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New concept in microtia repair.

Reconstruction of the severely deformed external ear has been a frustrating experience for many patients and surgeons alike. Until recently, children with microtia (1:10,000 births) have been subjected to multiple procedures with frequently ungratifying results. Because of the rarity of this deformity, most surgeons have had little personal experience with the common problems encountered in microtia reconstruction. The problems of (1) proper location of the reconstructed ear, (2) size and shape, and (3) durability of the implanted graft can be prevented by adherence to newer principles and techniques. When the four-stage reconstruction is begun at 5 years of age, an adequate and durable costal cartilage framework can be sculptured and implanted. Over the past four years, the use of these principles and techniques in 13 patients has resulted in gratifying results.

Child↗

Minor malformations and physical measurements in autism: data from Nova Scotia.

In the context of an epidemiological study of autism in Nova Scotia, subjects were evaluated for minor physical anomalies and physical measurements. Normal control children, children with autism and their siblings, and children with developmental disabilities and their siblings were compared. Posterior rotation of the external ears was found to be a characteristic related to autism specifically, rather than to developmental disabilities in general. Small feet and normal-to-large hands also were observed in the autism group. Children with autism had a significant reduction in interpupillary distance, but not intercanthic distance or head circumference. In contrast, children with other developmental disabilities were notable for general small stature, which affected the hands, feet, eyes, and head size, as well as height. Abnormal ear configuration was the minor malformation most characteristic of the developmental disability group, and the subset of Down syndrome children had single transverse creases of the palm and epicanthic folds that resulted in significantly increased rates of these anomalies in the developmentally disabled controls. Siblings of the two disabled groups were not significantly different from normal controls on any of the measures that characterized children with autism or other developmental disabilities. The results agree with those of several previous studies, which have suggested that abnormalities of the ears are the general category of minor anomalies most associated with autism. Recent evidence regarding the embryological origin of autism suggests that the ear effects may be an important marker of the initiating events that lead to the disorder.

Abnormalities, Multiple↗

Radial ray aplasia and renal anomalies in father and son: a new syndrome.

We describe a father and his son with bilateral absence of radius and thumb. Both have short stature, external ear malformation, and renal anomaly. In the son a high frequency of chromosome breaks in lymphocytes was found. We compare this familial syndrome to Fanconi anemia and other radial ray aplasia syndromes and conclude that we are dealing with a different entity, which apparently is inherited as a dominant trait.

Adult↗

Congenital contractural arachnodactyly: description of a new kindred.

A previously unreported case of congenital contractural arachnodactyly (CCA) is described. This hereditary connective tissue abnormality resembles Marfan's syndrome in certain respects, but is characterized by camptodactyly rather than joint laxity, as well as by congenital contractural deformities of the knees and elbows. In addition, there is a peculiar, fairly characteristic deformity of the external ear. Like Marfan's syndrome, it is transmitted in autosomal dominant fashion. Despite the superficial skeletal resemblance, however, the cardiovascular and ocular complications of Marfan's do not seem to occur and therefore differentiation of the two syndromes is important.

Adult↗

Epidemiologic characteristics of anotia and microtia in California, 1989-1997.

BACKGROUND: There is a paucity of epidemiologic information about the external ear malformations anotia and microtia. METHODS: Using data from a large population-based registry, we explored prevalences and maternal/infant characteristics associated with anotia and microtia. Data were derived from the California Birth Defects Monitoring Program, a population-based active surveillance system for collecting information on infants and fetuses with congenital malformations using multiple source ascertainment. Approximately 2.5 million births (live-born and stillborn) occurred during the ascertainment period, 1989-1997. Information on maternal and infant/fetal characteristics was obtained from California birth certificate and fetal death files. RESULTS: The prevalence of anotia/microtia was 2.50 per 10,000 live births and stillbirths. Isolated cases, i.e., those live births and stillbirths with no other accompanying anomaly diagnoses, revealed a prevalence of 0.63 per 10,000, whereas among nonisolated cases, the prevalence was 1.53 per 10,000 live births and stillbirths. Prevalences of isolated anotia/microtia were substantially higher among U.S.-born Hispanics, foreign-born Hispanics, and Asians compared to non-Hispanic whites, with relative risks ranging from three- to seven-fold. Similar prevalences for maternal race/ethnicity (except for Asians) were observed for live births and stillbirths with nonisolated cases of anotia/microtia, but the magnitude of risks were lower than those observed for isolated cases. Prevalence of nonisolated anotia/microtia was substantially lower among women whose education was 12 years or more. CONCLUSIONS: These data reveal descriptive epidemiologic features of anotia/microtia most notably substantial risks associated with race/ethnic groups and lower maternal education. Additional study for factors associated with these characteristics could reveal clues for the etiology of these ear anomalies.

Adult↗

Branchio-oto-renal dysplasia associated with tetralogy of Fallot.

Branchio-oto-renal dysplasia in its full expression consists of external ear malformations, cervical fistulas or cysts, preauricular pits, hearing loss of conductive sensorineural or mixed type, and renal anomalies. The syndrome is inherited in an autosomal dominant mode. We present a sporadic case of BOR dysplasia associated with tetralogy of Fallot in a 30-month-old girl. The clinical aspects of the disease are discussed briefly.

Branchial Region↗

Aesthetic surgery for microtia.

We describe here a technique for reconstruction of the external ear based upon an autogenous costal cartilage graft which is inserted into a cutaneous pocket dissected in the auricular area. Three subsequent procedures are then performed: rotation of the ear-lobe; reconstruction of the tragus; and elevation of the auricle. The ideal age for reconstruction is about 7 years. This technique was originally described by Brent, who has a very extensive experience with this kind of surgery. Skin deficiencies can be overcome by using either a temporo-parietal fascial flap or a skin expander.

Cartilage↗

Invasion of malignant C3H mouse fibroblasts from aggregates transplanted into the auricles of syngenic mice.

Spheroid aggregates of malignant fibroblasts (MO4), shown to be invasive in vitro, were implanted subcutaneously into the auricle of the external ear of syngenic C3H mice. The course of early invasion into the surrounding tissues and the formation of tumours was studied in serial sections of auricles fixed 6 h to 30 days after implantation. MO4 cells are first observed to make contact with the surrounding tissues after 6 h. They exhibit, cytoplasmic extensions and spread from the original aggregate. During the first day invading MO4 cells preferentially follow tissue crevices created by the inoculation procedure. Later they also invade the surrounding tissues. Polymorphonuclear leucocytes, and later monocytes infiltrate the aggregate, which is completely destroyed after 4 days. Palpable tumours arise from MO4 cells that have left the original implant and invaded the tissues of the auricles. These observations indicate that invasion of malignant cells is important for the take of a transplant.

Animals↗

Prenatal detection of microtia by MRI in a fetus with trisomy 22.

Trisomy 22 is a rare chromosomal abnormality infrequently detected prenatally. External ear abnormalities, in particular microtia, are often associated with trisomy 22, but prenatal detection of microtia has not been reported in association with trisomy 22. We report a fetus with trisomy 22, with fetal MRI findings of microtia, craniofacial dysmorphism, and polygyria. Fetal MRI is a useful tool for auricular assessment and might have utility in the prenatal detection of chromosomal abnormalities, especially among fetuses with structural anomalies.

Autopsy↗

Otoplasty: a comparison of techniques for antihelical defects treatment.

BACKGROUND: Prominauris, the most common malformative defect of the external ear, is dependent on a combination of elementary defects mainly affecting the antihelix and the concha. Transection versus cartilage weakening results in antihelix treatment are discussed. METHODS: In this study, 104 ears with antihelical defects were treated in 63 consecutive patients: the 33 patients (53 ears) in group A, received a transection-based treatment, whereas the 30 patients (51 ears) in group B, underwent cartilage weakening and suture. All the patients were treated using a posterior access. The aesthetic results were evaluated by an independent well-trained plastic surgeon. RESULTS: No differences were found in correction degree and stability between the two methods, but cartilage weakening techniques provided a smoother and more natural curvature. CONCLUSIONS: Cartilage weakening techniques can lead to better aesthetic results, primarily because of a softer and more natural ear curvature.

Adolescent↗

A tissue-engineering model for the manufacture of auricular-shaped cartilage implants.

The established surgical methods of external ear reconstruction using autogenous tissue represent the current state of the art. Because of the limited possibilities for shaping conventional harvested autogenous rib cartilage, the cosmetic results of auricular reconstruction are frequently unsatisfactory. Tissue engineering could represent an alternative technique for obtaining a precisely shaped cartilage implant that avoids donor site morbidity and unsatisfactory cosmetic results. In this study, the reliability and quality of a tissue-engineering model for the manufacture of auricular-shaped human cartilage implants was investigated, focusing on the feasibility of the manufacturing process and the in vivo and in vitro maturation of an extracellular cartilage-like matrix. Implants were molded within an auricular-shaped silicone cylinder, and human nasal septal chondrocytes crosslinked by human fibrin within bioresorbable PGLA-PLLA polymer scaffolds were used. After an in vitro incubation of up to 6 weeks, defined fragments of the prefabricated auricular-shaped construct were implanted subcutaneously on the backs of nude mice for at least 6 to 12 weeks ( n=7). Scaffolds without cell loading served as controls. Macroscopic and histochemical examination after 3 and 6 weeks in vitro showed a solid compound of homogenously distributed chondrocytes within the polymer scaffold, leading only to a limited pericellular matrix formation. Analysis after 6 and 12 weeks of in vivo maturation demonstrated a solid tissue compound and neocartilage formation with the presence of cartilage-specific matrix components. Implants obtained shape and size during the entire period of implantation. The model of cartilage implant manufacturing presented here meets all biocompatible requirements for in vitro prefabrication and in vivo maturation of autogenous, individually shaped cartilage transplants.

Animals↗

Cryptotia: our classification and treatment.

Our classification of cryptotia is based on recognition that the deformity is the result of an anomaly of the intrinsic transverse and oblique auricular muscles. Our treatment, in the first instance, is a non-surgical correction. This should be started early, if possible in the first 6 months of the baby's life. Even in the case of older children, success can sometimes be achieved and a non-surgical correction should be considered first. If non-surgical management fails the deformity can be corrected surgically by dividing the muscle fibres that produce the anomaly of the external ear through a small incision. Our new surgical approach involves moving the available skin in the region of the inferior cephalo-auricular sulcus to the superior part where skin is needed by a rotation flap. This operation has several advantages.

Age Factors↗

Mercury induces in vivo and in vitro secretion of interleukin-1 in mice.

Macrophages from SJL and DBA mice incubated with mercuric chloride (HgCl2) in vitro for 24-72 h secreted an increased amount of interleukin 1 (IL-1) to the supernatant compared with control-incubated macrophages, as determined by a sensitive thymocyte proliferation assay. The increase of IL-1 activity showed a highly significant dose-response relationship, being close to that in controls at 10(-8) M, and maximal after incubation with 10(-5)-10(-6) M HgCl2 in both strains. At optimal concentrations of HgCl2 the IL-1 activity started to increase after 6 hrs incubation and reached a maximum after 48 h. Incubation with concentrations of HgCl2 higher than 10(-5) M resulted in a severely reduced IL-1 activity, which correlated with a reduced cell viability. Extracts of HgCl2-incubated macrophages representing cell-bound IL-1 showed no increase in IL-1 activity, irrespective of the concentration or incubation time. Topical application of HgCl2 in a mixture of acetone-olive oil on the external ear of SJL mice induced a dose- and time-dependent increase in IL-1 activity. A maximal increase was seen after application of 1% HgCl2 for 24 h with lower IL-1 activity after 48 and 72 h. Application of 5%, but not 1% or 0.1%, slightly increased the IL-1 activity in the contralateral ear treated with acetone-olive oil only, as compared with the activity in ears from animals given no mercury treatment, suggesting a systemic effect by application of 5% HgCl2.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Rapid development of the auditory brainstem response threshold in individual ferrets.

The development of the ferret auditory system was examined using the auditory brainstem response (ABR). Longitudinal recordings were obtained under short-acting anaesthesia from individual animals at 4-h or 24-h intervals. Particular attention was focused on the period from postnatal day (P) 26 to P32 when the ferret auditory system becomes functional. ABR thresholds to click stimuli presented in a free-field were found to decline precipitously within a 4-h period during the first 24 h following initial responsiveness. Latencies of waves I and IV of the ABR also declined significantly during this period, but the 'central conduction time' remained stable. A temporal correlation was observed between the time of the precipitous threshold decline and the time of opening of the external ear canal. No changes in cochlear anatomy were observed during this time. We suggest that the opening of the ear canal and/or the clearance of fluid from the middle ear explain the major change in threshold of hearing following onset of function.

Aging↗

Totally implantable hearing aids: the effects of skin thickness on microphone function.

With the advent of totally implantable hearing aids, the question of the impact of the choice of implantation site on microphone function has yet to be fully addressed. We investigated the effects of skin thickness on microphone function over a 2-week period in a porcine model. Sound attenuation was found to be directly proportional to skin thickness within the range of 0.5 to 3.0 mm. A decrease in resonant frequency of 500 Hz was noted after implantation under a skin thickness of 3.0 mm. The general shape of the frequency response curves was maintained across all thicknesses tested. Attenuation (dB loss) across the range of 500 to 8,000 Hz appeared to be linear over the various skin thicknesses measured, with a regression coefficient (r = .99 at 1 kHz and r = .99 at 3 kHz). To minimize attenuation and exploit the sound-collecting qualities of the external ear, the deep meatal skin appears to be a favorable position for implantation.

Animals↗

Subtotal reconstruction of the burned auricle.

The goal for external ear reconstruction is to restore the function of the helical rim in order to provide support for eyeglasses and to provide a cosmetically acceptable auricle. Reconstruction necessitates both flap coverage and flexible framework formation. This article reviews 22 patients who underwent subtotal reconstruction of the auricles after burns. The study was conducted from 1996 to 2003. The study group consisted of 14 male and 8 female patients aged 6-43 years (mean 21.9 years). Different modalities have been used: the temporoparietal fascial flap (eight patients), the subcutaneous pocket technique (11 patients), the pre-auricular skin flap (four patients), and the post-auricular skin flap (three patients). The flaps were used for coverage of fabricated autogenous cartilage in all patients. Both aesthetic and functional outcome was satisfactory.

Adolescent↗

Reconstruction of the auricle.

The intricate and delicate anatomy of the external ear (auricle) makes reconstruction a daunting task for beginning as well as experienced surgeons. The complex framework of the auricle is composed of graceful curves, peaks, and valleys of a thin pliable uniform thickness of cartilage. This article discusses techniques for reconstruction of the auricle.

Cartilage↗

Isolation of Malassezia species from healthy cats and cats with otitis.

Lipid-dependent Malassezia species have recently been cultured from veterinary specimens. The identification of Malassezia species isolates from animals is important to clarify the epidemiology of these lipophilic yeasts. Malassezia species were cultured from the external ear canals of 63 out of 99 cats with otitis and 12 of 52 (23%) healthy control cats. The rate of isolation in affected animals versus controls was highly significant (P<0.01). Malassezia pachydermatis was isolated as a pure culture in 33 (45.2%) cats, associated with Malassezia globosa and Malassezia furfur in 20 (50%) and 17 (42.5%) animals, respectively. Three different species were isolated simultaneously in three cats (two cats with M pachydermatis, M globosa and M furfur, one subject with M pachydermatis, M furfur and Malassezia sympodialis). M globosa was isolated as the sole species in two animals. The present work confirms the presence of some lipid-dependent species of Malassezia in both healthy and otitic cats.

Animals↗