Chudley McCullough syndrome.
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.


METHODS
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.
Nadkarni
Trimurti D
TD
Department of Neurosurgery, King Edward Memorial Hospital, Seth G. S. Medical College, Parel, Mumbai 400 012, India. tdnadkarni@hotmail.com
Menon
Ram K
RK
Shah
Abhidha H
AH
Goel
Atul
A
D016080:Arachnoid Cysts / Q000150:complications*; D001927:Brain Diseases / Q000150:complications*; D003337:Corpus Callosum / Q000473:pathology*; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000150:complications* / Q000473:pathology*; D006801:Humans; D006849:Hydrocephalus / Q000150:complications*; D007223:Infant; D008279:Magnetic Resonance Imaging