Chudley McCullough syndrome

Childs Nerv Syst. 2008 May;24(5):541-4. doi: 10.1007/s00381-007-0518-z. Epub 2007 Oct 26.


Background: Chudley McCullough syndrome is characterized by partial agenesis of the corpus callosum, interhemispheric cyst, cerebral and cerebellar cortical dysplasias, and hydrocephalus. This syndromic form of sensorineural hearing loss is rare. Our literature search has located 13 siblings in 6 families with this syndrome. We report a case of Chudley McCullough syndrome and discuss the relevant literature. It is amply clear from the review of literature that treatment of ventricular dilatation or drainage of arachnoid cysts in these cases will not improve the sensorineural hearing loss.

Case illustration: A 14-month-old female child presented with bilateral profound sensorineural hearing loss. Neuroimaging revealed partial agenesis of the corpus callosum, colpocephaly, and an interhemispheric arachnoid cyst. These associations suggested a diagnosis of Chudley McCullough syndrome.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Arachnoid Cysts / complications*
  • Brain Diseases / complications*
  • Corpus Callosum / pathology*
  • Female
  • Hearing Loss, Sensorineural / complications*
  • Hearing Loss, Sensorineural / pathology*
  • Humans
  • Hydrocephalus / complications*
  • Infant
  • Magnetic Resonance Imaging