VISUAL ACUTENESS OF THE SQUINTING EYE.

Whether the state of refraction or the condition of the muscular equilibrium is held to be the chief cause of squint, defective vision of one eye will always have to be acknowledged as one of the most important favouring circumstances; in order to cure squint it is important to have regard to the visual acuity of both eyes, and not only to the defective condition. But this is no easy matter.

First it is to be observed, that most cases arise at an age when an objective determination of refraction is possible, but when the visual acuteness cannot be determined. Even in children who have received slight instruction, it is frequently difficult to distinguish whether imperfect knowledge of the letters or faulty visual acuteness is the cause of the non-recognition of the test-letters; when testing the vision of children it is often better to use figures than letters.

Further, in these cases it is much to be desired that the habit of determining the refraction and visual acuteness at the same time should be discontinued, particularly in reduced visual acuteness, as the test-tables only contain a few letters, which have to be recognised at a distance of 5 to 6 metres. If they have once been read with one eye it may easily happen that in testing the second eye they are repeated from memory, without being clearly recognised; even a child soon learns the few letters by heart. Therefore, when it has been a case of determining the visual acuteness I have always conducted the examination at a distance of one metre, as the choice of letters or figures which can be employed at this distance is much larger than for greater distances. In every case the reading of test-letters must be used as an additional means of examination. We must never forget that the test of vision is a perfectly subjective examination, and that we are obliged first of all to accept the statements of patients as they are given without knowing what they are worth. I have met with patients in the most highly educated classes of society who, in intra-ocular troubles, for example, hæmorrhage of the retinal artery in the macula lutea, could not distinguish the largest type in the first examination, and the next day (perhaps with slight difficulty) could read small print.

Such inaccuracies may continue to exist during repeated examinations and for long periods. One of my patients, for instance, who first came under treatment in the year 1873, had extreme myopia in the left eye with good visual acuity; with the right eye, which was also myopic, and had suffered for several years from choroiditis of the macula lutea he could read only No. 20 Snellen, and a year later 7-1/2 was read with difficulty, word by word. Choroiditis of the macula lutea gradually developed in the left eye, and in the same proportion the statements as to visual acuteness of the right eye improved, so that finally at the end of 1881, 0·5 was read with difficulty with this eye, while the left still sufficed to read 0·4 (at about 5 cm.). As I tried to comfort the patient, who was very anxious about his left eye, with the fact that the right eye had considerably improved in the course of the year, he replied that he might previously have seen just as well with the right eye if he had only taken the trouble, this was certainly my own opinion.

The attention and intelligence shown by patients during examination materially influences its results, and one should never hold the first trial of vision to be conclusive. We must always remember, however, that all conclusions drawn from visual acuteness become more unreliable in proportion as the latter is slight. We must attend to some peculiar difficulties in testing the vision of those who squint or we shall be liable to make great mistakes. When testing the squinting eye, particularly in children, it is not sufficient merely to cover the other or to hold the hand over it, for they know how to bring the usual eye into fixation by holding the head on one side or peeping between the fingers; we must keep it carefully closed with a bandage.

It is still more frequently the case that visual acuteness is stated to be less than it is in reality. The result of always using the better eye for fixation is, that fixation is not learnt with the weaker one. Even where there is no squint we see very frequently that in one-sided hypermetropia the accommodation is only used in that proportion which has become habitual to the emmetropic eye and does not therefore suffice to produce clear retinal images, while good visual acuteness is obtained by means of the correcting convex glasses. In the case of squinters (even without difference of refraction) it happens very frequently that the first statements as to the visual power are considerably below the truth. Patients who assert that they can only read the largest print with difficulty, frequently read smaller, and even the smallest type without more trouble, and we must be careful to ascertain this at first. Accurate reports are usually obtained more quickly by means of convex glasses or eserine. In any case insufficient accommodation is, according to this, one of the difficulties, but not the only one, which has to be overcome before the squinting eye can be put into fixation. We can understand that the innervation necessary for distinct vision can be set aside even without loss of visual acuteness, just as we see the movement of convergence disappear without the interni losing their capacity for contraction.

In order to explain the relation between squint and defective vision, we must first consider the question hitherto neglected, or what is worse, answered with preconceived opinion, as to whether the same form of defective sight which is so common in squint also occurs without squint. No one doubts the existence of congenital amblyopia, nevertheless it has received but little attention in the handbooks on ophthalmology. Leber, for instance (in the well-known compilation, vol. v), does not mention it at all.

A more or less considerable reduction of visual acuteness, with good field of vision, normal sense of colour and normal ophthalmoscopic condition, are characteristic of congenital amblyopia. Colour-blindness may of course be present at the same time. I also hold as probable the very rare occurrence of congenital defects of the visual field in good central vision, but I will reserve for the present the few observations I possess on the subject.

Together with congenital defective vision we must consider the depreciation in visual acuteness usually present in nystagmus, although it might be asserted that it can neither be the cause nor the result of the nystagmus, for we find very considerable degrees of congenital defective sight in both eyes without nystagmus, as well as nystagmus with remarkably good visual acuteness. Not to complicate the question, however, I have excluded all cases of nystagmus from the following investigation. All cases of myopia of higher degree (i. e. of more than 6 D.) have also been excluded, as in such cases for various well-known reasons the full visual acuteness is never present. In the case of individual patients who remained for years under my observation I have been able to convince myself that visual acuteness decreased in accordance with the increase of myopia; on the other hand, however, it appeared to me very probable that just those cases of myopia, which from the beginning do not possess full visual acuteness, have a special tendency to increase quickly.

For instance, if the examination of a hypermetropic eye, whose defect can be exactly determined by means of the ophthalmoscope, shows very faulty visual acuteness which is but slightly or not at all improved on correction of the hypermetropia, it is clear that the cause of defective sight is not to be sought in the hypermetropia. It is just the same with astigmatism. In defective vision with astigmatism proved by means of the ophthalmoscope, how frequently it is the case that not even the slightest improvement can be obtained with cylindrical glasses. This is usually attributed to the presence of an irregular astigmatism situated near the asymmetric meridian. If we illuminate the eye by means of a plane mirror, and observe one spot on the pupillary area which looks sometimes bright, sometimes dark, during slight rotations of the glass, this appearance can only be caused by the above-mentioned irregularity of the refraction of light, and it will be easy to determine whether the same takes place in the cornea or in the lens. But if this appearance is not present then irregular astigmatism cannot be proved. It is purely intentional, or a play upon words, if we refer an existing defective sight to an optic cause which cannot be proved. For instance, if haziness of the cornea exists, it is not difficult to learn to estimate by practical experience whether the amount of visual disturbance corresponds to the optic irregularities caused by the opacities and irregular refraction of the cornea. Slightly nebulous corneæ with disproportionately bad vision were therefore included in the following statistics; however, they do not influence the result as there are only ten cases in all. On the other hand, considerable opacity of the corneæ or cases which were complicated with anterior synechia, &c., were excluded from the statistics.

If then we find defective vision, the development of which has not been noticed by the patient, together with normal ophthalmoscopic condition and full visual field, and if it is further seen that the condition remains unchanged for years, we have every reason for considering the defective sight to be congenital. The statements of patients must of course be received with caution. If congenital amblyopia of moderate degree exists in both eyes, patients do not usually know that it is possible for anyone to see better; if the congenital defect is one sided, it is generally only casually noticed on closing the better eye. We can scarcely doubt that it is a case of congenital amblyopia if it happens in children. Acquired defective sight without ophthalmoscopic cause seldom occurs among children. I have seen a few cases as a result of severe cerebral disease (hydrocephalus, for example); so-called anæsthesia retinæ, or amblyopia marked by contraction of the visual field is not quite so rare. It is easy to avoid confounding both these cases with congenital amblyopia.

One must be more careful about drawing conclusions with regard to adults, for on the one hand it happens that gradually developed monocular visual disturbances are only accidentally observed by patients after they have reached a high degree, and it is very difficult then to persuade these attentive observers that it is not a case of sudden blindness of one eye. (Only a few people seem to be really aware that they have two eyes, and still fewer appear to suspect the existence of a visual field.)

In all these cases opportunity is hardly given for mistakes with reference to the diagnosis of congenital amblyopia, as slowly developed monocular defect scarcely occurs without ophthalmoscopic cause. On the other hand, ophthalmoscopic symptoms (such as hæmorrhage of the retinal artery in the macula lutea) may disappear without leaving a trace, while defective vision remains. The law of habit, however, usually helps us here. In congenital monocular defect patients are generally accustomed to this condition, and only notice it when special claim is made on the visual faculty of this eye,—he, on the other hand, who is accustomed to see with two equally good eyes, may not observe a gradually occurring blindness of one eye, if his talent of observation be faulty, but I have never had reason to suppose that a rapid depreciation of the central visual acuteness of one eye is also overlooked. Rapidly occurring monocular visual disturbances are noticed, whether detected with or without the ophthalmoscope.

Two peculiarities appear in isolated cases of congenital amblyopia, which may render the testing of vision difficult: rapid fatigue of the retina, and depreciation of the central visual acuteness in such a way, that an adjoining part of the retina possesses a better visual faculty than the centre.

Rapid fatigue of the retina occurs in comparatively good visual acuteness. For example:

Case 16.—Mr. W—, æt. 35, came under treatment for conjunctivitis. In testing the vision, emmetropia (or doubtful hypermetropia) was found on the left, V. = 5/6. Refraction of right eye similar to that of left, V. = 5/18 to 5/12, but with rapidly occurring fatigue of the retina. Patient had observed this fifteen years before, when shooting during his period of army service. Position and movements of the eyes are normal.

This peculiarity occurs more often in higher degrees of defective vision. For example:

Case 17.—Mrs. von G—, æt. 60, has always seen badly with the left eye. On the right H. 1·25 D., V. 5/12. On the left with + 2 D., V. 1/12 with + 5 D. words of No. 1·75 were recognised; but the visual acuteness above stated is only present at the first moment; after a few seconds everything disappears in a fog. The left eye has a slightly conical nebulous cornea, detected only on focal illumination, which does not, however, cause the slightest irregular astigmatism, and cannot, therefore, serve as explanation of the defective sight.

This rapid fatigue, which only permits the visual acuity present to be estimated for a short period at a time, may easily result in the visual acuity being supposed to be worse than it is.

The other phenomenon above mentioned, which occurs in defective vision without being actually a necessary symptom, is the depreciation of the central visual acuity, which we designate as central scotoma in acquired amblyopia. It should be remembered that the visual acuteness which we determine under these conditions is something different from what we are usually accustomed to designate by this idea. When we simply talk of visual acuity we always imply the central visual acuity; however, in cases where the centre of the retina is so injured in its function, that the peripheral parts lying near are too often called into requisition, we do not determine the central visual acuity at all, but that of the nearest and at the same time best, excentric part. We cannot prevent patients from using that part of the retina which seems best to them for recognising the test objects. In such cases (just as in acquired central scotoma) continuous print is read badly, and with more trouble than one would expect from the visual acuteness which is specified in the recognition of single letters. Of course spelling and reading are two different things; the excentric visual acuity may perfectly suffice for the recognition of single letters, central and also excentric visual acuity is necessary for reading. There are patients who, despite full visual acuteness, are unable to read continuously, as soon as a defect in the right half of the visual field extends close to the fixation point. To read fluently, the excentric vision must work on in advance for the width of several letters, but if the first letter is seen excentrically, the excentric visual acuteness rapidly sinking in a physiological way, does not suffice for the following ones.

When testing the vision these circumstances should be carefully regarded. The apparent contradiction between the visual acuteness specified with test-letters, and the uncertainty in reading continuous print, may be taken for simulation (I have seen some sad examples of this in acquired central scotoma), and, on the other hand, if in the form of defective vision now under discussion we content ourselves by merely employing reading tests, we take the visual acuteness to be worse than it is, or than we find it later when single test-letters are used, for even though excentric, it is yet always visual acuteness. The excentricity of that part of the retina put into fixation is usually so slight, that the oblique direction of the visual axis cannot be seen with the naked eye; if considerable and extensive defect of the centre of the retina is present, either varying fixation occurs, sometimes parts lying to the nasal and sometimes to the temporal side are put into fixation; or excentric fixation exists; an inner retinal area but sometimes also a temporal then usually has comparatively the best visual acuteness.

A third peculiarity which sometimes occurs in extreme degrees of congenital amblyopia, is monocular nystagmus of the weak eye. This trembling may be so slight that it is only observable during investigation with the ophthalmoscope; in other cases it is most marked as soon as the weak eye is put into fixation by exclusion of the sound one.

Cases of congenital amblyopia in both eyes, where no explanatory cause can be traced, and no nystagmus is present, are rare, but all the more interesting. For instance:

Case 18.—Mr. F—, æt. 56, has seen badly from childhood; the visual acuteness of each eye singly examined amounts to 1/18 to 1/12, binocular 1/12. No. 0·75 is read with difficulty at 8 cm. Ophthalmoscopic condition is normal. In mydriasis by atropine hypermetropia of 3 to 4 dioptres results. With convex 3· 5 D. on the right V. 1/18 to 1/12, on the left V. 1/12, binocular V. 1/12 to 1/9, with convex 6 D. still only 0·75 can be read, but more fluently than with the naked eyes.

Normal binocular fusion may continue to exist even in extreme degrees of monocular weak sight; I have observed it up to a visual acuteness of 1/24. The stereoscope is well adapted to prove binocular fusion in these cases; only we must then take care that sufficiently large letters are present in the visual field of the defective eye, so that they may easily be recognised with the existing visual acuteness. Binocular fusion is naturally rendered still more difficult if the weak-sighted eye is at the same time hypermetropic to a high degree, as it then receives simultaneously indistinct retinal images on account of the difference of refraction; and yet in the above table there are 117 cases with hypermetropia of at least 2 D. in the better eye, and faulty visual acuteness in the other, 7 with visual acuteness of less than 1/7 to V. 1/12, and 9 with less than 1/12 to V. 1/36.

In the highest degrees of congenital defective vision, binocular fusion cannot as a rule be proved; partly because the methods of investigation by which we are able to prove binocular fusion presuppose the existence of a sufficient visual acuteness. On the other hand, it cannot be expected that normal binocular vision can be learnt with such a large amount of monocular defective vision. If the relative strength of the muscles is normal, so also are the position and movements of the eyes, if elastic preponderance on the part of the muscles is present, which in monocular defective vision of considerable degree is no longer governed by binocular fusion, and this is frequently the case, squint is developed.

Sometimes other congenital anomalies are present at the same time with congenital defective vision (for example, congenital dermoid growths on the edge of the cornea), and undoubtedly hereditary influences play a considerable rôle therein.

In order to determine the relation of congenital defective vision without squint, to defective vision with squint, I have taken those cases where congenital defective vision without squint was observed, together with the cases of squint, from the diaries of my private practice for the last ten years. I have personally investigated every case, and the observations on each were carefully examined before being included in the statistics; all cases with myopia of six or more dioptres, all cases of double nystagmus, and, finally, all those cases where the previous existence of squint might be suspected, were excluded, as above stated. I must also remark that before the last ten years I had not begun to collect these cases. In order to find monocular congenital defective vision one must seek for it, as patients usually come under treatment for quite different disorders, and in the consulting-room there is not always time carefully to investigate what possesses interest for us but none for the patient. In cases of squint the opportunity for investigating the power of vision does not escape us so easily, and yet the same list, which contains among 629 patients 177 cases of squint with a visual acuteness of 1/8 to less than 1/36, furnished at the same time 98 cases with undoubted congenital defective vision of the same high degree without squint, which I place together in the following review.

Cases of congenital amblyopia with visual acuteness of 1/7 are so frequent, that I have not drawn up special statistics of them. I was not anxious to collect a large number of cases but only material for evidence. I have therefore divided the 98 cases I observed into 3 groups. (1) Cases with visual acuteness of less than 1/7 to V. 1/12; (2) V. less than 1/12 to V. 1/36; (3) visual acuteness less than 1/36. The limits between these groups are of course not very sharply defined, for what is designated as "measurement" of visual acuteness contains, even if we accept the statements of patients as trustworthy, not an inconsiderable number of sources of error; and we often find a remarkable absence of conformity in the analysed causes of congenital amblyopia, according as we seek to determine the visual acuteness by means of single test-letters or by reading printed matter. In a case of visual acuteness of 1/12 No. 0·75 with convex 6 was the smallest type that could be read, and that with difficulty, larger type was usually required; and in one case where at first only single words of No. 2·25 were read with difficulty—this test was on that account repeated in myosis by eserine—No. 1·75 was finally the smallest print which could with the same difficulty be deciphered. In the division of the groups here arranged the best visual acuteness ascertained in the various examinations was always used as the basis.

A. Vision less than 1/7 to V. 1/12 38 cases. The examination of the better eye showed:

(a) Emmetropia in 18 cases. A determination of refraction, mostly ophthalmoscopic, of the weaker eye is submitted in 11 cases, which divide themselves into, 4 with emmetropia, 3 with hypermetropia (of H. 2 D. and 2·25 D.), 3 with hypermetropic astigmatism, I with myopic astigmatism.

(b) Myopia in 5 cases (3 of M. 1 D. to 1·5 D., 2 of M. 4·5 D. and 4 D.), the condition of the defective eye was determined in 3 cases, and was twice hypermetropic, once astigmatic.

(c) Hypermetropia in 8 cases, hypermetropic astigmatism in 3. In 4 cases an exact determination of refraction even of the better eye was for some reason impracticable.

There are 4 cases in this group where the visual acuteness in both eyes did not exceed the above-stated small amount, and one which was interesting from another point of view.

Case 19.—Max L—, æt. 8-1/2, recognises No. 24, and a few letters of 18 at 5 metres with the better eye with convex 6 D.; at 1 metre V. 1/4 to 1/3, the left eye recognises only No. 60 at 5 m. with + 6 D. at 1 m. No. 0·75 is read with difficulty. If we exclude one eye it lapses into now less, now greater convergence, and still no squint is present, but diplopia as well as binocular fusion can be proved by the aid of prisms. The theory of Donders that squint is less frequent in hypermetropia of high degree because too strong convergence would not suffice to furnish clear retinal images, is scarcely tenable in the face of such cases. If indistinct retinal images are added to a visual acuteness of only 1/3 to 1/4 still, even with faulty accommodation, it is difficult to believe how a child could learn to read if it did not hold the book close to its eyes, which was not the case here, and indeed seldom happens. Therefore, in spite of defective vision the accommodation must have sufficed, without sacrificing binocular fusion, whilst in all probability accommodative convergence followed on exclusion of one eye.

B. 48 cases had visual acuteness from 1/12 to 1/36. The better eye was—

(a) Emmetropic in 16 cases; in 6 of them the refraction of the defective eye was determined, which showed in one case emmetropia, 3 hypermetropia, 2 astigmatism.

(b) Myopia of the better eye was present in 7 cases (in 3 myopia of 1 D., in 4 M. 3 D. to 6 D.).

(c) Hypermetropia in 18, astigmatism in 4 cases. In 3 cases the condition of the better eye was, for some reason or other, indeterminable.

In this group I should like to point out the following cases as worthy of attention:

Case 20.—Margarethe T—, æt. 16, has hypermetropia 2 D. in the right eye, V. 5/6, in the left the ophthalmoscope shows with an otherwise normal condition a higher degree of hypermetropia, with + 6·5 D., V. 1/18, with + 10 D. No. 3·0 is read. No spectacles have been used until now; for the past few years school tasks have been performed with a certain effort, only during the last year the asthenopia has increased. Squint is not present, and with prisms as well as with the stereoscope (by the use of objects, whose size corresponds to the defective sight on the left side) binocular fusion can be proved.

The case is the same as regards divergent strabismus.

Case 21.—Mr. H—, æt. 28, has myopia 6 D., V. 6/9 in the right eye; the left eye has always been weak sighted, emmetropia is detected with the ophthalmoscope, with normal fundus, V. 1/18. No squint, binocular fusion can be proved with prisms.

Case 22.—Mr. B—, æt. 47, has hypermetropia 5 D., V. 5/9 in the right eye. Left eye with + 5 D., V. 1/18 (a few letters of 12 also were recognised at 1 m.). It seems, however, that the patient is not able exactly to indicate the position of the retinal images of his left eye, he does not know, as he expresses himself, "whether the letters stand here or there." Patient observed the defective sight long ago; the ophthalmoscopic condition is normal. Patient really comes on account of his son, aged 7-1/2, in whom hypermetropia of 3·5 dioptres is detected with the ophthalmoscope, right eye with + 3·5 V. 5/9. Left eye has convergent squint, V. 1/36, No. 3·0 is read with + 6·5 D.

The hereditary tendency is seen also in the following case:

Case 23.—Mrs. S—, æt. about 46, on the left H. 4 D., V. 5/18 to 5/12, has used no spectacles until now, and reads No. 0·75 without glasses at about 15 cm. R. with + 4 D., V. 1/18, with + 6·5 D. large letters of No. 5·0 are recognised.

Two sons, present at the same time, are hypermetropic. One has in either eye V. 1/4, the other a slighter degree of congenital amblyopia.

Case 24.—Johanna L—, æt. 4, came under treatment for a congenital fibroma covered with hair, about the size of a cherry-stone, situated on the outer corneal margin of the left eye, which was removed. Three years later, when the child had learnt to read, emmetropia and full visual acuteness was observed in the right eye, with the left No. 4·0 only is read with difficulty. The ophthalmoscope shows a slight degree of irregular astigmatism of the cornea, which in no way explains the defective vision; the image of the fundus is perfectly clear and quite normal.

Case 25 afforded me a not altogether pleasant surprise. Martin M—, æt. 58, has matured cataract in the right eye, with perfectly satisfactory light reflex, proper projection, &c. On the left progressive cloudiness of the lens has begun. The course of operation and cure were regular in every respect, but the power of vision finally was so small that with a clear pupillary area, and otherwise normal condition, only single words of No. 3·0 were recognised with difficulty at 10 to 15 cm. with convex 20 D. For the first time the patient remembers that he noticed the defective sight in his right eye at the age of sixteen, and was for this exempt from army service. The operation performed later on the left eye procured satisfactory vision.

C. Visual acuteness of less than 1/36 12 cases.

Determination of refraction of the better eye is given in 6 cases, and showed twice emmetropia, twice slight myopia, twice hypermetropia. I only possess an exact ophthalmoscopic determination of the condition of the defective-sighted eye in one instance with H. 2·5 D.

This group is of special interest in that it represents the extreme degrees of congenital amblyopia, and, on the other hand, because it contains 5 cases of children under 10 years of age.

Case 26.—Constanze von M—, æt. 9-1/2. Defective vision on the left side had been noticed long before by the child's parents. On May 1, 1879, emmetropia was observed in right eye, V. 5/12 to 5/9. No. 0·4 is read at 15 cm. On the left, only movements of the hand are seen, fingers cannot be counted even when close to the eye; the visual field is good, that is, on moving the hand in the periphery of the visual field the child sees "something" without being able to state what it is. Reaction of the pupils as rapid and equal as usual. The ophthalmoscopic condition (even with dilated pupils) is perfectly normal. All tests for simulation were of course applied.

On account of the importance of the case, I suggested another examination a year and a half later, on the 22nd December, 1880, which showed precisely the same result as the former one—optic disc, macula lutea, &c., perfectly normal, the ophthalmoscopic determination of the refraction shows H. 2·5 D.

The child's father also possesses in the left eye a slight degree of congenital defective sight, observed for many years, with normal ophthalmoscopic condition; No. 0·5 is read with + 6·5 D. at 10 cm.

Case 27.—Tina S—, æt. 6. The defective sight of the left eye had been remarked some months previously; report on July 16th, 1878: R. full visual acuteness, L. movements of the hand are scarcely visible. The child cannot count fingers. Normal ophthalmoscopic condition. Eserine and separate use were prescribed. On September 9th, 1878, fingers were counted with the left eye at 1·5 m., single words of No. 4·0 were recognised, No. 3·5 with convex 6·5 D., but always with oscillating fixation. The improvement in the child's statements may be referred to the fact that she had meanwhile learnt to form right conclusions from the very imperfect sensual impressions of her left eye.

Case 28.—Frank J—, æt. 10. Left eye. V. 10/50 to 10/40, No. 1-1/2 Snellen is read at 4 inches. On the right, nystagmus on fixation, fingers are counted at 5-6 feet. The ophthalmoscopic condition is normal. A sister of the boy squints.

Case 29.—Ernest G—, æt. 8, has slight nebulæ on both corneæ. On the left V. 15/40. On the right, fingers are counted at 4 inches with visual axis deviating inwards.

Case 30.—I operated on Moritz L— for congenital cataract before he was a year old in 1869 by means of a needle operation. In June, 1877, a thin ophthalmoscopically transparent secondary cataract appeared in both eyes; on the left, with convex 12 D. V. 3/24 to 3/18, with convex 16 D. No. 0·4 is read at 10 cm. On the right, with convex 12 D., fingers are counted with difficulty at about 1 m., with inward deviation of visual axis.

Case 31 is also worthy of note. Carl H—, æt. 22, shows quite a number of congenital anomalies on the left side of the face, harelip, deformed nostril and a skin defect on the inner corner of the eyelid. There is a congenital dermoid growth of the size of half a pea situated on the inner lower corneal margin. A slight irregularity in the curve of the cornea near the dermoid is detected with the ophthalmoscope; the fundus of the eye is perfectly normal. Fingers are not counted further off than a metre with visual axis deviating inwards. The right eye is emmetropic (perhaps slightly hypermetropic), and has full visual acuteness. There is no squint.

It is customary to "explain" these cases of monocular amblyopia by previously existing squint, and one is quite satisfied if by the examination of patients it is only possible to prove that they have occasionally squinted, although the advocates of the amblyopia ex anopsia disallow the presence of the same under these conditions, that is, in periodic squint. Of course a theory which cannot exist without the assertion that occasional alternation suffices to hinder the development of defective vision caused by disuse, cannot possibly hold periodic squint to be the cause of it. Certainly permanent squint may also disappear, but this much I have been able to determine, that this seldom happens before the twelfth year of life, and one may surely reckon that children in whom permanent squint is developed at the usual early period of life, still squint at the age of ten years. Cases 24 and 26 to 30 can under no circumstances be explained by previous squint, notwithstanding that they represent the extremest degrees of amblyopia, but the question is undoubtedly that of congenital defective vision; moreover I have excluded from the statistics of congenital amblyopia all cases in which the previous presence of squint could even be supposed.

A table of the cases above described with reference to the defective condition is interesting; when a determination of refraction existed for the weak eye I have given it, and when this was not the case I have stated that of the better eye, thus it is seen that among 85 cases in which the refraction was determined, hypermetropia (including hypermetropic astigmatism) was present in 39. Hypermetropia was found then in 47 per cent of all the cases. The percentage would probably be higher, if all weak-sighted eyes had been examined from the beginning as to their state of refraction, but as I only learnt to know the relation between hypermetropia and the higher degrees of congenital amblyopia from my statistics, I did not take notice of this relation when investigating individual cases.

How does congenital amblyopia now stand in relation to that disturbance of vision which we observe in squint? I see no difference; whether squint is present or not, the form of defective vision is precisely the same, and nothing happens in the combination with squint which could not also be proved without it. The relation to hypermetropia, which is proved with congenital amblyopia, also appears in squint.

A collective table of cases of convergent and divergent squint included in the statistics (pp. 19 and 47) shows:

(a) In myopia, emmetropia, and doubtful hypermetropia, with convergent and divergent squint together 329 cases. Among them:

Visual acuteness	to 1/7		239
"	less than	1/7 to V. 1/12	19
"	"	1/12 to V. 1/36	46
"	"	1/36	25

Defective sight of higher degree than 1/7, 27·3 per cent.

(b) In hypermetropia 1 to 3 D., including the few cases of hypermetropia with divergent squint, 177 cases. Among them:

Visual acuteness	to 1/7		121
"	less than	1/7 to V. 1/12	17
"	"	1/12 to V. 1/36	27
"	"	1/36	12

Defective sight then, 31·6 per cent.

Vision to 1/7	39
V. < 1/7 to V. 1/12	8
V. < 1/12 to V. 1/36	14
V. < 1/36	9

Defective sight then, 44·2 per cent.

This regular increase of defective sight with the increase of the hypermetropia can be no mere accident, and speaks strongly for the identity of defective vision in squint with congenital amblyopia. Were defective vision caused by the squint the various states of refraction would show no difference in the percentage of defective vision.

Further, the circumstance is worthy of remark that among 198 cases of periodic squint (convergent and divergent) which are applicable for the statistics of visual acuteness—

170	possess	V. to 1/7.
16	"	V. < 1/7 to V. 1/12.
9	"	V. < 1/12 to V. 1/36.
3	"	V. < 1/36.
14·2 per cent. then of defective vision of considerable degree.

That defective sight on the whole plays an influential part as a cause of squint is doubted by no one, indeed we see blind eyes lapse into squint as soon as the conditions necessary to it are supplied by the muscles. Of all the prevailing causes present defective vision will be the more decisive in proportion as it is of high degree; for the motive which despite the presence of favouring circumstances can prevent the real occurrence of squint, binocular vision, becomes less efficacious as the defective vision becomes more considerable. As binocular fusion takes place frequently in periodic squint, for a time at least, that is as long as proper fixation lasts, one can understand that periodic squint exists chiefly in cases where the visual faculty of both eyes is good. Even the highest degrees of congenital amblyopia are not excluded, for periodic squint appears where the faculty of binocular fusion has been completely lost. Further, that considerable congenital defective sight is more frequent with than without squint, may be accounted for quite simply by the fact that, in extreme degrees of it, binocular fusion cannot be learnt at all, while in the lesser degrees it is more easily forgotten again.

If defective vision is undoubtedly one of the causes of squint, we must seek for the grounds upon which it has been taken to be a consequence of squint, and described as amblyopia ex anopsia. I will not inquire to whom the honour of this invention belongs. I do not want to write a history of mistakes but only to examine the basis of the views now current. The most complete record of the same may be found in the well-known journal on the 'Cure of Eye Diseases,' vol. v, p. 1011. Leber, who does not seem to recognise the existence of congenital amblyopia, has shown quite a special predilection for amblyopia ex anopsia.

Amblyopia from want of use, which formerly included all possible disturbances to vision, great and small, is now only accepted in two cases, for squint and congenital cataract, if the latter is not operated on very early in the first or second year of life.

The fact is simply this, that in congenital cataract even the most successful operation is frequently deceiving as to its issue without ophthalmoscopic report; this is the more disagreeable as the most exact reflection test before the operation fails to prove the existence of this defective sight. But does it follow from this, that congenital cataract has induced defective sight from want of use? We find the same defective vision also in congenital defective development of the transparent lenses (so-called luxation of the lens). On the whole, we often find several congenital defects in the same individual. The very circumstance that the cataract is congenital makes it probable that the defective sight is so also, or are we to take congenital cataract as being a guarantee against congenital amblyopia?

Von Graefe, who first considered this defective sight to be congenital, designated it in his later lectures as originating from want of use, probably in order to advise the earliest possible performance of an operation. There is no mention of his having brought forward evidence for this assertion; that the great master himself said it was enough, and the host of believers felt themselves to be the happy possessors of a new dogma.

A number of children appeared in my practice, in whom congenital cataract was needled by von Graefe in the first or second year of life with recovery of transparent media, who showed, however, the extremest degrees of defective vision when they were sufficiently intelligent to have their vision tested. Whoever is interested in this can find a number of such cases in the Royal Institution for the Blind at Steglitz, which I am accustomed to visit several times a year by request of the committee. On p. 91 I have related a case of monocular congenital defective sight in congenital cataract of both eyes.

Everywhere then the principle holds good, that whoever makes an assertion must be prepared to verify it; amblyopia from non-use is denoted as an inherited trouble, and still not a single observation exists which furnishes proof that an eye of previously ascertained good visual acuteness has become amblyopic in consequence of disuse, a fact I drew attention to ten years ago. Leber replies to this, he remembers "to have seen patients with complete amblyopia in the squinting eye, who stated that its visual faculty had been found to be good during an examination instituted years before." Is this intended as an observation? By that I mean is it a proof of facts, for the trustworthiness of which he holds himself responsible: in the handling of scientific questions I do not place the least reliance on the dim recollections of unnamed individuals. Even in personally conducted examinations we must be on our guard to avoid mistakes, and now we are confronted with mere recollections of tests of vision!

By means of the above observations the theory that "the peculiar variety of monocular amblyopia which is so frequent in monocular squint is hardly observed without squint" is sufficiently disproved.

Leber seeks to enfeeble Alfred Graefe's statement that the presence of extremely defective vision may sometimes be proved at a very early age, in children who have only squinted a short time (the rapid development of amblyopia in consequence of the squint really appears incredible), by the assertion "that just at the earliest age, when the activity of the optic nerve is not yet sufficiently strengthened by use, the conditions for producing amblyopia from non-use are most favorable with complete exclusion of one eye," but complete exclusion of the squinting eye does not take place even in extremely defective sight, as can easily be seen by the mirror test (p. 66) I described fourteen years ago. Which activities of the optic nerve apparatus are strengthened then by use? Perhaps visual acuteness? The physiological conditions of this are only to be sought in the anatomical structure, and the physiological arrangements of the retina or the visual organs, which cannot be changed much by use. What we can learn from the visual act relates solely to the conclusions which we are able to draw from sensual impressions; but visual acuteness, i. e. the faculty for the recognition of distinct points, is an anatomical, physiological gift, and not a thing to be acquired.

The opposing observation, that squint, even of monolateral character dating from earliest childhood, continued to the middle and later years of life, can still exist with very good visual faculty, may easily be explained by alternation from time to time. If that is so indeed, if squint begins during the presence of good visual acuteness, and nothing further is necessary to its maintenance than alternation from time to time, why should defective vision from non-use ever be developed? With good visual faculty on both sides alternations also occur from time to time.

Still more convincing are those cases which are numerous where the visual acuteness of the squinting eye only amounts to about 1/7 to 1/12, and where, on this account, there is no alternation. Were this defective sight acquired through non-use it must of necessity be progressive; it must exist in proportion to the duration of the squint. A moderate experience will suffice to show that this is not the case. And further, defective sight must continue progressive even after removal of the squint by operation, for by the operation nothing is changed in the relations of the binocular vision present in squint, which are dismissed with the one word, "suppression," by the advocates of defective vision from non-use.

Moreover, suppression may exist for years without the slightest disadvantage to the visual faculty.

Case 32.—In November, 1873, I operated on Fritz F— for a slight divergent squint of the left eye. Slight hypermetropia was present on both sides, and nearly full visual acuteness. In October, 1880, perfectly normal position of the eyes showed itself with the same visual acuity and emmetropia in both eyes; at the same time, however, the boy affirmed that when reading he could never see with his left eye but only with the right; in reality only the right visual field was perceived in the stereoscope.

The second reason brought forward is, that the variety of amblyopia from non-use is quite a peculiar one; "it consists of a functional disturbance of those parts of the retina whose images belong to the common V. F., and are suppressed in squint in order to render vision distinct—the macula and the temporal and only a part of the nasal halves of the retina." Does this hold good for all cases of amblyopia in squint, or do those cases only belong to amblyopia from non-use where excentric fixation takes place with an inward deviating visual axis? It would be difficult to draw the line. I have seen a case in which the squinting eye possessed a visual acuteness of 5/36 together with excentric fixation and nystagmus; however, I attach no value to isolated cases. We frequently find excentric fixation with a visual acuteness of 1/12 to 1/36. Further, those cases cannot possibly be regarded as results of squint, which possess unsteady oscillating fixation or rapidly trembling nystagmus, which occurs as soon as the squinting eye fixes. But this conclusion is false, even for the excentric fixation with visual axis deviating inwards; if it were right the angle at which the eye deviates inwards on fixation in convergent squint would always be greater than the squint angle. Those cases are, of course, more remarkable where this is not the case; however, on close investigation those cases are more frequent where the angle of deviation is about the same size or smaller than the squint angle, and is fixed with a part of the retina which undoubtedly belongs to the common visual field.

On p. 91 I have described two cases of excentric fixation in children who had never squinted, and it is only necessary to take a little trouble to repeat the mirror test which I described, to be convinced that squinting eyes have not lost the power "of using those parts of the retina," even if they are amblyopic to an extreme degree; without the slightest doubt the reflection is perceived as soon as it falls on the retina.

Value is attached to the improvement produced by the separate use of the squinting eye. According to my experience no higher visual acuity can be attained by use of the amblyopic eye, than that which is best detected by the aid of eserine in the first examination, if it is only carried out thoroughly enough. No doubt if we proceed otherwise, and rest content with whatever statements the patient likes to make, without giving ourselves any more trouble, we may expect the most superficial diagnoses to show the most astonishing therapeutic results, as, indeed, often happens. And now, talking of strychnine injections! When two celebrated ophthalmologists occupy themselves simultaneously with the therapeutics of strychnine, one of whom obtains the most astonishing results in atrophic troubles of the optic nerves, but, on the other hand, obtains no real improvement in "amblyopia from non-use," while the other can show brilliant success in the last-named form of defective vision, and, on the other hand, none in atrophy of the optic nerves, we may perhaps conclude that both are right, if even really on the negative side, and that the circumstances are the same in the tests of vision. Again, we must examine more closely some of the cases, in which strychnine injections showed a brilliant result. (Anyone interested in the original work can read up the 'Vienna Weekly Medical News' for the year 1873.)

"1. Wilhelm H—, a strong healthy boy, æt. 12, complains of defective vision. Right eye has nothing abnormal in its outward appearance, and just as little in the fundus. V. 16/100, H. 2·5 D., Snellen IV-I/II; is the smallest type he reads at 3 to 7 inches. With + 10, I-I/II is read at 4 to 6 inches. Left eye V. 16/70. H. 2·75 D. II-I smallest type legible at 3 to 12 inches. With + 4 D. I-I/II is read at 4 to 6 inches. On March 14th, 1872, first injection of strychnine with 0·002 gr. in the temples. An hour later V. of right eye 16/70, left unchanged. On March 23rd, 1872, after one injection daily, V. of each eye is 16/50."

Patient shows then in the right eye visual acuity 16/100, with manifest hypermetropia 2·5 D.; in all probability the total hypermetropia really present was higher, and was scarcely corrected by means of convex 4 D. If the patient now reads No. I-I/II Snellen with + 4 D. at 6 inches, this proves a visual acuity of 1/3 during the first investigation before the strychnine injection, and shows that the estimate of 16/100 was inaccurate. At the close of the treatment, only a visual acuity of 16/50 (almost exactly 1/3) is specified for distance. The result seems to me, then, to be this, that the patient during repeated examinations has gradually learned to make more accurate statements, indeed, with a boy twelve years old one can scarcely expect it to be otherwise.

"4. Paul A—, æt. 18, was operated on ten years ago for internal squint of the right eye, and dismissed with + 2 D. for distance, and + 6·6 D. for near use. He now complains of decrease of his visual acuity. The eyes are normal externally and internally. Hyperopic formation in a high degree. Right eye V. 1/20, with and without convex glasses, without glass only VIII-I/II with difficulty, with + 6 V-I/II the smallest. Left eye appears emmetropic, but is decidedly hyperopic. V. 5/4. Glasses are rejected; I-I/II is read fluently at 6 to 12 inches. After one injection the right eye recognises III-I/II with + 6, after the second II-I/II, after the eighteenth I-I/II with difficulty. The visual acuity, however, remains at 1/20, and is not changed after six months, although latterly patient daily practised with + 3 D."

Visual acuity of 1/20 suffices to read III-I/II at 2·5 inches, II-I/II at 1·5 inches, and I-I/II at about 1 inch; clear, retinal images are then scarcely obtainable, but we know what hypermetropes can do in that case; besides this, if the patient is examined for weeks by Snellen's method, he may get so far as to realise fairly well "the strange fate of that man" of I-I/II, despite larger diffusion circles; in any case vision remained at 1/20, despite strychnine and separate use.

In extremely defective vision little importance should be paid to the fact of slight diversity in the statements, as where visual acuity amounts only to about 1/36, or where fingers are counted at a distance of 1 to 2 metres, it is quite immaterial, as far as the usefulness of the eyes is concerned, whether fingers are counted at a half or a whole metre, and we ought never to forget that all conclusions which we draw from the state of the visual acuity, are unreliable in proportion as the latter is lowered. Indeed, on repeated examination of such cases we frequently find considerable fluctuation in the statements of the patients, therefore we ought not to expect accurate statements for very inexact sensual impressions.

By separate use, even in extremely defective vision, no improvement in visual acuteness is developed, but only a more complete acquirement of the power of deducing right conclusions from imperfect sensual impressions. That which has been most unscientifically designated as "suppression of diffusion circles," depends solely on this method of use. As with indistinct retinal images so with facial impressions which are insufficient, one can never learn to recognise larger objects aright.

We must never forget that vision is a conclusive act acquired by practice; whoever sees well with one eye, and is weak sighted with the other, acquires this end only for the sensual impressions of the better eye, and must first collect experience for the defective eye, before he can use it.

Leber has recently joined those cases which are described as blindness through blepharospasm, to amblyopia from disuse. First, I wish to observe that blepharospasm is not a necessary cause; I have seen the same disturbance of vision follow severe double blenorrhœa, which destroyed one eye but left the other uninjured. These children are always of an age which renders any trial of vision impossible, and we are therefore obliged to draw conclusions as to visual power from the movements of the body. If children move as though they were blind, it need not necessarily follow that they are so in the common meaning of the word. The art of vision is a difficult one, the acquisition of which begins with the earliest days of life; we do not call every person blind who does not see what is before his eyes, because he does not understand how to see it. A child who has only imperfectly learnt the conclusive act of vision, and forgotten it again during a continued disuse of both eyes, will not know how to use perfect visual acuity, and will move like a blind person till he again learns to estimate the relations between his retinal images and the things of the material world, which happens in a very short time.

After this digression let us turn again to amblyopia from disuse, and to the last trump which is played for it. "Those cases are very remarkable where an immediate improvement occurs after tenotomy in amblyopia of high degree, which according to this is certainly produced and maintained by the squint." As proof a case is cited by Knapp, who describes it in the following words:—"The improvement in visual power varied very much. In many cases it was indefinable, in others very pronounced; for example, in one case, where it was very great before the operation, only No. 16 Jaeger could be read at 1 inch, while after it No. 2 was read at 8 to 9 inches."

And we are to believe wonders on the strength of this scanty communication! It is an every-day experience that a person who squints, who has just asserted his inability to read the largest type, immediately afterwards reads smaller and the smallest type, and it would at least first have to be determined that all endeavours to produce a better visual result before tenotomy were unsuccessful; but as the communication stands, the conclusion as to the effect of tenotomy is quite a superficial post hoc ergo propter hoc. Moreover, I had this case in view when I spoke on this matter in the first edition of my 'Handbook:'—"The frequently repeated assertion that a considerable improvement of vision may occur as a direct result of tenotomy, is so little in accordance with all the laws of physiology, that inquiries must be instituted ad hoc, and carried out with the most perfect exactitude. Only trials of vision which are carefully carried out and repeated several times before the operation, and which have regard to visual acuteness for distance as well as for near objects, the latter indeed by the aid of convex glasses or Calabar extract, can be recognised as proving anything in face of such a perfectly improbable assertion. In the course of examinations so instituted I have not myself found that tenotomy exercises any direct influence on visual acuity."

I would not have given so much space to this explanation had not a principle been in question. The occurrence of amblyopia as a result of non-use has been deductively constructed and is not inductively proved by observation. It is just an article of faith, and in science we cannot rely on such things; we must not depart from the inductive method.