Branchial Cleft Anomalies: A Review of 87 Cases Treated at the Toronto General Hospital

The embryology, anatomy and pathology of branchial cleft anomalies are discussed and 87 cases reviewed.The most frequent anomaly was branchial cleft cyst, of which there were 77 cases. Treatment in all cases consisted of complete excision.There were five cases of external branchial sinus and five cases of complete branchial fistula. Sinograms were helpful in demonstrating these lesions. Excision presented little difficulty.No proved case of branchiogenic carcinoma has been found in the Toronto General Hospital. Five cases are described in which the original diagnosis was branchiogenic carcinoma—in four of these a primary tumour has already been found. The authors believe that the diagnosis of branchiogenic carcinoma should never be accepted until repeated examinations over a period of at least five years have failed to reveal a primary tumour.     

压力波对听觉器官的致伤作用——Ⅰ.致伤部位和损伤特性

本工作用二十余种武器压力波源在不同暴露条件下对近二千只豚鼠进行了系统的致伤实验。结果表明,压力波暴露后致伤部位主要在中耳和内耳;即使压力峰值已高达190dBSPL,听觉中枢还未见有损伤迹象。中耳损伤和内耳损伤的程度都与压力波的强度有关,但两者并不平行,压力峰值大时压力波可以只损伤或主要损伤中耳,压力峰值不很大但重复发数较多或脉宽较大时,则可能只损伤或主要损伤内耳。在一定的条件下,中耳的损伤能缓冲压力波对内耳的冲击,从而减轻内耳的损伤。在中耳损伤或中耳、内耳混合损伤时,豚鼠的听力丧失并没有象在人的噪声性耳聋时(内耳损伤为主)常见的那种突出的高频选择性。     

Craniofacial changes in unilateral microtia: II. An X-ray study

Roentgenocephalometry was used for studies into the extent and character of craniofacial changes in 45 adult males with unilateral (right-sided) microtia. Out of the whole complex of changes associated with this malformation the mandibular ramus showed the most marked involvement and represented the main cause of the accompanying deviations and asymmetries. On the average, the affected half of the face was compressed toward the level of the external auditory meatus both from above and below, but there was a marked variability in individual patients examined. No signs of asymmetry were disclosed in one third of the patients while severe asymmetry was present in one fifth of the patients. Facial hemihypoplasia exerted no substantial influence on the facial profile (when no retrusion of the lower jaw was present) on the sagittal maxillomandibular relations or on the occlusion of incisors, while in transverse direction a laterosuperior deviation of the mandible towards the affected side was clearly visible. A branchiogenic malformation affected the neighboring structures, the cranial base (a more marked curving), frontonasal segment (septum and premaxillar deviation), and the neurocranium (posterior rotation of the cranial vault). The inner ear structures (semicircular canals) were affected only rarely (in 4% of patients). These findings complemented the results obtained in the first part of our study and confirmed the complex character of this inborn anomaly.     

Levator veli palatini muscle and eustachian tube function

Thirty previously unoperated patients with submucous cleft palate, occult submucous cleft palate, and unilateral congenital paralysis of the levator veli palatini muscle were examined. All patients were subjected to a comprehensive otoscopic, endoscopic, audiologic, and tympanometric evaluation. A correlation was made between levator veli palatini muscle anomalies, eustachian tube orifice anomalies, and middle ear ventilation and disorders. Normal middle ear ventilation was found in 23 patients. Negative middle ear pressure that consequently normalized following treatment of coexisting sinusitis was found in 3 patients. Only in 4 patients was chronic middle ear disease found. In one of them, middle ear effusion disappeared following successful treatment of sinusitis. Our conclusion is that the levator veli palatini muscle has no significant function in the opening mechanism of the eustachian tube and must be considered as a velopharyngeal valve muscle only.     

Craniofacial changes in hemifacial microsomia

Cephalometry, X-ray cephalometry, and somatoscopy were used in the studies of 65 adult males with a severe unilateral microtia subdivided into three groups: with marked asymmetry, with slight asymmetry, and without any obvious facial asymmetry. The group with marked asymmetries was designated as hemifacial microsomia. In this group the affected side of the face was depressed on the average from above and from below towards the level of the external auditory canal. The center of the anteroposterior reduction was situated in the region in front of the pterygomaxillar fissure. The anteroposterior and vertical facial dimensions on the affected side were reduced most markedly, while the width dimensions showed the slightest changes. Hypoplasia was most severe within the lower face and increased towards the otocephalic centre. The mandibular joint was displaced in an anteroinferior and medial direction. Hypertelorism did not occur, but the orbit on the affected side was smaller in height and was frequently vertically dislocated. The facial profile was unchanged except for retrusion of the chin and increased frequency of bite disorders. The mobility of the mandible was limited. Hemihypoplasia also exerted an influence on structures that were not of branchiogenic origin, e.g., the cranial base (narrowing, asymmetry, and more pronounced curvature), the neurocranium (depression in mastoid and tympanotemporal regions, posterior rotation of the vault), and the frontonasal component (deviation of the nose and premaxilla). The cranial vault and the bottom of the occipital bone showed on the average no asymmetries. The similar character of deviations in slightly affected groups revealed that in spite of the high variability of changes typical for branchiogenic malformations the development of the face in these defects was subjected to certain rules. Marked facial asymmetry occurred only in every fifth patient with a severe degree of microtia, while definite signs of asymmetry were absent in every third patient.     

Endotemporal branching of the facial nerve

Out of 400 specimens of temporal bone the authors found two cases of branching of the facial nerve in the mastoid course, i.e., 0.5%. Knowledge of this anomaly has a certain practical value in surgical intervention of the middle ear.     

Symphalangismus,Strabismus und Mittelohrmißbildung

Zusammenfassung Es wird über die Kombination von Symphalangismus und Mittelohrmißbildung innerhalb einer Familie berichtet. Für die Skeletfehlentwicklung und für die Mittelohrmißbildung wird der gleiche Gendefekt verantwortlich gemacht. Der kongenitale Strabismus bei den Geschwistern kann nicht sicher zu den anderen beiden Mißbildungen in Beziehung gebracht werden. Der genetische Zusammenhang einer erstmals bei Symphalangismus beschriebenen Spina bifida mit den Finger- und Zehenmißbildungen wird abgelehnt.

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Symphalangism, strabism, and anomaly of the middle ear

Summary This report describes a combination of symphalangism and anomaly of the middle ear in a family. The same genetic defect is thought to be responsible for both the deformation of the skeleton and the anomaly of the middle ear. The authors are not sure whether there is any connection between the congenital strabismus and the two other anomalies of brother and sister. The assumption of a genetic connection between spina bifida, which was described for the first time with symphalangism, and the anomalies of the fingers and toes is rejected.

     

Hemifacial microsomia: use of the OMENS-Plus classification at the Royal Children's Hospital of Melbourne

Hemifacial microsomia is the most common facial congenital disability after cleft lip and palate, but as yet its pathogenesis remains unknown. Clinical classification systems have evolved over the last 30 years from those classifying only single components of the disorder, to those classifying according to the combination of deformities, to the most recent systems that grade each anatomical component separately, such as the Orbit, Mandible, Ear, Nerve, and Soft tissue (OMENS) system. The aim of the present study was to review the classification of patients with hemifacial microsomia treated by the Melbourne Craniofacial Unit at the Royal Children's Hospital using the OMENS-Plus system of classification and to correlate the findings with data from other centers. Records of patients treated by the craniofacial unit were reviewed and included in the study if adequate clinical records, photographs, and radiographs (anteroposterior, lateral, basal cephalometry, panoramic views) were available. The data were entered into a database file developed for this purpose. Seventy-one patients were identified from the hospital database, of which six were excluded because of incomplete data. Of the 65 patients, there were 31 (48 percent) with right-sided microsomia, 25 (38 percent) with left-sided microsomia, and nine (14 percent) with bilateral microsomia, with an overall male-to-female ratio of 1.2:1. The majority of patients had a normal orbit (77 percent), mildly hypoplastic mandibular ramus-condyle with functioning temporomandibular joint (57 percent with type M1 or M2a), normal facial nerve (76 percent), and mild soft-tissue hypoplasia (73 percent). There was a similar proportion of patients with mild ear anomalies (53 percent with grade 0 or 1) compared with those with more severe anomalies (47 percent with grade 2 or 3). Correlative analysis demonstrated a slight but positive correlation between the severity gradings of the five individual components. The correlation was lowest between the grading of the nerve and ear and that of the mandible and nerve. The data demonstrate the phenotypic variability of hemifacial microsomia and suggest a degree of relationship among the components of hemifacial microsomia. The OMENS-Plus system has provided a major advancement in the classification of hemifacial microsomia. The authors suggest refinements to the grading of the orbit and nerve components.     

Unilateral duplication of the cerebellar hemisphere and internal, middle, and external ear: a clinical case study

This article presents the unique congenital anomaly complex of ipsilateral cerebellar hemisphere duplication and internal, middle, and external ear duplication. Diagnostic techniques included a head CT scan, a three-dimensional head CT scan, and a head MRI. An aberrant notochordal split was proposed as the embryologic mechanism leading to the development of such anomalies.     

Multiple correlations between craniofacial characteristics: an x-ray study

The method of multiple correlations was used to assess the interrelations between basic characteristics of the facial bony framework and the cranial base. The study was based on x-ray measurements in 50 normal adult males, and the result disclosed the extent to which the variability of the investigated characteristics was determined by the variability of several combined facial parameters. The characteristics of shape and position of individual facial structures were more closely interrelated than characteristics of size. The lowest degree of association was shown by the parameters of the cranial base, which confirmed its independent development. The highest degree of interrelations was shown by the parameters of shape and position of the lower jaw, which characterized the marked adaptation and compensation capacity of the mandible. Some of these relations were causal. The present findings could be useful in orthodontic therapy and during anthropologic reconstructions.     

The lizard ear: Gekkonidae

The gecko ear was studied in 36 species belonging to 24 genera. This receptor has attained an advanced level of structure and performance in this group of lizards, but there are many variations among species. To a large extent these variations follow subfamily lines as represented in Kluge's system of classification. Brief consideration is given to features of the outer and middle ear, but chief concern is with inner ear structures and their relations to auditory sensitivity as represented by the cochlear potentials. The auditory papilla is segmented, with a dorsal portion whose hair cells have their ciliary tufts attached to a tectorial membrane, and a ventral portion in which these cells form tow assemblages, one with tectorial connections and the other with connections to a line of sallets. The dorsal segment varies greatly in length and in the form of ciliary orientation. In Eublepharinae and most Gekkoninae the ciliary orientation is unidirectional, and the degree of sensitivity relates to the length of this segment. In Diplodactylinae and Sphaerodactylinae the orientation is bidirectional, and this segment functionally hardly differs from the ventral segment. Auditory sensitivity as measured in terms of the cochlear potentials shows close relations with subfamily groupings, except for the Gekkoninae in which considerable diversity is found. The evidence from structural differentiation, along with that derived from the forms of the cochlear potential functions, leads to the suggestion that these ears possess a high degree of pitch discrimination and capability for the analysis of complex sounds.     

Recognition of emotions by facial and verbal samples in healthy humans and patients with local brain pathology

The efficiency of emotion recognition by verbal and facial samples was tested in 81 persons (25 healthy subjects and 56 patients with focal pathology of premotor and temporal areas of brain hemispheres). The involvement of some cortical structures in the recognition of the basic emotional states (joy, anger, grief, and fear) and the neutral state was compared. It was shown that the damage to both right and left hemispheres impaired the recognition of emotional states by not only facial but also verbal samples. Damage to the right premotor area and to the left temporal area impaired the efficiency of the emotion recognition by both kinds of samples to the highest degree.     

Experiences with development of morphological typology of the face

On base of the component analysis of facial dimensions the original diagnostic model of definition of a morphological facial type in Russian males and females is developed. The first typological characteristic feature describes the variability of general face size, allocating extreme micro- and macrotypes. The second one describes the face shape as a ratio of frontal and mandibular parts. On poles of variability it demonstrates a type with massive frontal face part and rather reduced mandibular one, and a contrast type with a massive mandibular part at relative gracile frontal face part. Central position in this model is occupied by variants with equally proportional development of the top and bottom face parts. On the basis of seven traditional anthropometric facial dimensions the place of any individual in terms of face size and form can be easily found in the suggested model. The chosen contrast variants of the face form are comparable to the appropriate characteristics of cerebral and digestive Sigaud types. The offered morphotypology allows to estimate accurately individual and group features in the face form. The methods of multivariate analysis reveal steady connection between somatotypes and features of a facial structure. Though the level of correlation does not exceed 0.3-0.4 of correlation coefficients, it is statistically significant at the highest degree of probability. The basic direction of habitus variability in men is observed at general face size, in women at face shape.     

Preliminary observations on isotretinoin-induced ear malformations and pattern formation of the external ear.

Retinoic acid is a morphogenic substance capable of inducing a variety of limb malformations, including duplications and reduction-type defects. Whether retinoic acid plays a similar role in controlling pattern formation of other vertebrate structures is unclear. Many fetuses and infants exposed to isotretinoin (13-cis-retinoic acid) in utero have a characteristic pattern of anomalies, chiefly involving brain, craniofacial, and thymic morphogenesis. Among the craniofacial anomalies, external ear malformations are common and the specific types of auricular malformations include partial duplications, and tissue reductions and displacements. These similarities to the types of limb malformations that retinoic acid can induce suggest that retinoic acid may play an important role in controlling pattern formation of facial structures.     

Midline cervical cleft

Midline cervical cleft is a rare congenital anomaly of the ventral neck. A series of 12 cases of midline cervical clefts over a 30-year period is reported. This anomaly is part of a spectrum of midline branchiogenic syndromes resulting from abnormal migration of cells derived from the branchial arches. The preferred operative correction requires complete excision of the cleft with its underlying fibrous cord and closure with multiple Z-plasties.     

Detection of Alloiococcus otitidis in the nasopharynx and in the outer ear canal

Alloiococcus otitidis has been recovered from the middle ear of children with otitis media with effusion, but its natural habitat is not known. To determine whether the nasopharynx and the outer ear canals are the natural habitats of A. otitidis, 145 swabs (50, nasopharynx; 95 outer ear canal) collected from 50 children were screened by polymerase chain reaction. A. otitidis DNA was detected in seven (4.8%) of the 145 specimens, of which four were nasopharynx, and three outer ear canal. These results indicate that the nasopharynx and outer ear canal may be the body sites for localization of A. otitidis.     

Abnormal distribution of the periaxonemal structures in a human sperm flagellar dyskinesia

The spermatozoa from four infertile patients showing a flagellar dyskinesia due to abnormal flagellar wave development have been studied by light and transmission electron microscopy (TEM) for flagellar morphology. No axonemal anomalies were found but modification of the periaxonemal structures was observed. The results of a stereological analysis revealed abnormal extension of the individual dense fibres along the axoneme in the four cases as compared with a control group. The order of termination of those structures was therefore altered. However, the overall fibre extension was the same in both groups (ie, 60% of the principal piece). The number and the location of the longitudinal columns were also modified, the predominant anomaly being the presence of a single column. The possible influence of those structural anomalies on the pattern of sperm movement is discussed. Our observations seem to agree with a previous hypothesis of the literature, that the dense fibres might play a role in flagellar flexibility. More particularly, we suggest that the abnormal extension of dense fibres No. 2, 3, and 4 and the symmetric distribution of the dense fibres on both sides of the plane of beating may alter the flagellar curvature amplitude and the cell rotation frequency.     

长足大竹象虫口密度与虫孔数、竹笋受害率的关系

2007和2008年8月上旬,通过野外调查研究了四川长足大竹象虫口密度、虫孔数、竹笋受害率之间的相关关系.结果表明：研究区长足大竹象平均虫口密度与平均虫孔数、平均虫口密度与竹笋受害率、竹笋受害率与平均虫孔数3对变量用一元三次函数拟合的效果最佳,其相关系数均达到极显著水平.根据最优拟合方程建立了虫口密度、竹笋受害率的预测表,得出长足大竹象的防治指标为虫口密度0.13 头·笋^-1、虫孔数0.40 个·笋^-1.