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Enlarged vestibular aqueduct in two male siblings

Published online by Cambridge University Press:  10 October 2002

C M Varghese ,
P Scampion ,
V K Das ,
J Gillespie  and
D Umapathy
C M Varghese
Affiliation:
Children's Centre, Bolton, UK.
P Scampion
Affiliation:
Children's Centre, Bolton, UK.
V K Das
Affiliation:
Manchester Royal Infirmary, Manchester, UK.
J Gillespie
Affiliation:
Manchester Royal Infirmary, Manchester, UK.
D Umapathy
Affiliation:
Children's Centre, Bolton, UK.
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Abstract

This report describes the case history of two male siblings with sensorineural hearing loss and an enlarged vestibular aqueduct (EVA). Sibling 1 presented with a history of intermittent self-limiting ataxia and hearing loss at the age of 25 months and sibling 2 presented with a similar history at the age of 18 months. MRI showed an enlarged endolymphatic duct and sac bilaterally in both children. Perchlorate discharge tests were positive in both infants leading to a diagnosis of Pendred syndrome. A number of conditions associated with EVA are discussed with a view to devising management strategies.

Type
Case Reports
Information
Developmental Medicine and Child Neurology , Volume 44 , Issue 10 , October 2002 , pp. 706 - 711
Copyright
© 2002 Mac Keith Press

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