An unusual case of X-linked adrenoleukodystrophy with auditory processing difficulties as the first and sole clinical manifestation

Journal of the American Academy of Audiology

Volumn 15, Issue 2, 2004, Pages 152-160

  Bamiou, Doris Eva ^a   Davies, Rosalyn ^a   Jones, Steve ^a   Musiek, Frank E ^b   Rudge, Peter ^a   Stevens, John ^a   Luxon, Linda M ^a  

^a NATIONAL HOSPITAL FOR NEUROLOGY AND NEUROSURGERY   (United Kingdom)

^b UNIVERSITY OF CONNECTICUT   (United States)

Author keywords

Adrenoleukodystrophy; Auditory processing; Brainstem; Central auditory disorders; Corpus callosum

Indexed keywords

ACOUSTIC IMPEDANCE; ACOUSTIC REFLEX; ADRENOLEUKODYSTROPHY; ADULT; ARTICLE; AUDITORY NERVOUS SYSTEM; CASE REPORT; CORPUS CALLOSUM; DICHOTIC LISTENING; EVOKED BRAIN STEM AUDITORY RESPONSE; GENETIC LINKAGE; GENETICS; HUMAN; LANGUAGE DISABILITY; MALE; NUCLEAR MAGNETIC RESONANCE IMAGING; PATHOLOGY; PURE TONE AUDIOMETRY; SPONTANEOUS OTOACOUSTIC EMISSION;

ACOUSTIC IMPEDANCE TESTS; ADRENOLEUKODYSTROPHY; ADULT; AUDIOMETRY, PURE-TONE; AUDITORY PATHWAYS; CORPUS CALLOSUM; DICHOTIC LISTENING TESTS; EVOKED POTENTIALS, AUDITORY, BRAIN STEM; HUMANS; LANGUAGE DEVELOPMENT DISORDERS; LINKAGE (GENETICS); MAGNETIC RESONANCE IMAGING; MALE; OTOACOUSTIC EMISSIONS, SPONTANEOUS; REFLEX, ACOUSTIC;

EID: 3042621841     PISSN: 10500545     EISSN: None     Source Type: Journal    
DOI: 10.3766/jaaa.15.2.5     Document Type: Article

References (23)

1. British Society of Audiology. (1981). Recommended procedure for pure tone audiometry using a manually operated instrument. Br J Audiol 15:213-216.
2. Cavaletti G, Boglium G, Tredici G. (1990). Adrenoleukodystrophy. Report of a case with extremely slow progression of symptoms. Acta Neurol 12:109-114.
3. Cohen M, Prasher D. (1988). ABR and ART in neurootological diagnosis. Scand Audiol 17:153-162.
4. Jacobson GP, Jacobson JT, Ramadan N, Hyde M. (1994). Auditory brainstem response measures in acoustic nerve and brainstem disease. In: Jacobson JT, ed. Principles and Applications in Auditory Evoked Potentials. London: Allyn and Bacon.
5. Kaga K, Tokoro Y, Tanaka Y, Ushizima H. (1980). The progress of adrenoleukodystrophy as revealed by auditory brainstem evoked responses and brainstem histology. Arch Otol Rhinol Laryngol 228:17-27.
6. Kemp DT, Ryan S, Bray P. (1990). A guide to the effective use of Otoacoustic Emissions. Ear Hear 11:93-105.
7. Korenke GC, Pouwels PJ, Frahm J, Hunneman DH, Stoeckler S, Krasemann E, Jost W, Hanefeld F. (1996). Arrested cerebral adrenoleukodystrophy: A clinical and proton magnetic resonance spectroscopy study in three patients. Pediatr Neurol 15:103-107.
8. Loes DJ, Hite S, Moser H, Stillman AE, Shapiro E, Lockman L, Latchaw RE, Krivit W. (1994). Adrenoleukodystrophy: A scoring method for brain MR observations. Am J Neuroradiol 15:1761-1766.
9. Migeon BR, Moser HW, Moser AB, Axelman J, Sillence D, Norum RA. (1981). Adrenoleukodystrophy: Evidence for X linkage, inactivation, and selection favoring the mutant allele in heterozygous cells. Proc Natl Acad Sci USA 78:5066-5070.
10. Möller AR. (1994). Neural generators of auditory evoked potentials. Semin Hear 19:11-27.
11. Moser HW, Loes DJ, Melhem ER, Raymond GV, Bezman L, Cox CS, Lu S. (2000). X-linked Adrenoleukodystrophy: Overview and prognosis as a function of age and brain magnetic resonance imaging abnormality. A study involving 372 patients. Neuropediatrics 31:227-239.
12. Moser HW, Smith KD, Watkins PA, Powers J, Moser AB. (2001). X-linked Adrenoleukodystrophy. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The Metabolic and Molecular Bases of Inherited Disease. 8th edition. New York: McGraw Hill.
13. Mosser J, Lutz Y, Stoeckel ME, Sarde CO, Kretz C, Douar AM, Lopez J, Aubourg P, Mandel JL. (1994). The gene responsible for adrenoleukodystrophy encodes a peroxisomal membrane protein. Hum Mol Genet 3:265-271.
14. Musiek FE. (1983). Assessment of central auditory dysfunction: The Dichotic Digit Test revisited. Ear Hear 4:79-83.
15. Musiek FE. (1994). Frequency (pitch) and duration pattern tests. J Am Acad Audiol 5:265-268.
16. Musiek FM, Baran JA, Pinheiro ML. (1994). NeuroAudiology: Case Studies. San Diego: Singular Publishing Group.
17. Musiek FE, Pinheiro ML. (1985). Dichotic speech tests in the detection of central auditory dysfunction. In: Musiek FE, Pinheiro ML. Assessment of Central Auditory Dysfunction: Foundations and Clinical Correlates. Baltimore: Wakerly Press.
18. Oka A, Saito M, Kubota M, Sakakihara Y, Yanagisawa M. (1996). Temporary improvement of neurological symptoms with gammaglobulin therapy in a boy with adrenoleukodystrophy. Brain Dev 18:119-121.
19. Pinheiro ML, Musiek FE. (1985). Sequencing and temporal ordering in the auditory system. In: Musiek FE, Pinheiro ML. Assessment of Central Auditory Dysfunction: Foundations and Clinical Correlates. Baltimore: Wakerly Press.
20. Schwartz DM, Morris MD, Jacobson JT. (1994). The normal Auditory Brainstem Response and its variants. In: Jacobson JT, ed. Principles and Applications in Auditory Evoked Potentials. London: Allyn and Bacon.
21. Shimizu H, Moser HD, Naidu S. (1988). Auditory brainstem response and audiologic findings in adrenoleukodystrophy: Its variant and carrier. Otolaryngol Head Neck Surg 98:215-220.
22. Snell KB, Mapes FM, Hickman ED, Frisina DR. (2002). Word recognition in competing babble and the effects of age, temporal processing, and absolute sensitivity. J Acoust Soc Am 112:720-727.
23. Webster DB. (1992). An overview of mammalian auditory pathways with an emphasis on humans. In: Webster DB, Popper AN, Fay RR, eds. The Mammalian Auditory Pathway: Neuroanatomy. London: Springer-Verlag.