Restoration of brain stem auditory-evoked potential in maple syrup urine disease

Otology and Neurotology

Volumn 28, Issue 4, 2007, Pages 566-569

  Spankovich, Christopher ^a   Lustig, Lawrence R ^a,b  

^a UNIVERSITY OF CALIFORNIA   (United States)

^b NONE   (United States)

Author keywords

Brain stem auditory evoked potential; Branched chain amino; Glutamate excitotoxicity; Maple syrup urine disease; Newborn infant screening; Sensorineural hearing loss

Indexed keywords

BRANCHED CHAIN AMINO ACID;

ARTICLE; CASE REPORT; CLINICAL FEATURE; CONTROLLED STUDY; DIET THERAPY; DIFFERENTIAL DIAGNOSIS; EVOKED BRAIN STEM AUDITORY RESPONSE; HEARING LOSS; HEMODIALYSIS; HUMAN; MALE; MAPLE SYRUP URINE DISEASE; NEWBORN; OTOACOUSTIC EMISSION; PRIORITY JOURNAL; AUDIOMETRY; DIALYSIS; GENETICS; HOSPITAL INFORMATION SYSTEM; INFANT; NUCLEAR MAGNETIC RESONANCE IMAGING; PATHOPHYSIOLOGY; PHYSIOLOGY;

AUDIOMETRY; DIALYSIS; EVOKED POTENTIALS, AUDITORY, BRAIN STEM; HEARING LOSS; HUMANS; INFANT; MAGNETIC RESONANCE IMAGING; MALE; MAPLE SYRUP URINE DISEASE; POINT-OF-CARE SYSTEMS;

EID: 34447635531     PISSN: 15317129     EISSN: 15374505     Source Type: Journal    
DOI: 10.1097/MAO.0b013e31802fbaa9     Document Type: Article

References (10)

1. Chuang D, Shih V. Maple syrup urine disease. In: Scriver CR, Beaudet AL, Valle DL, Sly WS, eds. The Metabolic and Molecular Bases of Inherited Disease. 8th ed. New York, NY: McGraw-Hill, 2000.
2. Chuang DT, Chuang JL, Wynn RM. Lessons from genetic disorders of branched-chain amino acid metabolism. J Nutr 2006;136:243S-9S.
3. Muller K, Kahn T, Wendel U. Is demyelination a feature of maple syrup urine disease? Pediatr Neurol 1993;9:375-82.
4. Sakai M, Inoue Y, Oba H, et al. Age dependence of diffusion-weighted magnetic resonance imaging findings in maple syrup urine disease encephalopathy. J Comput Assist Tomogr 2005;29:524-7.
5. Patay Z. Diffusion-weighted MR imaging in leukodystrophies. Eur Radiol 2005;15:2284-303.
6. Wendel U, Saudubray JM, Bodner A, et al. Liver transplantation in maple syrup urine disease. Eur J Pediatr 1999;158:S60-4.
7. Geal-Dor M, Adelman C, Levi H, et al. Changes in the auditory nerve brainstem evoked responses in a case of maple syrup urine disease. Dev Med Child Neurol 2004;46:184-6.
8. Tavares RG, Santos CE, Tasca CI, et al. Inhibition of glutamate uptake into synaptic vesicles from rat brain by 3-nitropropionic acid in vitro. Exp Neurol 2001;172:250-4.
9. Puel JL. Chemical synaptic transmission in the cochlea. Prog Neurobiol 1995;47:449-76.
10. Jewett DL, Williston JS. Auditory-evoked far fields averaged from the scalp of humans. Brain 1971;94:681-96.