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External ear deformities by themselves do not require emergent treatment, but associated abnormalities need to be defined to enable long-term planning. The pediatrician can orchestrate the specialists and keep parents and patient informed maximally.
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Congenital deformities of the external and middle ears were shown by tomography in 246 patients. Surgical exploration has been undertaken at some time in many of these ears. The appearance of the tomograms and their significance were reviewed in the light of the operative findings and the authors' experience of congenital ear lesions. In nearly every case a middle ear cavity could be demonstrated although this varied from a normally aerated middle ear and mastoid in association with an isolated unilateral atresia of the external auditory meatus to a small slit-like hypotympanum in patients with craniofacial abnormalities. The ossicles were nearly always present but deformed. The typical appearances of the ossicles and the frequently aberrant pathways of the facial nerve are described.
The present study describes an anomaly of the pinna of the ear and its distribution in Highland Cattle. The investigation is based on a questionnaire survey in farms in Bavaria keeping robust breeds and on registering data in 15 selected farms. In the year 1991, there were registered 548 Highland stud book cows in 108 farms. In four herds animals of the breed Highland Cattle were observed which showed crop ears. Always both ears were affected and this ear defect could be already observed in newborn calves. Crop ears appeared in very different forms. In some cases only small changes of the external ear could be recognized, in more severe cases, grooves in the external ear could be found and in the most severe case, the external ear was totally deformed and drastically reduced in size. In one herd, 45 animals out of the progeny of 46 of one affected breeding bull showed crop ears. The pedigrees indicated that this defect of the ear pinna is inherited and a single autosomal gene with nearly additive (incomplete dominant) action may be involved. Homozygote recessive animals are free from crop ears, animals homozygote for the mutated allele carry totally deformed external ears and heterozygote animals do not show as severe forms of crop ears as homozygote ones.
Ten children with gross deformity of the external ear were observed. In six the facial bones were underdeveloped on the same side as the deformed ear. In all six there was a congenital abnormality of the kidney or upper urinary tract, usually on the same side as the deformed ear. In addition there were usually other associated congenital defects in each case.In the remaining four children the facial bones appeared normal, and pyelography showed no abnormality of the urinary tract. In these four children there were no other associated defects.These observations emphasize the importance of investigating the urinary tract in children with gross deformity of the external ear, especially where there is an associated underdevelopment of the facial bones.
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The author treated 146 cases of congenital external ear deformities, proposing their classification into four groups: anotia, microtia, macrotia, and prominent ears. The importance of a normal appearing ear in the overall facial harmony and contour is emphasized and reconstructive techniques to accomplish this are described.
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