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The oculo-auricular phenomenon. Findings in normals and patients with brainstem lesions.

The oculo-auricular phenomenon consists of bilateral coactivation of external ear muscles during lateral gaze. The electromyogram of the transverse auricular muscle was recorded in 25 healthy volunteers and 1186 patients. In normal subjects bilateral coactivation was observed with lateral gaze (96%), convergence (61%), active and intended head rotation (100%) and passive head rotation (50%). Uni- and bilateral labyrinthine excitation (60 and 80%) and proprioceptive input from the neck muscles (38%) are also effective. In patients with brainstem disease abnormal transverse auricular muscle coactivation is characterized by absence of activity in one or both ear muscles during lateral gaze in either or both directions. The most frequent abnormality was absence of transverse auricular muscle activity homolateral to right or left gaze (type Ia pattern). It was related to homolateral impairment of the blink reflex R1-response (90%) and the caloric response (90%), and to contralateral masseter reflex abnormality (70%). Electrophysiological data, clinical signs and imaging findings indicate that the type Ia pattern is caused by homolateral pontine or contralateral midbrain lesions. It is suggested that the supranuclear organization of the oculo-auricular phenomenon is based on descending tracts crossing at a mid-pontine level.

Brain Diseases↗

Auricular granuloma annulare. A consequence of trauma?

Localized granuloma annulare (GA) is a cutaneous disorder characterized by the formation of palisading necrobiotic granulomas. Although trauma and vasculitis have been considered, the etiology of most cases remains obscure and the disease is generally considered to be idiopathic. The condition may occur at any age and at any site, but usually involves the hands or feet of children or young adults, predominantly affecting females. We present two cases occurring as nodules involving the external ears of males. One of these showed a bilateral distribution and was clearly related to trauma.

Adult↗

Rosette formation within a proliferative nodule of an atypical combined melanocytic nevus in an adult.

Rosette formation is a feature that has not been described as occurring in melanocytic neoplasms. We present such a unique case. A 59-year-old man presented with an asymptomatic, soft, hairy 3.0 x 2.0-cm pigmented lesion that had been present for many years in the right external ear, extending from the conchal bowl onto the antitragus area. Examination of histologic sections showed a proliferation of nonatypical and heavily pigmented melanocytes in the superficial dermis and around deep adnexal structures, characteristic of a congenital nevus. In other areas, pigmented spindled and dendritic cells infiltrated thickened collagen bundles in a pattern of a blue nevus. A nodular proliferation of epithelioid melanocytes was seen within the deep dermis and subcutaneous tissue. The periphery of the nodule merged with the surrounding nevus cells. Neoplastic cells with nuclear atypia, melanin pigment, pseudonuclear inclusions, and balloon cell change were present. In addition, there was rosette formation by the tumor cells, with a central aggregate of coarse cell processes. Neuroid cords were also noted. No prominent mitotic figures, necrosis, or significant inflammatory infiltrate were noted. The neoplastic cells were positive for S-100 protein, Mart-1, tyrosinase, neuron-specific enolase, and vimentin. HMB-45 and Ki-67 (MIB-1) labeled only rare neoplastic cells within the proliferative nodule. The tumor cells were negative for synaptophysin, protein gene product 9.5, CD57, epithelial membrane antigen, CD31, and CD34. The central cell processes of the rosettes were negative for trichome, type IV collagen, neurofilament protein, glial fibrillary acidic protein, and tyrosine hydroxylase. We also retrospectively examined 78 congenital nevi of 65 pediatric patients at our institution. Rosette formation was not seen in any of these cases.

Biomarkers, Tumor↗

Giant cell tumor of the petrous temporal bone with direct invasion into the middle ear.

Giant cell tumor (GCT) is an uncommon primary bone tumor. The GCT mostly involves the extremity long bones. Rare is a GCT with tumoral growth in the cranium, especially other than the sphenoid bone. We herein report a 31-year-old male patient presenting with ipsilateral hearing loss and episodes of vertigo due to a large lobulated GCT of the right petrous temporal bone extending into the contiguous middle cranial fossa, infratemporal space, middle ear and external ear canal. He was treated with a macroscopically radical tumor excision followed by conventional megavoltage radiotherapy. The patient remains free of clinical and radiological evidences of tumoral recurrence six years after treatment. This experience supports the rationale for the combined treatment with radical excision plus irradiation toward this rare neoplasm when vigorous invasion of the skull base is encountered.

Adult↗

A quantitative morphometric study of the kinetics of tissue regeneration after administration of cisplatin.

The effect of the anti-neoplastic drug cisplatin was investigated on several rat tissues using a novel computerized morphometric image analysis system. The rats were sacrificed in pre-determined intervals, ranging from 1 to 36 days after drug administration, and their liver, kidneys and external ears were sampled and sectioned. Tritiated thymidine was injected into each rat 1 h prior to sacrifice to enable autoradiographical explorations. All sections were histomorphometrically studied by a computerized system. The kidneys of the experimental group revealed increased tubular diameters, especially in the corticomedular region. In the ears, decreases of the mean epithelial thickness, in the mean number of nuclei and in the mean nuclear surface were observed. The thickness of the connective tissue also decreased significantly by the end of the first week and returned to its normal size later on. No changes were detected in the ear cartilage. In the liver, no morphometrical differences were noticed in the hepatocyte density, Kupffer cell density or nuclear area. The nuclei of the hepatocytes and Kupffer cells retained a constant ratio to the total number of cells throughout the whole experiment. Liver autoradiography revealed that the hepatocyte and Kupffer cell labelling indexes after cisplatin administration were significantly higher than those of the control group, indicating enhanced replication of cells and increased synthesis of DNA, which in turn is accumulated in the nuclei and turn the cells into polyploids. In addition, the mean radius of the liver acini after cisplatin was diminished, followed by a significant reduction in the size of the progenitor compartment. It is concluded that while the kidneys are the organs most affected by cisplatin, the progenitor compartment of the liver is heavily affected as well. Computerized morphometry was demonstrated as a highly reproducible and accurate quantitative method for evaluation of histopathological changes in various mammalian tissues.

Animals↗

Facial and neuroepithelial abnormalities in a neurological mutant with congenital hydrocephalus. A scanning electron microscope study.

This investigation was carried out to determine the early structural abnormalities of the cephalic region in a genetic mutant of the rat characterized by prenatal aqueductal stenosis and hydrocephalus. The appearance of hydrocephalic and control embryos was examined on days 13-15 of gestation, and the structure and organization of the neuroepithelium and basal lamina were studied using scanning electron microscopy. In addition to some overall developmental delay, hydrocephalic embryos were characterized by abnormalities of forebrain and midbrain development, and eye and external ear anomalies. There were also associated defects of the midfacial region. The lateral cell surface of the neuroepithelium reflected the developmental delay of hydrocephalic embryos, and failed to undergo the morphogenetic cell-shaping changes seen in control embryos. There were also variations in the number of lateral cell-cell specializations as well as regions of neuroepithelial disorganization and occasional herniation into the mesenchymal compartment. The role of the neuroepithelial basal lamina and extracellular matrix in the development of these defects is considered.

Animals↗

Free groin flap revisited.

Reported herein are 130 consecutive cases of free groin flap transfer performed by one surgeon over a 19-year period. Transplantation was performed for soft-tissue cover or augmentation of contour defects involving the head and neck (68 cases), trunk (4 cases), upper limb (14 cases), and lower limb (44 cases). Indications for flap coverage/augmentation were classified broadly into tumor, trauma, radiation induced, and miscellaneous. Specific reconstructive problems included augmentation for Romberg's hemifacial atrophy, external ear canal reconstruction after tumor ablation, and coverage of lower limb defects. There were nine failures (total flap loss), seven cases of partial flap loss, and two cases were abandoned intraoperatively. Of 15 cases that were urgently re-explored, 9 flaps were salvaged. The failure rate for the groin flap series (130 cases) was 8.5 percent compared with the failure rate of 4.2 percent for the other 517 cases of microvascular transfer performed over the same period by the same surgeon. Donor-site complications occurred in 24 cases and included hematoma or seroma formation, hypertrophic scars, nerve paresthesiae, infection, and dehiscence. Secondary debulking procedures were performed in 26 cases. The free groin flap, contrary to some reports, is a reliable flap that provides relatively thin pliable soft-tissue cover or augmentation, with minimal donor-site morbidity. The specific indications for its use have undergone an evolution since first described in 1973.

Adolescent↗

Auricular reconstruction: an update.

PURPOSE OF REVIEW: One of the greatest challenges in facial plastic surgery is total auricular reconstruction. The ability to construct a fully satisfactory complete external ear has for centuries been an elusive goal. With advancing standards and expectations in plastic surgery, greater focus is being directed to this arena of reconstruction as well. RECENT FINDINGS: With advances both in surgical technique and biotechnology, an expanding range of options is available to the reconstructive surgeon who is willing to take on this still formidable task. While progress continues to be made in the detailing of costal cartilage grafts and soft-tissue rearrangement, it is also being made with the use of alloplastic implants and prostheses. The most promising field of advances, with the hope of eventual clinical utility, lies in the realm of bioengineering with cultured cartilage. SUMMARY: Review of the literature over the past year reveals several reports describing advances made in the development of cultured chondrocytes with attempts to direct special shape to the manufactured neocartilage. Though advances continue to be made, actual clinical applicability of this technology is still insufficient while expectation continues that this will eventually become integral to total auricular reconstruction.

Cartilage↗

The earlobe crease, coronary artery disease, and sudden cardiac death: an autopsy study of 520 individuals.

The majority of previous studies have demonstrated a correlation between diagonal earlobe creases (ELC) and coronary artery disease (CAD). In this study of 520 forensic autopsy cases, the earlobes were studied and photographed before autopsy, and the existence of a diagonal ELC was noted in 55%. The cause of death, the degree of coronary atherosclerosis, aortosclerosis, and cerebrosclerosis, as well as heart, kidney, and spleen weights, were noted in each case. The body mass index (BMI), thickness of abdominal fat, baldness, and excessive hair in the meatus externa of the external ears were also assessed. Nonparametric methods were used in the statistical calculations. It was found that ELC was strongly correlated with CAD in both men and women (P < 0.0001) but with sudden cardiac death (SCD) only in men (P < 0.04). The sensitivity of the ELC sign was 75% and the positive predictive value (ppv) was 68%. In individuals below 40 years, the ppv was as high as 80%. Using multiple logistic regression analysis, ELC was found to be the strongest independent risk factor for CAD and SCD apart from age and BMI (both genders), as well as baldness and hair in the meatus externa (in males). It is concluded that in a patient population similar to that in the present study the ELC sign could be especially useful in screening for premature CAD in younger individuals.

Adolescent↗

Auricular arteriovenous malformation: evaluation, management, and outcome.

BACKGROUND: The external ear is the second most common site for extracranial arteriovenous malformation in the head and neck. METHODS: This retrospective review of 41 patients with auricular arteriovenous malformation was based on medical records, imaging studies, and photographs. Data were collected on natural history, progression, and outcome; patients were questioned about quality of life after treatment. RESULTS: The median age at initial presentation was 26 years (range, 1 to 55 years), and Schobinger stage was I in two patients, II in 19 patients, and III in 20 patients. No patients had a Schobinger stage of IV. Expansion occurred during childhood in seven patients, adolescence in 14 patients, pregnancy in 10 patients, and adulthood in 10 patients. Distribution of auricular and extra-auricular arteriovenous malformation was not limited to "watershed" areas between vascular territories (angiosomes). Twelve patients were untreated (follow-up, 0.5 to 6 years). Mean follow-up time for the 29 treated patients was 5.19 years (range, 1 to 18.75 years). Proximal ligation in nine patients caused progression: eight of them underwent amputation and one had embolization. Fifteen patients had embolization only: the arteriovenous malformation worsened and amputation was necessary in six patients; in the remaining nine patients, two improved, four persisted, and three worsened. Of 20 patients who had auricular amputation, 16 (80 percent) were controlled, three (15 percent) improved, and one had unresectable, residual cervicofacial arteriovenous malformation. Of 22 of 29 treated patients surveyed, 81 percent were satisfied with their management. Hearing was either unaffected (n = 15) or diminished (n = 5); two patients noted decreased sound localization. CONCLUSIONS: The authors recommend periodic evaluation for stage I to II auricular arteriovenous malformation and intervention if there is evolution to stage III. Preoperative embolization and partial or total amputation effectively control auricular and para-auricular arteriovenous malformation. Embolization can be palliative in children or in patients who are not psychologically prepared for amputation. Extensive extra-auricular arteriovenous malformation requires individualized endovascular therapy and resection.

Adolescent↗

The role of the antitragicus muscle in plical folding of the pinna.

BACKGROUND: The antitragicus is one of six intrinsic muscles of the external ear. This study examined the anatomical variations of the muscle and its association with prominent ears. METHODS: Twenty-seven consecutive patients presenting for correction of prominent ears were recruited into the study. In each case, the antihelical fold was assessed preoperatively by a single investigator. A standard anterior cartilage scoring otoplasty was performed, and the presence and quality of the antitragicus muscle were assessed by a second investigator. For statistical analysis, the qualitative assessment of the fold and the muscle was given a numerical value. It was then possible to plot the qualitative assessment of the fold against the muscle, and correlations between muscle and fold were calculated. RESULTS: The presence of the muscle was associated with an absent antihelical fold, whereas a well-defined antihelical fold was associated with an absent muscle (p < 0.0001). CONCLUSIONS: The authors propose that the presence of a well-formed antitragicus muscle, which exerts an anterior pull on the cauda helicis, contributes to the poor development of the antihelical fold.

Adolescent↗

Congenital auricular anomalies: topographic anatomy, embryology, classification, and treatment strategies.

Congenital auricular anomalies are heterogeneous, with various descriptive and eponymous terms being used. Current classification systems are useful in guiding surgical treatment of severe anomalies. However, they do not generally account for the less severe anomalies, which form the majority of congenital auricular anomalies, nor their contemporary treatment. In this article, the authors review the anatomy and embryology of the external ear and propose a simple classification of congenital auricular anomalies that encompasses all forms of congenital auricular anomalies, facilitates proper diagnosis, and guides treatment. Congenital auricular anomalies should be classified as malformational or deformational anomalies. Malformational auricular anomalies are caused by embryologic maldevelopment that occurs between the fifth and ninth week of gestation resulting in deficient and/or supernumerary auricular components. Deformational auricular anomalies result from in utero or ex utero deformational forces, including those caused by an aberrant insertion of the intrinsic or extrinsic auricular muscles. Malformational auricular anomalies generally require surgical correction during childhood or adolescence. For practical purposes, deformational auricular anomalies have a full complement of chondrocutaneous components that can be digitally manipulated to a normal shape. These anomalies are best treated by auricular molding, which is effective if it is initiated within the first 3 months of life. Deformational auricular anomalies are best regarded as a pediatric public health issue and are best managed nonsurgically. Education of neonatal pediatricians, obstetricians, family doctors, and midwives will allow proper early diagnosis of all congenital auricular anomalies, which is vital to appropriate treatment. These practitioners should be encouraged to manage deformational auricular anomalies early in life so that surgery can be largely avoided in these patients.

Congenital Abnormalities↗

Pigmented epidermal cyst.

A 58-year-old Japanese woman with a pigmented epidermal cyst on the upper arch of the right external ear is described. The blue appearance of the cyst was striking and uncommon in Japanese. Histologically, abundant melanin pigment was observed in the keratinous mass of the cavity, and epithelial cells showed epidermoid keratinization. Increased numbers of melanocytes in the basal layer, large globular cells of melanocytic origin in the spinous layer, and hair-germ-like aggregates of basaloid cells and melanocytes were also observed. The blue clinical appearance is probably due to the rich melanin granules in the cyst, keratinous mass, keratinocytes, and hair-germ-like structures.

Ear, External↗

Enhancement of comedogenic substances by ultraviolet radiation.

Ultraviolet radiation enhanced the capacity of human sebum, sulphur, cocoa butter, squalene, and coal tar to produce comedones in the external ear canals of rabbits. An enhancement of the comedogenicity of coal tar and squalene was similarly demonstrated in man. We conjecture that in occasional patients sunbathing may aggravate acne by augmenting the comedogenicity of sebum.

Acne Vulgaris↗

Basal cell carcinoma of the external auditory meatus.

Five of a personal series of 47 patients with carcinoma of the middle and external ear had basal cell carcinoma. Two patients had extensive disease, one having petrosectomy and the other refusing. All treated patients did well.

Adult↗

Lacrimo-auriculo-dento-digital (LADD) syndrome with renal and foot anomalies.

A patient who presented with most features of lacrimo-auriculo-dento-digital (LADD) syndrome, an autosomal dominant trait, is described. There was no deafness, and anomalies of the external ear and the upper limbs were discrete. Renal anomalies, consisting of progressive caliectasis with stone formation, were revealed by macroscopic hematuria. There were also skeletal anomalies of both feet, a feature not previously described. Renal and distal limb anomalies are probably features of LADD syndrome.

Child↗

Auricular ossification.

A patient with extensive bilateral auricular ossification presented with chondrodermatitis nodularis helicis on one side. The condition was otherwise asymptomatic. Ossification was detected on radiological and histological examination. Underlying medical conditions were not found. We believe this developed as a consequence of cold injury. Auricular ossification is an unusual cause of the so-called petrified external ear, in which the subcutaneous tissue is stony hard. It is more commonly caused by dystrophic calcification. Calcification and ossification are clinically identical and histological examination is required to definitively differentiate them.

Aged↗

Renal hypoplasia and situs inversus totalis.

A spectrum of renal abnormalities of patients with situs inversus has been reported. Renal dysplasia is the most common. Herein is described for the first time, an association of situs inversus totalis, unilateral congenital renal hypoplasia and external ear cartilage deformity.

Adult↗