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.[201]
[Illustration: FIG. 31.]
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,[202] 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 aetiology,
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, Trelat, Hutchinson, Fere, 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.
[Illustration: FIG. 32.]
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[203] (who claims that the eyes were derived from the median
eye of the ascidian lancelet) and Semper[204] (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[205] 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,[206] 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[207] 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.
[Illustration: FIG. 33.]
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 retinae, apparently springing
from a single optic vesicle. The nose was represented by a short process
attached to the forehead, above the median eye.
[Illustration: FIG. 34.]
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[208] 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 Fere,[209] 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,[210] 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.[211]
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[212] 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.
[Illustration: FIG. 35.]
[Illustration: FIG. 36.]
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).
[Illustration: FIG. 37.]
[Illustration: FIG. 38.]
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.[213] 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.
[Illustration: FIG. 39.]
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.[214]
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 deg. (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.
[Illustration: FIG. 40.]
[Illustration: FIG. 41.]
[Illustration: FIG. 42.]
[Illustration: FIG. 43. EAR ALMOST HORIZONTAL AND AT RIGHT ANGLES WITH THE
HEAD.]
[Illustration: FIG. 44. EAR ALMOST ROUND WITH THREE DARWINIAN TUBERCLES AT
INNER BORDER.]
[Illustration: FIG. 45.]
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 deg., or be less than 15 deg. 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,[215]
from the examination of several hundred subjects, concludes that the
auricle-temporal angle undergoes a gradual progress from below 90 deg. in
criminals and the insane to above 90 deg. 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 deg.; 152 at an angle of 45 deg., and 115 at right
angles or 90 deg. Of 1,041 criminals at Elmira 285 were close, 567 at an
angle of 45 deg., and 187 at right angles.
[Illustration: FIG. 46.]
[Illustration: FIG. 47.]
[Illustration: FIG. 48.]
[Illustration: FIG. 49.]
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.