NEGLECT OF EAR-SYMPTOMS IN THE DIAG- 
NOSIS OF DISEASES OF THE NERVOUS 
SYSTEM 
BY 
G. L. WALTON. M.D. 
BOSTON 
[Reprinted from the Journal of Nervous and Mental Disease, Vol. x. No. 4, 
October, 1883] NEGLECT OF EAR-SYMPTOMS IN THE DIAG- 
NOSIS OF DISEASES OF THE NERVOUS 
SYSTEM.* 
By G. L. WALTON, M.D., 
BOSTON. 
THE great advance in the knowledge of the nervous 
system, made during the past twenty years, is due 
not simply to laboratory investigation, but largely to clin- 
ical observation and study. 
One branch of clinical work seems, however, to have 
been left far behind in this connection, for the study of the 
ear attracts but little more interest to-day among neurolo- 
gists than it did twenty years ago. This is the more 
remarkable in that otological research itself, while limited 
for the most part to specialists, occupies at present a front 
rank in scientific progress. 
That the general practitioner should gain an extended 
knowledge of the ear is perhaps too much to expect, 
although it is unfortunate for every-day practice that the 
interest in this line of work should be at such a very low 
* Read before the Suffolk District Medical Society, Section for Clinical Medi- 
cine, Boston, October io, 1883 
Reprinted from the Journal of Nervous and Mental Disease, Vol. 
x. No. 4, October, 1883. 2 
G. L. WALTON. 
ebb. Dr. Edward H. Clarke,1 and more recently Dr. 
Woakes,2 have, for example, commented upon the lack of 
knowledge and interest which allows a practitioner to ignore 
aural symptoms in the exanthemata, and to inform the con- 
valescent from typhoid fever that deafness is a favorable 
sign ; the fact being that a little knowledge of the pathology 
of the ear, a little forethought, and a moderate degree of 
experience might have prevented, for example, a hyper- 
aemia from becoming a purulent inflammation, causing such 
destruction of tissue as to render the ear useless as an 
organ of special sense, to say nothing of the dangers from 
extension of the process. 
It is not, however, the object of the present paper to dis- 
cuss the gaps in the general practitioner’s knowledge, but to 
consider briefly the interest which the study of otology 
should have for the neurologist, and for the practitioner 
interested in neurology. 
It has now become so much a part of the neurologist’s 
education to acquaint himself with the examination of the 
eye, and of his practice to interest himself in it, that the 
diagnosis of cerebral tumor or locomoter ataxia is rarely 
made without examination of the fundus oculi, while the 
mention of “ blindness ” as a symptom in cerebral disease, 
unaccompanied by a description of the eyes and the exact 
nature of the defective vision, would call down severe criti- 
cism. Almost every number of the neurological journals 
contains a discussion either of the course of the fibres of the 
optic nerve or of some other question regarding the physi- 
ology or pathology of the eye. 
The result of this enthusiastic study is that as large a 
part perhaps of neurological advance is due to ophthalmo- 
logical research as to any one branch of investigation. 
1 Am. Jota. Med. Sciences, January, 1858. 
5 “ Deafness, Giddiness, and Noises in the Head.’’ Edward Woakes, M.D., 
London, 1879. EAR-SYMPTOMS IN NERVOUS DISEASES. 
3 
Now, the situation of the optic nerve is such as to render 
it peculiarly susceptible to alteration from cerebral lesion, 
while the relation of the auditory nerve to the brain is not 
such as to warrant the assumption that the study of its 
functions would throw an equal amount of light upon 
nervous pathology. It is also true that the optic nerve is 
generally eligible for direct examination, while the auditory 
nerve is not. Notwithstanding these facts, none would 
claim that the neurologist has nothing to gain from the 
study of the ear, and it is highly probable that systematic 
examination of the hearing and of the ear in cerebral dis- 
ease, whether deafness is suspected or not, would, like the 
examination of the sight and the eye, not only aid much 
in actual diagnosis, but add greatly to our knowledge of the 
central nervous system. 
As a recent example of the value of adding aural to 
ophthalmological study in diagnosticating central nervous 
disease may be cited the subject of hysteria. Thanks in 
greatest measure to the efforts of Prof. Charcot and his pupils, 
this disease has been rescued from the uncertainty and con- 
tempt of former times, and has been found as worthy of 
scientific study as any other, and once subjected to sys- 
tematic analysis it repays us by solving physiological prob- 
lems with an accuracy not to be attained in laboratory 
research. 
Prior to the studies at the Salpetriere hysterical blindness 
was considered a vague symptom unworthy of careful in- 
vestigation. It has now been known for some time that 
this, like the other functional anaesthesias, is subject to 
given laws and offers marked and individual characteristics, 
so that amblyopia, with concentric retraction of the field of 
vision and loss of certain colors has become a pathognomonic 
symptom. In a similar way hysterical deafness, long 
noticed but not investigated, has now been shown to follow 4 
G. L. WALTON. 
equally fixed laws, the hearing through the bone disap- 
pearing before that through the air, and that for high be- 
fore that for middle tones.1 The writer has already found a 
knowledge of these peculiarities of great practical value, in 
connection with other symptoms, in establishing the diag- 
nosis of functional anaesthesia,2 a diagnosis of importance, 
not only with regard to treatment and prognosis, but par- 
ticularly in a medico-legal point of view in cases of so-called 
“ railway spine,” as pointed 6ut recently by Dr. J. J. Put- 
nam.3 With regard to the physiological interest of these 
facts, assuming as we may fairly do that the deafness is due 
in these cases to disturbance in the cerebral centres, the 
analogy at once appears between hysterical and senile 
deafness. In the latter also the hearing through the bone 
and for high tones disappears first. Previous explanation 
of these peculiarities, as, for example, by the assumption of 
impaired bone-conduction in old age, has never been satis- 
factory; and the same peculiarities occurring in young girls, 
with no evidence of alteration in the conducting media, 
but with other evidences of anaesthesia of central origin, 
tends to show that the hearing for high tones and that 
through the bone disappear first during impairment of the 
auditory centres, simply because these are the sounds of 
which those centres are the least tenacious. 
It will not be out of place at this point to observe thart 
the importance of testing the hearing for high tones, as 
proposed by Dr. Clarence J. Blake,4 has indeed hardly at- 
1 Deafness in hysterical hemianaesthesia. Brain, No. xx, 1883; also 
Verhandlungcn derphysiologischen Gesellschaft zu Berlin, Feb. 9, 1883. 
3 In one case, published in the Archives of Medicine, Aug., 1883, the hear- 
ing was examined with Kbnig’s rods at intervals during convalescence, and it 
was found that the scale of notes audible on the affected side gradually rose un- 
til the rod of 30,000 vibrations could be heard on this side, to that of 35,000 on 
the other. The diagnosis in this case was settled by the disappearance of the 
deafness on application of an electro-magnet. 
3 Boston Med. and Surg. Journal, Sept. 6, 1883. 
4 Summary of the results of experiments on the perception of high musical 
tones. Trans. Am. Otological Soc., 1872. Diagnostic value of high musi- 
cal tones. Trans. Am. Otological Soc., 1873. EAR-SYMPTOMS IN NERVOUS DISEASES. 
5 
tracted sufficient attention, even among otologists, though 
already, in 1877, the “ m£thode de Blake ” was described by 
Guerder 1 as furnishing the key to diagnosis between deaf- 
ness resulting from lesion of the nervous apparatus and that 
resulting from impaired transmission. Dr. Blake has shown, 
by testing for high tones after incision of the membrana 
tympani, that the inner ear is capable of conveying, and the 
brain of perceiving, normally, at least 80,000 vibrations, al- 
though the ordinary limit while the membrane is intact may 
be placed at 40,000 vibrations. It is certainly remarkable 
that the high tones have received so little attention, and 
that the Konig’s rods are so little in general use for diag- 
nosis. 
The attention of the writer was particularly drawn some 
time ago to the lack of attention given the ear in diagnos- 
ticating cerebral disease, while looking through the neuro- 
logical journals. Attention having been attracted to the 
subject, the search for cases of cerebral disease has been 
continued at some length through leading German, French, 
English, and American journals, simply to observe how 
often note is taken of the aural symptoms, and when taken, 
how often systematic examination of the ear is made. 
The result has not been satisfactory. The ocular symp- 
toms are rarely neglected; the condition of the fundus is 
almost invariably reported where it can throw the least light 
on the nature of the lesion. It is needless to say that the 
statement is rarely found that defective vision exists, 
without such information as tends to show whether the 
difficulty is due, for instance, to opacity of the lens, optic 
neuritis, or refractive irregularity. 
The contrast between this desirable accuracy and the 
loose statements with regard to the aural symptoms, not 
so noticeable in the single reports, becomes absurd when a 
large number are passed in review. 
• Annales des maladies de l ’ oreille, du larynx, et des organes connexes. 6 
G. L. WALTON. 
The cases in which ear-symptoms are neglected in the 
diagnosis of diseases of the nervous system may be classed 
as follows : (1) The cases in which no note whatever is 
taken of the condition of the hearing, although the presence 
or absence of deafness would prove of diagnostic value; (2) 
the cases in which deafness is mentioned as a symptom in 
disease of the nervous system, without particulars as to its 
nature and degree, and without sufficient examination of 
the ear to eliminate defective transmission of sound. 
(1) With regard to the first class of cases, those in which 
the hearing is totally neglected, this is by far the most 
numerous, and comprises perhaps the large majority of 
cases of cerebral lesion, such as tumor, hemorrhage, and 
abscess, to say nothing of hysteria and allied disturbances. 
Why the auditory nerve in particular should be left out in 
the otherwise systematic analysis of such cases is not clear, 
and is only to be explained by the fact that attention has 
never been especially called to its importance. The fact 
that the examination of the hearing and of the ears is 
difficult, is certainly no reason for leaving them altogether 
out of consideration in reporting cases where they would 
be of value. 
The presence or absence of deafness is, for example, of 
the utmost importance in diagnosticating lesion of the pons 
or medulla oblongata, or of the cerebellum. As an illus- 
tration of this fact, Seymour has recently reported a case of 
cerebellar tumor pressing on the pons,1 in which absolute 
deafness (unilateral), both for sounds conveyed by air and for 
those conveyed by the skull, added greatly to the certainty 
of the diagnosis, which was afterward corroborated by post- 
mortem examination. 
Although this case unfortunately comes under the sec- 
ond class just mentioned, in that no examination of the ear 
1 Boston Med. and Surg. Journal, August 30, 1883. EAR-SYMPTOMS IN NERVOUS DISEASES. 
7 
was made, the probabilities were greatly in favor of the 
tumor as the origin of the deafness, and the case is quoted 
as a step in the right direction, chiefly because Nothnagel, 
in his standard work on the diagnosis of cerebral disease 
(edition of 1879), has stated that no authentic case is on 
record of deafness resulting from cerebellar disease.1 The 
same author, in considering lesions of the pons, remarks,4 
that considering the situation of the auditory nerve it is 
curious that deafness is rarely found as a symptom. The 
fact of its non-mention in a long series of cases he regards 
as proof of its rarity, for, he says, it is hardly probable that 
a marked degree of deafness could go unnoticed. This by 
no means follows, for deafness is a symptom which, in the 
majority of cases, unless carefully sought for, escapes the 
notice, not only of the patient himself, but of the medical 
practitioner, many persons going through life unaware of 
the fact that they are almost totally deaf in one ear. This 
point will be alluded to again farther on. By far the most 
probable explanation of the seeming rarity of deafness as a 
symptom in tumors and hemorrhages in the pons and cere- 
bellum is, that the hearing is rarely examined in such 
cases. 
(2) Examination of the second class of cases (those in 
which deafness is noticed but not accompanied by descrip- 
tion of its degree and nature, or by examination of the ear) 
reveals the lack of interest—not to say lack of knowledge— 
prevalent in this branch of symptomatology. 
Omitting the cases reported by otologists, a case is rarely 
found in which a systematic examination of the ear and of the 
hearing is made, so that in many cases the reader is even left 
in doubt as to whether the deafness is really due to the 
cerebral lesion, or, perhaps, to a plug of cerumen, or catarrh 
1 “ Topische Diagnostik der Gehirnkrankheiten,” von Dr. Hermann Noth- 
nagel, Berlin, 1879, S. 595. 
1 Ibid., S. 154. 8 
G. L. WALTON. 
of the middle ear. This neglect is the more noticeable in 
that the writers include observers otherwise most accurate, 
for it seems to be only the most careful clinicians who test 
the hearing at all. By these, the symptom deafness is re- 
peatedly mentioned in connection with the various dis- 
turbances of the central nervous system—such as locomotor 
ataxia, trauma, new growth, and hysteria,—without the least 
record of the hearing for different tones, or for that through 
the bone, and often with either no examination of the ears 
or a very superficial one. In some exceptionally careful re- 
ports appears the statement, “ membranes normal,” as if 
this fact absolutely established the central origin of the 
deafness, whereas it is well known to otologists that an 
apparently normal membrana tympani may have beyond 
it a middle ear so far injured by catarrhal inflammation as 
to cause marked loss of hearing.1 If, in such a case, the 
hearing through the bone and that for different tones is 
unimpaired, a central origin for the deafness is eliminated, 
and even if the hearing through the bone is lost, the fact is 
not yet established that the nervous auditory mechanism is 
affected, for catarrh of the middle ear, though generally 
apparently increasing the intensity of sounds conveyed by 
the bone, by preventing the passage outward of the vibra- 
tions, sometimes suspends the hearing for sounds conveyed 
in this way (probably by causing anchylosis of the stapes, 
thus preventing the vibration of the labyrinthine fluid). 
Even when disease of the outer and middle ear has been 
eliminated, we are by no means justified in jumping at once 
to the cerebral auditory centres, or even to the auditory 
fibres in the brain, for there still remain the intricacies of 
the labyrinth, and the course of the auditory nerve to the 
brain, either of which may be the seat of lesions causing 
1 Urbantschitsch : “ Traite des maladies de 1’ oreille,” trad, franc., 1881, 
p. 267. EAR-SYMPTOMS IN NERVOUS DISEASES. 
9 
loss of auditory function. In consideration of these facts 
how little scientific is the report, for example, of a case of 
locomotor ataxia with the symptom “ deafness ” mentioned 
without further particulars, and how unfortunate that the 
diagnosis of probable traumatic lesion of Ferrier’s auditory 
centre should be made, as in a case recently reported in the 
journal Brain, with no examination of the ear until some 
months after recovery (which in the case alluded to, by the 
way, revealed old purulent inflammation of the middle ear), 
and no more careful test for the hearing at any time than 
that for the watch by ear and through the skull, a test so 
untrustworthy as to be discarded by many otologists. 
Amongst other inaccuracies may be noticed the fact that 
the patient’s own statement is evidently often relied on in 
eliminating the question of deafness as a symptom. Now, 
the patient’s opinion on this point, even when he has 
noticed it, is generally unreliable, a deafness from catarrh 
or cerumen of several years’ standing being often considered 
by the patient to have commenced a few months pre- 
viously. This illustrates the well-known fact, that a con- 
siderable degree of deafness may go unnoticed for an 
indefinite time ; and, indeed, provided it be unilateral, the 
deafness may become total, and yet remain unperceived, 
until suddenly revealed by accident, as by rolling over upon 
the well ear in bed. 
These facts render the custom of questioning the patient 
with regard to his hearing of very little value as compared 
with objective tests. 
Even the tests for hearing by the voice, tuning-fork, and 
rods, are subject to such inaccuracies as to require careful 
study, considerable experience, and repetition in the given 
case. For example, the patient generally understands so 
well what is said to him by gesture, look, and the motion of 
the lips, as to render the voice test unreliable unless these 10 
G. L. WALTON. 
sources of error are removed. Deaf children are often 
brought to the aural clinics who not only deceive their 
parents, who are generally willing to be deceived, but who 
would deceive the medical practitioner unless he exercised 
great care, because they turn so quickly when a noise 
is made that it seems as if they must have heard it. The 
same children will, perhaps, take absolutely no notice of the 
shrillest sound if made stealthily behind the head by an 
experimenter who remembers that the field of vision 
extends laterally over not far from 180 degrees when 
the head remains quiet, and much farther when it is con- 
tinually in motion. 
These single instances of the many sources of error fa- 
miliar to otologists, will serve to remind us of the need of 
careful study before making scientific reports of the hearing 
power in cerebral disease. 
As an example of lack of care among neurologists in 
eliminating trouble in the ear itself, Lucae,1 as early as 
1866, exhibited the peripheral auditory apparatus of two 
patients who had died from locomotor ataxia, demonstrat- 
ing the real seat of the deafness, and calling attention to 
the fact that deafness in this disease should not be attribu- 
ted to central disturbance without careful examination of 
the ears. Since that time, however, deafness has been in 
many cases reported in connection with locomotor ataxia, 
sometimes with no examination, and sometimes with but 
the most casual examination of the ears, and Pierreta has 
even suggested, on the most insufficient evidence, that de- 
generation of the auditory nerve is one of the early symp- 
toms of this disease, the fact being probably that if 
peripheral disturbance were carefully eliminated, according 
to Lucae’s suggestion, the cases of deafness from locomotor 
1 Verhandl. der Berl. tried. Gesellsch., Bd. i, p. 127, 1866. 
5 Revue mensuelie, fevrier, 1877. EAR-SYMPTOMS IN NERVOUS DISEASES. 
11 
ataxia would be reduced to a minimum. Careful search by 
the writer through a series of forty cases has, indeed, failed 
to reveal a single case in which central deafness could be 
established, though the majority of the patients were more 
or less deaf. 
As another example, may be cited the frequency of the 
diagnosis and treatment of “Meniere’s disease” without 
examination of the ear, while, in fact, the so-called Me- 
niere’s complex of symptoms (deafness, noises in the ear, 
nausea, and giddiness, with or without tendency to fall in 
a particular direction) occurs so frequently in connection 
with disease of the external and middle ear, that the cases 
are comparatively rare in which otologists refer these 
troubles to lesion of the auditory nerve or its termi- 
nations. 
It is not the object of this paper to contend that the 
skill of an otologist should be added to the already varied 
requirements for neurological training. It is rather to offer 
the suggestion that the ear deserves an interest at least ap- 
proximating that accorded to the eye in the diagnosis of 
nervous diseases. It is certainly not too much to expect 
that every practitioner, whether neurologist or not, should 
either refer cases to a specialist or practise the examination 
of the ear and hearing to such an extent as to avail himself 
at least of the aid gained from the appearance of the mem- 
branes, the patency of the Eustachian tubes, and the hear- 
ing by air and bone by the various tests, as well as the 
hearing for different tones, before making a diagnosis of 
lesion of the nervous auditory apparatus. And further, it 
is to be hoped that the time is not far distant when reports 
of cerebral disease ignoring the condition of the hearing and 
the examination of the ears, will be considered as incom- 
plete as they are at present without record of the condition 
of the eyes. g. p. putnam’s sons, printers 
NEW YORK