one where diagnosis which, of course, is an a11 important

FIG. 2. Line of incision, size and position of bony defect for approach to sensory root of eighth nerve.

therapy pontine

is considered. The other condition is a cerebeIIotumor. ObjectiveIy there are two findings (often onIy

one) in M&-&-e’s disease, both are referabIe to the acoustic nerve. One is a reduction in hearing, but the deafness is not tota1; the other is a diminished caIoric response. When the ear is irrigated with hot or coId water there may be a diminished thermic response. However, onIy about haIf of our cases give such a change. In many instances, therefore, the objective signs of M&A-e’s disease are referabIe to both the acoustic and the vestibuIar divisions of the eighth nerve. It happens that these are the objective signs that obtain in the earIy cases of acoustic tumors. But the resembIance between M&A-e’s disease and acoustic tumors ends there, for aIthough there is a gradua1 Ioss of hearing from acoustic tumors they never

696

WALTER

E. DANDY

cause the attacks of dizziness that characterize M&&-e’s disease. M&&-e’s disease has run the gamut of varied forms of

FIG. 3. Operative

sketch showing method of sectioning

eighth nerve for MCni&e’s disease.

treatment; practicaIIy everything has been suggested. As a matter of fact, nothing has been of the sIightest benefit unti1 the recent advent of the operation of which I shaI1 speak brieffy. The great Charcot who was a contemporary of Mitniitre was very much interested in this condition and made the very astute observation that if a patient became stone deaf in the affected ear (an unusua1 occurrence) the attacks ceased. At that time Charcot suggested (and that was before the dawn of aseptic surgery) that perhaps at some time in the future surgery wouId deveIop to such a point that it might be possibIe to divide the eighth nerve and cure M&Gre’s disease. Charcot’s suggestion is the treatment of M&-&-e’s disease today. We have now had 30 cases in which the nerve has been sectioned intracraniaIIy, and in not a singIe case (and some of

M6NIkRE’S

DISEASE

697

our cases now have gone over a period of about six years) has there been a singIe subsequent attack resembhng Merrier-e’s disease in any patient. It reaIIy offers an aImost perfect cure because the patient Ioses nothing by section of the auditory nerve, except the remains of hearing which are of no practica1 vaIue. The treatment entaiIs practicaIIy no risk. There has been no fataIity, and there have been no after effects, except in two of the earIy cases when the facia1 nerve was injured. That wiI1 not happen again. The facia1 nerve Iies in very cIose apposition to the eighth nerve, but it is now a very simpIe matter to separate them and to avoid injury to the former. We know the pathoIogy, we do not know the etioIogy, but just as in trigemina1 neuraIgia, we have Iearned that the disease may be permanentIy cured and without subsequent attacks, by section of the ventra1 root of that nerve. Just a word about the etioIogy and pathoIogy of Mitnikre’s disease. We know of no cause for Meniere’sdisease. CertainIy there is no infection. Never does M&&-e’s disease foIIow infections of the middIe ear. And so far as we have been abIe to Iearn from our series of cases, there has been no foca1 or systemic infection to which this condition might be ascribed. So far as I know a necropsy of a case of M&&-e’s disease has never been reported. However, this point seems cIear in regard to the pathology of Merrier-e’s disease: We know (I) that the acoustic division of the auditory nerve is affected because of the Ioss of hearing, and that (2) the vestibuIar branch is affected because of the diminished caIoric response. It is necessary to assume, therefore, that we are deaIing with a Iesion not in the middJe ear, nor in the inner ear, but rather in the auditory nerve. A Iesion of the inner ear couId not affect both the acoustic and auditory divisions of the eighth nerve. We beIieve the attacks of M&&e’s disease are preciseIy simiIar to the paroxysmaZ attacks in trigemina1 and gIossopharyngea1 neuraIgia. For reasons that have been advanced

698 ekewhere

WALTER

we beIieve

that

E. DANDY

the cause

of trigemina1

neuraIgia

is

aIways in the sensory root of the nerve. And for the reasons mentioned above we think there is ampIe proof that the Iesion of M&&-e’s disease Iies in the sensory root of the auditory nerve. DISCUSSION

DR. CLAUDE C. COLEMAN, Richmond, Va.: A great deaI has been known about Mimi&e’s syndrome since about 1861, but there was no successful treatment of the condition unti1 1928, when Dr. Dandy proposed a cIear-cut surgica1 M&A-e’s syndrome, for which he advocated intracrania1 section of the auditory nerve on the affected side. At that time Dr. Dandy published a report on g cases which had been reheved by this operation. Since Dr. Dandy’s report, we have operated upon 12 cases by his technique, with perfectIy satisfactory resuIts in every case so far as the vertigo was concerned. Tinnitus has not been entireIy aboIished in every case, but if not aboIished, it has been improved. WhiIe a few isoIated attempts had been made to reIieve vertigo by division of the auditory nerve, the credit for pIacing the syndrome upon a sound surgica1 basis is due to Dr. Dandy. He has referred in his paper to the simiIarity of M&i&e’s disease to tic douIoureux, and this simiIarity impresses itseIf upon anyone who has had experience with the surgica1 treatment of both diseases. The attacks in both conditions are of paroxysma1 and expIosive character. The operation in neither carries more than a negIigibIe risk, and aItogether the operation for either tic douIoureux or M&&re’s disease is one of the most successful in crania1 surgery. In the 12 cases in our series we have had no bad resuIts, except a temporary facia1 paraIysis in two. Both of these patients recovered from the facia1 paraIysis in a short time. The operation is done satisfactoriIy under IocaI anesthesia, suppIemented by some such drug as morphine or hyoscine. There is every reason to beIieve that the operation wiI1 become more and more used, because of its satisfactory resuIts, and because there are a considerabIe number of such cases. It might seem on first thought that the operation is a formidabIe procedure, and might cause some patients to hesitate because of the tota deafness on one side produced by the operation. ParadoxicaI as it may seem, our patients generaIIy stated that they heard better fohowing operation than before, due to the fact that the disturbing tinnitus was either removed or very considerabIy improved.