association of vertebral, anal, cardiac, tracheoesophageal, renal and  limb anomalies (vacterl) is rare anomaly with an incidence of 1.6 per 10000 births. this condition is a combination of anomalies recognized  as a hereditary entity with poor prognosis. herein, we report vacterl association presenting with auricle atresia. a male neonate with a birth weight of 2690 grams, head circumference 3...

Background: Congenital malformations of the auricle are often combined with atresia of the outer ear canal and malformations of the ossicles, representing aesthetic as well as functional deficits. Optimal treatment should therefore address both aspects equally. This report describes a new approach, combining the reconstruction of the auricle with implantation of an active middle ear hearing aid...

Association of vertebral, anal, cardiac, tracheoesophageal, renal and  limb anomalies (VACTERL) is rare anomaly with an incidence of 1.6 per 10000 births. This condition is a combination of anomalies recognized  as a hereditary entity with poor prognosis. Herein, we report VACTERL association presenting with auricle atresia. A male neonate with a birth weight of 2690 grams, head circumference 3...

OBJECTIVES To quantitatively analyzing the anatomic variants on temporal computed tomography (CT) in congenital external auditory canal stenosis (EACS), congenital aural atresia (CAA), and normal ear structure. METHODS Through a retrospective study, we analyzed 142 temporal high-resolution CT studies performed in 71 microtia patients. The following 6 parameters were compared among the three g...

A case of atresia of the aortic orifice with associated hypoplasia of the aorta, patent ductus arteriosus, patent foramen ovale, miniature mitral orifice, rudimentary left ventride, hypertrophy and dilatation of the right auricle, ventride, and pulmonary artery, and hemangiomata of the tricuspid valve has recently been observed in this laboratory. In Abbott's reviewt of 1000 cardiac malformatio...

Congenital aural atresia is the failure of development of the external auditory canal. It usually occurs in conjunction with microtia, which is the malformation of the auricle due to a failure of development of the external ear. Aural atresia, with or without microtia, may significantly affect the hearing and social life of the patients. It is important for every medical practitioner to be awar...

Acquired external auditory canal (EAC) atresia is an infrequent entity which can originate from a number of different causes including trauma, infection, neoplasia, inflammation, and radiotherapy. Posttraumatic atresias are exceptionally rare, only 10% of atresias are attributed to trauma in most of the series. The management of stenosis of the EAC is challenging as it is associated with residu...

Wolfram-like syndrome (WFSL) is a rare autosomal dominant disease characterised by congenital progressive hearing loss, diabetes mellitus, and optic atrophy. The patient was a boy with the juvenile form of diabetes mellitus and findings which clinically matched the symptoms of Wolfram syndrome. At the age of 3 1/4 years, diabetes mellitus was diagnosed in this boy who also had severe psychomoto...

Hemifacial Microsomia (HFM) is a term used to identify facial deformities associated with the development ofthe first and second pairs of branchial arches, characterized by underdevelopment one half face. One typeof hemifacial microsomia oculo-auriculo-vertebral dysplasia or Goldenhar syndrome.The incidence HFM 1:3500-1:7000 live births occurs in 1 case per 1000 children congenitaldeafness. The...

OBJECTIVE To evaluate the relationships between temporal bone abnormalities and the severity of microtia in Japanese patients using objective grading systems. DESIGN Retrospective case series study conducted between 1992 and 2003. SETTING Academic, tertiary care, referral medical center. PATIENTS One hundred forty-two ears of 109 Japanese patients (85 male and 24 female patients; mean age...