Inner ear dysplasia with agenesis of the corpus callosum

Lee P. Smith, Kian Karimi, Simon I. Angeli

Research output: Contribution to journalArticlepeer-review

1 Scopus citations


In this case report we describe a patient with agenesis of the corpus callosum and asymmetric bilateral sensorineural hearing loss, who was found to have a bilateral inner ear dysplasia. Both of these anomalies have been associated with different members of the Pax gene family. However, to our knowledge this is the first report demonstrating the coexistence of these two unusual entities. By uncovering the inner ear malformation, we are able to better counsel this patient with regard to avoidance of high-risk activities such as contact sports and recommend prophylactic vaccination for meningitis prevention.

Original languageEnglish (US)
Pages (from-to)1985-1988
Number of pages4
JournalInternational Journal of Pediatric Otorhinolaryngology
Issue number11
StatePublished - Nov 2006


  • Cochlear dysplasia
  • Corpus callosum

ASJC Scopus subject areas

  • Otorhinolaryngology
  • Pediatrics, Perinatology, and Child Health
  • Pulmonary and Respiratory Medicine
  • Surgery


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