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Hearing loss in infants with craniofacial anomalies.

Results of auditory brainstem response (ABR) evaluation of 145 infants, ages 6 months and younger with craniofacial anomalies (CFA), were examined to determined predicted degree and nature of hearing loss. Approximately 50% of infants demonstrated at least mild bilateral hearing loss. Presence and degree of hearing loss varied by CFA. All infants with bilateral aural atresia exhibited at least a moderate bilateral hearing loss; less than 20% of infants with isolated external ear anomalies (ear tags, isolated microtia) exhibited any degree of hearing loss. In 92% of infants with hearing loss, results of ABR were consistent with primarily conductive dysfunction. Implications for early identification and audiologic management of infants with CFA and hearing loss are discussed.

Acoustic Stimulation↗

Auricular chondropathy in aging rats.

An external ear lesion designated as auricular chondropathy in aging Crl:CD rats was reported. One hundred eighty (180) of 4,876 rats (3.7%) had ear deformity. Grossly, the ears were thickened, nodular, or disfigured. Both ears were affected in most rats. The lesions primarily involved the cartilaginous plate and were associated with two processes--chondrolysis and granulomatous inflammation. The detailed morphogenesis of the lesions is described, but the etiology of the lesions is unknown. Auricular chondropathy in rats and relapsing polychondritis in man are compared.

Animals↗

A case of Duane's retraction syndrome with multiple congenital malformations.

Duane's retraction syndrome is associated with various ocular and systemic abnormalities. We report a case with bilateral Duane's retraction syndrome type I accompanied by renal agenesis, vesico-ureteral reflux, patent ductus arteriosus, and external ear malpositioning. Because of disabling consequences, we recommend a thorough physical examination and screening for renal and cardiac abnormalities in all patients presenting with Duane's reaction syndrome.

Abnormalities, Multiple↗

External auditory canal duplication anomalies associated with congenital aural atresia.

Maldevelopment of the first branchial cleft can produce a broad spectrum of anomalies in its derivative structure, the external auditory canal (EAC). Failure of the cleft to develop normally can result in either the absence of a normally patent EAC (atresia, or stenosis) or a duplication anomaly (cyst, sinus, or fistula). Despite their common origins, the coexistence of these anatomical abnormalities is quite unusual. We present four patients with both aural atresia and duplication anomalies of the EAC. Three patients had non-syndromic unilateral aural atresia and presented with periauricular lesions originating from the first branchial cleft. The other patient had a variant of Treacher Collins syndrome and presented with draining infra-auricular fistulae. The classification and management of first branchial cleft anomalies is reviewed in light of these cases. An understanding of the embryogenesis of the external ear is necessary to successfully recognize and treat this spectrum of deformities. A classification system is presented that encompasses the full spectrum of first cleft anomalies.

Adult↗

Local control of auricular, periauricular, and external canal cutaneous malignancies with Mohs surgery.

Three hundred ninety-seven patients with 407 cutaneous malignancies of the auricle, periauricular region, and cartilaginous external ear canal were reviewed. Tumors were most commonly located in the preauricular and postauricular regions, followed by the helix, concha, antihelix, and ear canal. All lesions were excised with Mohs microscopic control of margins. For lesions requiring lateral temporal bone resection, an adaptation of fresh-tissue microscopic control was used to analyze deep and anterior margins suspected of harboring residual tumor. Two-year minimum follow-up of 229 patients with periauricular and auricular tumors (N = 231 tumors) and 14 patients with cartilaginous ear canal tumors (N = 14 tumors) revealed recurrence rates of 6.9% and 14.3%, respectively. Recurrences were most common in cases of large tumors (greater than 2.5 cm), basal cell carcinomas with morphea elements, and multiply recurrent lesions. We conclude that Mohs surgery is comparatively effective, though not uniformly curative, and can be adapted to supplement excision of large tumors in these regions.

Basal Cell Carcinoma↗

A new approach to serial monitoring of ultra-high frequency hearing.

Early detection of drug-induced hearing loss is best accomplished by monitoring hearing at the ultra-high frequencies. Unfortunately, at these frequencies, sound pressure at the tympanic membrane (TM) critically depends on the placement of the sound source and on the size and shape of each individual external ear. Thus, presentation of the same sound may yield substantially different sound pressures in different ears. Moreover, only a slight change in the position of an earphone may yield large changes in sound pressure at the tympanic membrane. As a consequence of these characteristics, the reliability of ultra-high-frequency audiometry is poorer than at conventional audiometric frequencies. However, for the early detection of ototoxicity, it is necessary only to monitor for increases in thresholds. Accordingly, a sound-delivery system was developed which fixes the relative position of the sound source and the ear. This system ensures that sounds at the same level may be presented during different test sessions. To assess the stability of ultra-high-frequency thresholds, normal hearing subjects were tested in sessions separated by several weeks. Thresholds were obtained between 1 and 16 kHz and were found to be reliable. It is concluded that this type of system can be used for monitoring the ototoxic effects of drug therapy.

Acoustics↗

Cutaneous reactions of alloxan diabetic rats to local thermal trauma.

Thermal injury was induced on the external ears of nondiabetic and untreated alloxan diabetic rats of various ages. The skin reaction (erythema and necrosis) was assessed by naked eye inspection, 1, 7, 14 and 21 days after injury. Erythema was found to be more intense in young than in old controls after 1 and 7 days. The late erythematous reaction was more pronounced in short-term diabetic animals than in controls of the same age, indicating that the diabetic metabolic derangement per se alters the reaction. In addition, long-term diabetic rats had a markedly increased skin redness after 1, 7, 14 and 21 days when compared with controls of the same age. Thus, long-term diabetes enhances the erythematous reaction. As to the extent of necrosis, there was no significant differences between the experimental groups. There was, however, a tendency for an increased amount in the long-term when compared with the short-term diabetic rats.

Age Factors↗

Colobomatous microphthalmia, heart disease, hearing loss, and mental retardation--a syndrome.

A syndrome consisting of colobomatous microphthalmia, heart disease, abnormalities of the external ear with associated hearing loss, and mental retardation is described. Nine children and one adult were evaluated. There is not race or sex predilection. The syndrome can be heritable, as shown by a mother and daughter who were among the patients. In addition to the four major components enumerated, multiple other anomalies may be associated. In some cases, the syndrome may occur incompletely. Whenever two or more of the four components are recognized, the other systems usually affected should be investigated.

Abnormalities, Multiple↗

Facial fuzz and funny findings. Facial hair causing otalgia and oropharyngeal pain.

Three patients with referred otalgia and/or oropharyngeal pain due to ectopic facial hair found in either the external auditory canal or oropharynx were treated at the Cleveland Clinic Foundation, Department of Otolaryngology and Communicative Disorders. In each of these patients, annoying symptoms were relieved by simple removal of the misplaced facial hair. Sensory nerve innervation of the external ear and oropharynx and their interrelationship in referred pain are described in detail following the case reports.

Adult↗

Townes-Brocks syndrome.

Townes-Brocks syndrome (TBS) is an autosomal dominant disorder with multiple malformations and variable expression. Major findings include external ear anomalies, hearing loss, preaxial polydactyly and triphalangeal thumbs, imperforate anus, and renal malformations. Most patients with Townes-Brocks syndrome have normal intelligence, although mental retardation has been noted in a few.

Abnormalities, Multiple↗

[Syndrome of the 1st and 2nd branchial arches. Report of 3 cases].

Three newborn babies were studied in our hospital with the following associated unilateral deformities: macrostomia, hemignathia and anomalies of the external ear. The main symptom was the inability for suction causing some weight loss. These cases could be logically named "syndrome of the first and second branchial arches" because of their common origin. We discuss the pathogenesis and the differences with other diseases including those of hereditary origin. The initial management is discussed.

Abnormalities, Multiple↗

Self-mutilation in mice associated with otitis media.

Mutilation of the external ear and adjacent tissues was an enzootic problem in outbred Mcr:(ICR) breeder mice in a commercial colony. Necropsy examination of affected and randomly selected, nonaffected adult breeder mice revealed a probable association of the cutaneous lesions with suppurative otitis media. Several microorganisms, including Mycoplasma pulmonis and Pasteurella pneumotropica, were isolated from the tympanic bullae.

Animals↗

[The reconstruction of the conchae auriculae in congenital and acquired defects in children].

The article is concerned with the method of reconstruction of auricles in 53 patients aged from 6 to 15 years. Before 1994 the reconstructing operations were performed by the N. I. Iarchuk method (19 patients) while during the recent 5 years the E. Joganic method has been used (34 patients). Using the total autocostal cartilage for the formation of the external ear basis is thought by the authors to be the preferable method as compared with the transplantation of the allogenic tissues.

Adolescent↗

Management of the preauricular sinus.

The preauricular sinus is a relatively common physical finding especially in the pediatric population. It is defined as a congenital lesion in which a small skin opening located in front of the external ear communicates with a subcutaneous network of cysts. The vast majority are benign in nature and require no intervention. Draining sinus tracts are prone to infection and should be excised. Complete excision of the pit and sinus tract provides the only definitive cure. To prevent problematic recurrence, we recommend wide exposure of the lesion by the technique described.

Branchial Region↗

[Barotrauma in diving].

Boyle's law is important for the understanding of the pathophysiology of barotrauma. Barotrauma--any disease or injury due to unequal pressures between a hollow space inside the body and the ambient pressure, or between two spaces within the body; examples include arterial gas embolism, pneumomediastinum, and pneumothorax. It may affect the lungs, the middle ear, the inner ear, the external ear, the eyes, the teeth, the gastrointestinal tract, the skin. Symptoms remain the same whether it all happens during ascent or descent.

Barotrauma↗

[Hypomelanosis of Ito: report of one case (author's transl)].

A case of hypomelanosis of Ito in a 22-month-old North African girl is reported. The family history is negative for skin disease. Whorls of depigmentation are present on the trunk and abdomen. Associated defects include: growth retardation, microcephaly, strabismus, bilateral epicanthus, myopia, hypertelorism and abnormalities of external ear. Associated non-cutaneous abnormalities documented in patients with hypomelanosis of Ito are reviewed.

Child, Preschool↗

Beals-Hecht syndrome.

Beals-Hecht syndrome, also known as congenital contractural arachnodactyly, is caused by a defect in fibrillin as in Marfan syndrome. This syndrome is characterized by a multitude of clinical findings including arachnodactyly, narrow body habitus, scoliosis, congenital contractures, and external ear deformities. Restrictive lung disease may be associated with the severe scoliosis and thoracic cage abnormalities in this syndrome. We describe a child with Beals-Hecht syndrome and review the literature.

Child↗