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Anomalies of the ear in the Pierre Robin triad

Philipp M Gruen1, Alfonso Carranza, Collin S Karmody

  • 1Department of Otorhinolaryngology, University of Ulm, Ulm, Germany.

The Annals of Otology, Rhinology, and Laryngology
|September 30, 2005
PubMed

Insights

The Pierre Robin triad (PRT) causes numerous ear anomalies, including middle ear infections and inner ear malformations. These findings link PRT to early branchial arch development.

Area of Science:

  • Otolaryngology
  • Developmental Biology
  • Genetics

Background:

  • The Pierre Robin triad (PRT) is a congenital condition characterized by micrognathia-retrognathia, glossoptosis, and palate abnormalities.
  • Previous temporal bone studies on PRT have provided foundational insights into its craniofacial and otic manifestations.

Purpose of the Study:

  • To conduct a detailed temporal bone analysis in infants with PRT.
  • To identify and compare otic anomalies in PRT cases with prior research.
  • To elucidate the developmental origins of PRT-associated ear malformations.

Main Methods:

  • Histopathological examination of 13 temporal bones from 7 infants diagnosed with PRT.
  • Light microscopy analysis of 20-micrometer thick sections.
  • Correlation of temporal bone findings with clinical data, including gestational age and signs of hypoxia.

Main Results:

  • Widespread architectural anomalies of the entire ear were observed.
  • Common findings included abnormal auricles, ossicular anomalies (e.g., abnormal stapes footplates), and frequent middle ear infections.
  • Inner ear anomalies included lateral semicircular canal aplasia, large vestibular aqueduct, and abnormal otoconia.
  • Mastoid process abnormalities included cartilage remnants and fallopian canal dehiscence.
  • Cochlear hair cell loss was associated with antemortem hypoxia.

Conclusions:

  • The observed ear anomalies in PRT are predominantly linked to developmental processes of the first and second branchial arches.
  • Genetic factors influencing branchial arch development are implicated in the pathogenesis of PRT.
  • These findings underscore the complex interplay between craniofacial development and otic structures in PRT.
Abstract

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