The oto-vertebral syndrome

The oto-vertebral syndrome is a rare combination of malformations of the ears, spinal anomalies, and congenital heart disease. The syndrome appears to be caused by early embryonic exogenous damage during the 6th or 7th week of embryonic development. The case is reported of a 28-year-old man with bil...

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Bibliographic Details
Published inSchweizerische medizinische Wochenschrift Vol. 108; no. 42; p. 1638
Main Authors Hess, O M, Steurer, J, Kuhlmann, U, Krayenbühl, H P
Format Journal Article
LanguageGerman
Published Switzerland 21.10.1978
Subjects
Abnormalities, Multiple
Adult
Ear Canal - abnormalities
Ear Canal - surgery
Ear, External - abnormalities
Heart Defects, Congenital
Humans
Jaw Abnormalities
Male
Mandible - abnormalities
Physical Therapy Modalities
Scoliosis - congenital
Scoliosis - therapy
Syndrome
Thoracic Vertebrae - abnormalities
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Summary:The oto-vertebral syndrome is a rare combination of malformations of the ears, spinal anomalies, and congenital heart disease. The syndrome appears to be caused by early embryonic exogenous damage during the 6th or 7th week of embryonic development. The case is reported of a 28-year-old man with bilateral aplasia of the external ear, bilateral aplasia of the ear canal, hypoplasia of the mandibula, severe thoracic scoliosis, and ventricular septal defect with pulmonary hypertension. He was admitted with dyspnea on exertion, syncope, and severe cyanosis. Cardiac catheterization revealed severe pulmonary hypertension with moderate right-to-left and slightly left-to-right shunt (Eisenmenger syndrome). Right and left ventricular function, as evaluated by angiocardiography, was slightly reduced. Because of the severe hemodynamic alterations, symptomatic therapy with digitalis, repeated venesection, and anticoagulation was initiated.
ISSN:0036-7672