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NEUROLOGY 2004;63:385-387
© 2004 American Academy of Neurology
Brief Communications

Deterioration of the auditory brainstem response in children with type 3 Gaucher disease

Pauline E. Campbell, BSc, Chris M. Harris, PhD and Ashok Vellodi, FRCP MRCP

From the SensoriMotor Laboratory (P. Campbell and Dr. Harris), Centre for Theoretical and Computational Neuroscience, Plymouth University, and Audiology Department (P. Campbell), Derriford Hospital, Plymouth, and Metabolic Medicine Unit (Dr. Vellodi), Great Ormond Street Hospital, London, UK.

Address correspondence and reprint requests to P. Campbell, Centre for Theoretical and Computational Neuroscience, Plymouth University, Plymouth, PL4 8AA, UK; e-mail: pauline.campbell{at}plymouth.ac.uk

Enzyme replacement therapy (ERT) has improved the quality of life in patients with Gaucher disease (GD). An emerging concern is whether ERT can also halt the neurologic progression in type 3 GD. The authors examined the auditory brainstem response (ABR) in eight children with type 3 GD undergoing high-dose ERT and found a consistent deterioration in ABR response. They conclude that ERT does not halt brainstem degeneration and that alternative therapies must be sought.

Received January 26, 2004. Accepted in final form March 2, 2004.




This article has been cited by other articles:


Home page
 J Child NeurolHome page
C. M. Harris and P. Campbell
The Importance of Correct Phenotyping in Gaucher Disease
J Child Neurol, August 1, 2007; 22(8): 1056 - 1057.
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