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. 1988 Jul;43(1):46–51.

Auditory brain-stem responses in the fragile X syndrome.

T Arinami 1, M Sato 1, S Nakajima 1, I Kondo 1
PMCID: PMC1715284  PMID: 3376943

Abstract

Auditory brain-stem responses (ABRs) were recorded from a group of 12 mentally retarded males with the fragile X (fra[X]). The responses were analyzed in terms of ABR thresholds, absolute latencies, and interpeak latencies. One patient had increased ABR thresholds, indicating hearing impairment. Five fra(X) subjects had prolonged I-V interpeak latencies. Comparisons between the fra(X) group (excluding one possible hard-of-hearing subject) and a control group of age-matched males with normal intelligence showed that the fra(X) group's interpeak latencies were significantly prolonged for the III-V and I-V but not for the I-III. This pattern of prolongation of interpeak latencies suggests that central, as opposed to peripheral, nervous-system dysfunction predominates in many patients having this syndrome. In addition, frequently observed prolongation of the transmission time may indicate that brain-stem white-matter functioning is also apt to be involved in this syndrome.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Aguglia U., Farnarier G., Tinuper P., Quattrone A. Brainstem auditory evoked responses in Lafora disease. Clin Electroencephalogr. 1985 Oct;16(4):202–207. doi: 10.1177/155005948501600407. [DOI] [PubMed] [Google Scholar]
  2. Arinami T., Kondo I., Nakajima S. Frequency of the fragile X syndrome in Japanese mentally retarded males. Hum Genet. 1986 Aug;73(4):309–312. doi: 10.1007/BF00279092. [DOI] [PubMed] [Google Scholar]
  3. Chiappa K. H., Harrison J. L., Brooks E. B., Young R. R. Brainstem auditory evoked responses in 200 patients with multiple sclerosis. Ann Neurol. 1980 Feb;7(2):135–143. doi: 10.1002/ana.410070208. [DOI] [PubMed] [Google Scholar]
  4. Davis S. L., Aminoff M. J., Berg B. O. Brain-stem auditory evoked potentials in children with brain-stem or cerebellar dysfunction. Arch Neurol. 1985 Feb;42(2):156–160. doi: 10.1001/archneur.1985.04060020070019. [DOI] [PubMed] [Google Scholar]
  5. Folsom R. C., Weber B. A., Thompson G. Auditory brainstem responses in children with early recurrent middle ear disease. Ann Otol Rhinol Laryngol. 1983 May-Jun;92(3 Pt 1):249–253. doi: 10.1177/000348948309200307. [DOI] [PubMed] [Google Scholar]
  6. Garg B. P., Markand O. N., Bustion P. F. Brainstem auditory evoked responses in hereditary motor-sensory neuropathy: site of origin of wave II. Neurology. 1982 Sep;32(9):1017–1019. doi: 10.1212/wnl.32.9.1017. [DOI] [PubMed] [Google Scholar]
  7. Gillberg C., Persson E., Wahlström J. The autism-fragile-X syndrome (AFRAX): a population-based study of ten boys. J Ment Defic Res. 1986 Mar;30(Pt 1):27–39. doi: 10.1111/j.1365-2788.1986.tb01295.x. [DOI] [PubMed] [Google Scholar]
  8. Hagerman R. J., Altshul-Stark D., McBogg P. Recurrent otitis media in the fragile X syndrome. Am J Dis Child. 1987 Feb;141(2):184–187. doi: 10.1001/archpedi.1987.04460020074031. [DOI] [PubMed] [Google Scholar]
  9. Hecox K. E., Cone B., Blaw M. E. Brainstem auditory evoked response in the diagnosis of pediatric neurologic diseases. Neurology. 1981 Jul;31(7):832–840. doi: 10.1212/wnl.31.7.832. [DOI] [PubMed] [Google Scholar]
  10. Hecox K., Burkard R. Developmental dependencies of the human brainstem auditory evoked response. Ann N Y Acad Sci. 1982;388:538–556. doi: 10.1111/j.1749-6632.1982.tb50815.x. [DOI] [PubMed] [Google Scholar]
  11. Lenhardt M. L., Shaia F. T., Abedi E. Brain-stem evoked response waveform variation associated with recurrent otitis media. Arch Otolaryngol. 1985 May;111(5):315–316. doi: 10.1001/archotol.1985.00800070067009. [DOI] [PubMed] [Google Scholar]
  12. Loesch D. Z., Hay D. A., Sutherland G. R., Halliday J., Judge C., Webb G. C. Phenotypic variation in male-transmitted fragile X: genetic inferences. Am J Med Genet. 1987 Jun;27(2):401–417. doi: 10.1002/ajmg.1320270219. [DOI] [PubMed] [Google Scholar]
  13. Markand O. N., Garg B. P., DeMyer W. E., Warren C., Worth R. M. Brain stem auditory, visual and somatosensory evoked potentials in leukodystrophies. Electroencephalogr Clin Neurophysiol. 1982 Jul;54(1):39–48. doi: 10.1016/0013-4694(82)90229-2. [DOI] [PubMed] [Google Scholar]
  14. Nielsen K. B. Diagnosis of the fragile X syndrome (Martin-Bell syndrome). Clinical findings in 27 males with the fragile site at Xq28. J Ment Defic Res. 1983 Sep;27(Pt 3):211–226. doi: 10.1111/j.1365-2788.1983.tb00293.x. [DOI] [PubMed] [Google Scholar]
  15. Ochs R., Markand O. N., DeMyer W. E. Brainstem auditory evoked responses in leukodystrophies. Neurology. 1979 Aug;29(8):1089–1093. doi: 10.1212/wnl.29.8.1089. [DOI] [PubMed] [Google Scholar]
  16. Rhoads F. A. X-linked mental retardation and fragile-X or marker-X syndrome. Pediatrics. 1982 May;69(5):668–669. [PubMed] [Google Scholar]
  17. Rudelli R. D., Brown W. T., Wisniewski K., Jenkins E. C., Laure-Kamionowska M., Connell F., Wisniewski H. M. Adult fragile X syndrome. Clinico-neuropathologic findings. Acta Neuropathol. 1985;67(3-4):289–295. doi: 10.1007/BF00687814. [DOI] [PubMed] [Google Scholar]
  18. Satya-Murti S., Cacace A. T., Hanson P. A. Abnormal auditory evoked potentials in hereditary motor-sensory neuropathy. Ann Neurol. 1979 May;5(5):445–448. doi: 10.1002/ana.410050506. [DOI] [PubMed] [Google Scholar]
  19. Schiff J. A., Cracco R. Q., Cracco J. B. Brainstem auditory evoked potentials in Guillain-Barré syndrome. Neurology. 1985 May;35(5):771–773. doi: 10.1212/wnl.35.5.771. [DOI] [PubMed] [Google Scholar]
  20. Turner G., Robinson H., Laing S., Purvis-Smith S. Preventive screening for the fragile X syndrome. N Engl J Med. 1986 Sep 4;315(10):607–609. doi: 10.1056/NEJM198609043151002. [DOI] [PubMed] [Google Scholar]
  21. Webb T. P., Bundey S., Thake A., Todd J. The frequency of the fragile X chromosome among schoolchildren in Coventry. J Med Genet. 1986 Oct;23(5):396–399. doi: 10.1136/jmg.23.5.396. [DOI] [PMC free article] [PubMed] [Google Scholar]
  22. Wisniewski K. E., French J. H., Fernando S., Brown W. T., Jenkins E. C., Friedman E., Hill A. L., Miezejeski C. M. Fragile X syndrome: associated neurological abnormalities and developmental disabilities. Ann Neurol. 1985 Dec;18(6):665–669. doi: 10.1002/ana.410180607. [DOI] [PubMed] [Google Scholar]

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