CHAPTER XII. Degeneracy of the Lip, Palate, Eye, and Ear

DEGENERACY OF THE LIP, PALATE, EYE, AND EAR

Each part or sense organ may, independently of the face as a whole, exhibit signs of degeneracy. The palate, lip, nose, eye, and ear have their own expressions of degeneracy. The palate, so far as the joining of its two parts is concerned, resists degeneracy to a remarkable degree. The cleft, which results from non-union, is usually an expression of general degeneracy acted on by its nutritive expression, albeit cleft-palate may be associated with the graver degeneracies like idiocy.

As the subjects of the deeper degeneracies associated with cleft-palate usually perish in early infancy, the nutritive degeneracies are most frequently found associated with cleft-palate in adult life. The mouth in the vertebrates does not agree in character with the invertebrate mouth. The mouth has grown in proportion to evolution in the lower vertebrates. It is larger, however, in proportion in the human embryo than in the adult. In this it agrees with the general lower type of the human face. The human chin is at first retreating, and does not become distinctly prominent until the fifth month of foetal life. The nose separates from the mouth toward the end of the second month. It is at first short and broad and resembles at three months the type of some of the lower negro races. When the nose is separated from the mouth a partition forms between the cavities of the nose and the mouth, later supplemented by the true palate, which divides the mouth into an upper respiratory passage and a lower digestive passage. This palate is partly composed of bone and partly of flesh. The fleshy part ends in what is called the uvula. This is very subject to abnormalities, as has been pointed out by C. L. Dana; it is a very frequent mark of degeneracy. Dickens noticed the uvular tone of voice in young thieves, due to deformity of the uvula.

Cleft palates are comparatively rare in proportion to other forms of nutritive degeneracy. Palatal embryology casts light on the causation. At a very early period of foetal life a series of clefts appear on each side of the cephalic extremity, separated by rods of tissue called branchial arches. The clefts communicate with the alimentary canal. These various clefts have usually coalesced about the ninth and tenth week of foetal life, but occasionally this coalescence fails or is incomplete. This leads to various deformities, the chief of which are cleft palate and hare-lip. Cleft palate has been known to affect several members of the same family, and to occur in the offspring of the affected members. There are instances of the transmission of this deformity from an affected pug-bitch to her offspring. If it were possible to practise selective breeding in man as in dogs a race of men with hare-lips and cleft palates could probably be produced.

Cleft palate may be divided into two classes, congenital and acquired, acquired cleft palate being the result of disease either inherited or acquired, but only affecting the part after birth. Congenital cleft palate is divisible into two kinds, complete and partial; complete when the fissure extends the entire length, from the uvula to and including the anterior alveolar process and even the lips (Fig. 31), partial when only a small part of the structure is involved. Thus the cleft may extend through the anterior alveolar process, involving the incisive bone only, which is very rare; when present single or double hare-lip almost invariably coexists. I have observed in practice six cases where a small portion of the interior alveolar process was involved, with the jaw and one or two teeth. The hard palate only may be involved to the extent of a small fissure, or the whole palate may be wanting. The soft palate only may contain the cleft, or simply the uvula. Cases are on record in which the non-development of the intermaxillary bones produces fissures in the lip. A priori, cleft palate is an evident expression of hereditary defect. Langdon Down found a constant relation between brain deformity, cleft palate, and deformed vaults.

In a case cited elsewhere, three members of one family had cleft palate; one 17 years old, the other 30, and the third 35. The first and last are women, the other a married man with a family, who have no trace of the father's deformity. In these cases no instance of cleft palate could be found either among the ancestors or the collateral branches of the family.

Another family has a remarkable history. G. H. C., born 1853, perfect; L. C., born 1855, single hare-lip and cleft palate; J. F. C., born 1856, perfect; F. W. C., born 1860, double hare-lip and cleft palate; H. E. C., born 1868, perfect. The paternal grandmother also had cleft palate.

Knecht found that 5 per cent. of 1,200 criminals examined had cleft palate. Fourteen per cent. of the prostitutes examined by Pauline Tarnowsky had cleft palates. Langdon Down, among congenital idiots, found only a half per cent. of cleft palates. Grenzer found only nine cases in 14,466 children, or one in 1,607. I examined 1,977 feeble-minded children without finding a single case. In 207 blind only one case was observed. In 1,235 deaf mutes two cases were found. The percentage among the defective classes, while not large, is, undoubtedly, much larger than among normal individuals.

A keeper of the Zoological Gardens in Philadelphia observed cleft palate in the mouths of lion cubs born in the gardens. Cleft palates were also observed in a number of pups born in Buffalo. Dr. Ogle found that 99 per cent. of the lion cubs born in the London Zoological Gardens had cleft palates. He claims that this is due to the artificial diet, as the result of enforced captivity. Similar results observed in other gardens in Europe are charged to feeding the mothers with meat without bone, as feeding with the whole carcass of small animals greatly diminished these deformities. It would seem that if cleft palate is due to this cause other bony structures should also be involved, and, as a matter of fact, many of the lions born in captivity are rickety. Cleft palate has been observed among dogs, sheep, goats, &c. The question, therefore, whether domesticity here does not play the alleged part of civilisation in man can be solved only by a knowledge of the frequency of the condition among wild animals of the same genus. It is evident that, in dealing with the question of ætiology, the influence of shock on the mother's nervous system cannot be excluded in the cases charged to feeding.

It should be remembered that cleft palate is a factor which predisposes to death by infectious diseases whose local manifestations are in the mouth and throat; hence the majority of the degenerates in whom cleft palate occurs are liable to die before the completion of their fifth year. Hare-lip (Fig. 32) is an exceedingly hereditary disorder, as Murray, Demarquay, Trélat, Hutchinson, Féré, Marsh, Lucas, Ireland, and others have shown. It occurs with great frequency in almost all forms of degeneracy. Kiernan, of Chicago, has found that 5 per cent. of the hereditary lunatics in that city and in New York City have hare-lip.

How far degeneracy may turn back the page of evolution is excellently illustrated in the cyclopian descendants of degenerate families elsewhere cited from Kiernan, of Chicago. While there is a seeming conflict as to the primitive eye type of vertebrates between morphologists represented by Howard Ayres (who claims that the eyes were derived from the median eye of the ascidian lancelet) and Semper (who is of the opinion that the existing vertebrate eyes represent the paired eyes of a hypothetical annelid precursor); still both opinions are fully reconcilable through the results of study of the ascidian and lancelet eye collated with cyclopian and triophthalmic (three-eyed) degeneracies in man, the human eye and the third eye of reptiles like the hatteria of New Zealand. The eye of the ascidian tadpole agrees fundamentally with the type of eye peculiar to the vertebrates in that the retina is derived from the wall of the brain. On this account it is called a myolonic eye. In the typical invertebrate eye, on the contrary, the retinal cells are differentiated from the external ectoderm.

The ascidian eye, however, differs, as Osborn remarks, essentially from the paired eyes of the craniate (skulled) vertebrates in that the lens as well as the retina is derived from the wall of the brain. The lens of the lateral eye of the vertebrates is derived by an invagination of the ectoderm, which meets and fits in the retinal cup at the end of the optic vesicle. The ascidian eye, however, agrees in respect to the origin of its lens with the parietal or pineal eye of the lizard, in which the lens is likewise derived from cells which form part of the wall of the cerebral outgrowth which gives rise to the pineal body.

The pineal body is another of those remarkable rudimentary structures whose constant presence in all groups of vertebrates forms such an eminently characteristic median outgrowth from the dorsal wall of the brain (thalamencephalon), the distal extremity of which dilates into a vesicle and becomes separated from the proximal portion. The distal vesicle becomes entirely constricted off from the primary epiphysial (pineal) outgrowth of the brain, and the parietal nerve does not represent the primitive connection of the pineal eye with the roof of the brain, but arises quite independently of the proximal portion of the epiphysis.

The remote ancestors of the vertebrates possessed a median, unpaired, myolonic eye, which was subsequently replaced in function by the evolution of the paired eyes. The cyclopic condition occurs very frequently among human monstrosities, much more frequently than among animals, Hannover claims, but this is clearly due to the fact that human monstrosities are much more frequently recorded. Of the 120 cases I have been able to collect from literature, 56 presented other evidences of degeneracy than cyclopic conditions, and 60 had neuropathic or other taint in the ancestry. Dareste has shown that the production of a single eye, the changes in the structure of the mouth, the atrophy and abnormal situation of the olfactory apparatus and of the vesicle of the hemispheres, all result from an arrest of development. The determining influence must be exerted very early in the life history of the embryo.

Hannover points out that there often is coincident hydrocephalus and harelip, imperfect genital development, and allied arrest of development. J. R. Folsom, of Cecil, Georgia, has reported a female, born alive to a negro multipara, who died two hours after birth. The eye was centrally located in the forehead, on a line with the nose. The brow was a complete arch, as was the upper eyelid. The lower lid had a mark midway, indicating an attempt at division. The nasal bones were wanting, but the soft part of the nose, destitute of the orifice, hung over the mouth, which was completely covered. The chin was recedent. C. Phisalix described a case in which the nose was wanting. Its place in the median was occupied by a single eye; on the horizontal diameter were two pupils separated by a narrow space. Landolt, discussing a case reported by Valude, points out that in cyclopic eyes all the parts may be double or united in every degree except that there is never a single lens or double vitreous. Bock and others, however, describe cases in which the eye has not been formed by the conglomeration of two separately developed eyes, but it is a single developed eye; the other being wanting entirely. Bruce reports a cyclop in which there was a single socket for the eye of a lozenge-shape, situated in the lower middle of the forehead. The socket was furnished with two pairs of eyelids, upper and lower. The eye was found to consist of two rudimentary eyes, with two rudimentary retinæ, apparently springing from a single optic vesicle. The nose was represented by a short process attached to the forehead, above the median eye.

The cyclops illustrated (Figs. 33, 34) was born to a 17-year old neuropathic primipara, after a protracted labour. The child was living, but was killed by pressure on the funis. The mouth contained an ivory, tusk-like tooth at each corner. There was mane-like hair around the neck.

Cyclopia is very frequently associated with the absence of both the internal and external ear, and with synotia (joined ears).

In triophthalmic cases the three eyes are usually separate, two occupying the usual position, while the third is situated as illustrated in the case cited. Ninety families of degenerates, averaging eleven children each, had five cases of cyclops.

Degeneracy, which affects so deeply the development of the eye, naturally tends to evince itself in other anomalous states in the organ. As excessive asymmetry of the body is one of the most noticeable of the stigmata of degeneracy, it is not astonishing to find that this asymmetry expresses itself both in the position as well as in the size and structure of the eye. As Kiernan pointed out, twenty years ago, asymmetrical irides are exceedingly frequent in the types of insanity due to hereditary defect. This observation has since been confirmed by Féré, not only as to the insane, but as to other classes of the degenerate. The conditions of the eye known as microphthalmia (small eye), macrophthalmia (big eyes) and anophthalmia (absence of eyes), are found quite frequent in degenerate families. Very frequently the pupil of the eye is asymmetrical. This was pointed out by Kiernan in the case of Guiteau. Corectopia (displacement of the pupil so that it is not in the centre of the iris) often exists. Coloboma (eye fissure) is also not infrequent among the degenerate. These vary greatly in situation and general results. The iris is sometimes completely absent on one or both sides. Beside the anomalies, diseased conditions like retinitis pigmentosa, congenital cataract and the macular degeneracy cited by C. P. Pinckard, of Chicago, are far from infrequent expressions of degenerate taint in the eye; the organ in this particular obeying the general law that degeneracy may show itself in the minute change resulting in disturbance of function or in that producing disease, or, finally, in atavism. The defects of the eyes requiring glasses are exceedingly frequent in degenerates, and often aggravate their morbidity. Here, as in the case of the teeth, the chief factor is often ignored.

The external ear is, of all organs, that most affected by degeneracy. It is a cartilaginous organ extending from a bony base, without a bony framework for its support and with very deficient blood supply, on account of its distances from the great blood centres, so that any defect in the nerve centres which control the local blood supply is likely to affect its nutrition. As a cartilaginous organ it has no lymphatics, which of necessity affects its growth. The sensitiveness of the ear to vasomotor changes is evident by the results of the extremes in heat and cold, emotional blushing and fatigue. Galton reports a schoolmistress who judges the fatigue of her pupils by the condition of their ears. If the ears be white, flabby and pendant, she concludes that the children are much fatigued. If they be relaxed, but red, they are suffering, not from overwork, but from a struggle with the nervous system rarely under control in children. These states are very common among degenerates.

To appreciate the degeneracy observed in the ear its embryology requires study. Before the end of the first month there appears around the external opening of the first gill-cleft (Fig. 35) a series of six tubercles, two in front on the hind edge of the first visceral arch, one above the cleft and three behind it (Fig. 36). A little later a vertical furrow appears down the middle of the hyoid arch, in such a way as to mark off a little ridge which joins on to tubercle 3 and descends behind tubercles 4 and 5. The second stage is reached by the growth of all the parts; the fusion of tubercles 2 and 3 and the growth of the ridge down behind tubercle 5 to become continuous with 6. After these changes it is not difficult to identify the parts.

Tubercle No. 1 is the tragus; 2 and 3, together with the arching ridge, represent the helix; 4, the anti-helix; 5, the anti-tragus; and 6, the lobule; the pit between the tubercles the fossa angularis. During the latter part of the second month the ear changes in its proportion somewhat in the irregular development. The third stage begins at the third month. The upper and posterior part of the concha arises from the surface of the head, and gradually but rapidly bends forward so as to completely cover the anti-helix and the upper portion of the fossa angularis. During this stage in mammals the assumption of the pointed form of the ear commences. The fourth stage begins at the fourth month, when the tubercles, which are now joined together by cartilages, commence to unfold and are completed by the fifth month. Finally, the sixth tubercle develops to form the lobule. This unfolding or development of the tubercles to produce the different portions of the ear and make it complete is not unlike the development of a flower from the bud. By this process may be understood how if, by malnutrition in one tubercle or bud, or should there be a larger supply of nutriment in one than another, malformation of the ear would result. If arrest of development of all the tubercles should take place at any period, from the first to the fifth month of foetal life, the ear would resemble a semi-developed flower! (Fig. 37).

As in other cases, it is necessary to fix an approximately normal standard for the ear from the standpoint of man's status in evolution. The ear grows more or less through life, but, like the skeleton, practically reaches its full development about the twenty-sixth year. That this is not always the case, however, is demonstrated by the results of the examination of 546 persons. In an examination of 63 children between 6 and 18 months old the ears measured from 1·60 to 2·12, the average being 1·90 inches; in width from ·75 to 1, with an average of ·96. In 127 children from 8 to 12 years old the ear measured from 1·95 to 2·32 inches, the average being 2·19; width ·81 to 1·50, the average being 1·06. In 356 persons between 12 and 50 years old the shortest ear was 2, the longest 3, the average 2·50; width, smallest 1, largest 1·50, the average being 1·22 inches. The normal ear, or rather the ideal one (for few persons possess it in its entirety), should have a gracefully curved outline, be nowhere pointed or irregular, have a well-defined helix, separated from the anti-helix by a distinct scaphoid fossa extending down nearly to the level of the anti-tragus (Fig. 38). Its root should be lost in the concha before reaching the anti-helix. The anti-helix should not be unduly prominent, and should have a well-marked bifurcation at its superior extremity. The lobule should be shapely, not adherent, not too pendulous and free from grooves extending from the scaphoid fossa. The whole should be well shaped, and its proper proportion and size may be inferred from the table just given; in the adult it should not average over two and a half inches in length and one and a quarter in breadth.

The aural deformities that fall under the head of stigmata, or have been classed as such, affect all portions of the external ear. The helix may be imperfect, it may be angular, from Darwin's tubercle it may lack its inward roll, it may be interrupted, the root of the helix may extend inward completely across the concha, and in very rare instances it may be bifurcated. The anti-helix may be unduly prominent or be insignificant; the scaphoid fossa may extend through the lobule or be triple. The lobule may be adherent and sometimes almost absent, thus producing the jug-handle-shaped, or so-called Morel ear. It may be exaggerated in size; the whole ear may be misshapen, too large or too small. These deformities may exist in nearly every degree, only when pronounced can they be considered as stigmata. Others have been noted, but their importance as signs of degeneracy is not very significant unless they co-exist with several of those above mentioned.

The ears of degenerates frequently grow in later life to an enormous size. On examination of 207 paupers over 50 years old the shortest ear was found to be 2·25 inches, the longest 3·36; the average on the right side was 2·73, on the left 2·76; the narrowest ear was ·88, the widest 1·50, the average 1·26. These results, compared with the results of the measurements of normal persons from 12 to 50 years, plainly demonstrate that the ears of degenerates grow after the twenty-sixth year, when the skeleton has completed its development. With such large ears other stigmata are generally associated. A few of the best types of stigmata of the ear illustrate better than any description the general characteristics. Fig. 39 illustrates the ear par excellence of degeneracy, the typical jug-handled ear first described by Morel and called by his name. This consists of a long, narrow ear attached its entire length, and tapering upward and outward from the lobe to the point where the Darwinian tubercle is located, and there it may take any shape--round, straight, or pointed--as illustrated in the drawing. The most singular deformity of the helix is the tubercle of Darwin, which is a little blunt point, projecting from the inwardly folded margin or helix. When present it is developed at birth and, according to Ludwig Meyer, more frequent in men than in women. Fig. 40 shows ear and tubercle taken from Darwin. These points not only project in toward the centre of the ear, but open a little outwards (Fig. 41) from its plane, so as to be visible when the head is viewed from directly in front and behind. They are variable in size, number and somewhat in position (Fig. 42), standing either a little higher or lower, and they sometimes occur on one ear and not on the other. Another marked form of ear degeneracy is one in which the ear is developed backward at an angle of about 45° (Fig. 43). The general outline of the ear is fairly good. The anti-helix is much larger than it should be. Degeneracy usually extends deeply into the organisation of those in whom this ear is present.

Fig. 44 illustrates noticeable stigmata. The ear stands at right angles with the head. It is, however, almost as broad as it is long and differs in shape. The outer helix is excessively developed. The scaphoid fossa extends through the lobe, which is continuous with the body of the ear and is not distinct. The root of the helix is excessively developed. There are three Darwinian tubercles on its border. The anti-tragus is undeveloped. The tragus is very small and divided into two parts. The auricle-temporal angle, for functional purposes, should not exceed, according to Buchanan, 30°, or be less than 15°. The difference in this respect is not clear, since free movement of head and body will readily adjust the auricle to receive sound. For functional purposes it would seem that the construction and shape of the ear, especially the anti-helix and concha, would be of greater importance than its position to the head, since it is necessary to collect the waves of sound for transmission. From a degeneracy standpoint, however, the position of the ear is important. Frigerio, from the examination of several hundred subjects, concludes that the auricle-temporal angle undergoes a gradual progress from below 90° in criminals and the insane to above 90° in apes. He found the large angle very marked in homicides, less so in thieves. There is no question but that Frigerio is correct in regard to this point. In an examination of the ears of 465 boy criminals at Pontiac, it was found that 198 were close to the head, or from 10 to 15°; 152 at an angle of 45°, and 115 at right angles or 90°. Of 1,041 criminals at Elmira 285 were close, 567 at an angle of 45°, and 187 at right angles.

Fig. 45 shows the ear which Hawthorne gives Donatello in the Marble Faun; it has been called the "Satanic" ear, because of the pointed extremities and its narrowness. The helix is rolled upon itself its entire length, giving the impression of great thickness. The anti-helix is excessively developed. Figs. 46, 47, 48 (ears of three illegitimate children at birth) show different stages of development as well as marked stigmata. Arrest of development occurs between the fourth and fifth month, owing to the trophic nerve centres being affected by the malnutrition of the mother. Fig. 49 exhibits the development of a congenital, elephantine ear in monstrosities. The resemblance to the large ear of the orang and chimpanzee is very marked.