Patterns of anomalies in children with malformed ears

Isabelle Rapin, Robert J. Ruben

Research output: Contribution to journalArticle

26 Citations (Scopus)

Abstract

Sixteen children with anomalies of the auricle and/or middle ear who presented malformations of the face, mouth, upper airway, spine, limbs, heart, gastrointestinal (GI), and/or genitourinary (GU) systems, were described. While clusters of anomalies suggested syndromes such as the oculo-auriculo-vertebral syndrome of Goldenhar, hemifacial microsomia, mandibulo-facial dysostosis (Treacher Collins syndrome), Pierre Robin, Klippel-Feil, Moebius, Duane, and/or VATER syndromes, many children did not fit what are usually considered even minimal criteria for these syndromes. Several children had malformations which fit the description of more than one syndrome. The importance of investigating the children for unsuspected anomalies, especially of the GU system, was emphasized. Life threatening problems in this group consisted of airway problems, congenital heart disease, and major anomalies of the GI and GU systems. Better management of sucking, Swallowing and airway problems might have decreased the early morbidity and mortality (3/16) in this group. Children with multiple defacing anomalies may not be mentally retarded so that aggressive management of their visceral anomalies and hearing problems, and early educational intervention are mandatory. Delay in development may be due to hearing loss, vestibular impairment, ataxia, the consequences of early malnutrition, and multiple hospitalizations rather than to mental retardation. A pessimistic attitude in infancy is unwarranted since it is impossible to predict which children will end up competitive individuals.

Original languageEnglish (US)
Pages (from-to)1469-1502
Number of pages34
JournalLaryngoscope
Volume86
Issue number10
StatePublished - 1976

Fingerprint

Ear
Urogenital System
Goldenhar Syndrome
Mandibulofacial Dysostosis
Dysostoses
Songbirds
Mentally Disabled Persons
Middle Ear
Ataxia
Deglutition
Hearing Loss
Malnutrition
Intellectual Disability
Hearing
Mouth
Heart Diseases
Hospitalization
Spine
Extremities
Morbidity

ASJC Scopus subject areas

  • Otorhinolaryngology

Cite this

Patterns of anomalies in children with malformed ears. / Rapin, Isabelle; Ruben, Robert J.

In: Laryngoscope, Vol. 86, No. 10, 1976, p. 1469-1502.

Research output: Contribution to journalArticle

Rapin, I & Ruben, RJ 1976, 'Patterns of anomalies in children with malformed ears', Laryngoscope, vol. 86, no. 10, pp. 1469-1502.
Rapin, Isabelle ; Ruben, Robert J. / Patterns of anomalies in children with malformed ears. In: Laryngoscope. 1976 ; Vol. 86, No. 10. pp. 1469-1502.
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