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J Am Acad Audiol 1999; 10(08): 436-444
DOI: 10.1055/s-0042-1748517
Original Article

Otoacoustic Emission Findings in Rett Syndrome

Sarah Nicholas
Department of Speech Pathology and Audiology, The University of Queensland, Brisbane, Australia
,
Joseph Kei
Department of Speech Pathology and Audiology, The University of Queensland, Brisbane, Australia
,
Gail Woodyatt
Department of Speech Pathology and Audiology, The University of Queensland, Brisbane, Australia
,
Bradley McPherson
Department of Speech Pathology and Audiology, The University of Queensland, Brisbane, Australia
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Abstract

Rett syndrome is a neurologic disorder affecting mainly females after a seemingly normal 6 to 18 months of life. The resulting developmental disabilities include apparent dementia and loss of acquired language, social skills, and purposeful hand use. The present investigation assessed 10 individuals with Rett syndrome and a control group matched for age and sex. The present study aimed to determine the clinical feasibility of obtaining otoacoustic emissions (OAEs) from the Rett syndrome group and to compare the characteristics of the transient evoked and distortion-product OAEs obtained from the two subject groups. Results indicated that OAE evaluation is a clinically feasible method of assessing individuals with Rett syndrome. The Rett syndrome group had less robust OAEs, especially in the higher frequencies, when compared to the control group. Seven of the Rett syndrome group were identified as having reduced or absent OAEs in at least one ear. These findings suggest a need for hearing screening at an early age and monitoring of hearing on a regular basis.

Abbreviations: ABR = auditory brainstem response, DPOAEs = distortion-product otoacoustic emissions, LVR = late vertex response, MLR = middle latency response, OAE = otoacoustic emission, SNR = signal-to-noise ratio, TEOAEs = transient evoked otoacoustic emissions, VRA = visual reinforcement audiometry, WWR = whole-wave reproducibility

Key Words

Hearing impairment - otoacoustic emissions - Rett syndrome


Publication History

Article published online:
03 May 2022

© 1999. American Academy of Audiology. This article is published by Thieme.

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  • REFERENCES

  • Bader G, Witt-Engerstrom I, Hagberg B. (1989). Neurophysiological findings in the Rett syndrome, II: visual and auditory brainstem, middle and late evoked responses. Brain Dev 11: 110–114.
  • Baldwin M, Watkin P. (1992). The clinical application of oto-acoustic emissions in paediatric audiological assessment. J Laryngol Otol 106: 301–306.
  • Bonfils P, Bertrand Y, Uziel A. (1988). Evoked otoacoustic emissions: normative data and presbycusis. Audiology 27: 27–35.
  • Coleman M, Brubaker J, Hunter K, Smith G. (1988). Rett syndrome: a survey of North American patients. J Ment Defic Res 32: 117–124.
  • Gaskill SA, Brown AM. (1990). The behaviour of the acoustic distortion product 2f1-f2 from the human ear and its relation to auditory sensitivity. J Acoust Soc Am 88: 821–839.
  • Gorga MP, Worthington DW, Reiland JK, Beauchaine KA, Goldgar DE. (1985). Some comparisons between auditory brainstem response threshold, latencies, and the pure-tone audiogram. Ear Hear 6: 105–112.
  • Hagberg B, Hagberg G. (1997). Rett syndrome: epidemiology and geographical variability. Eur Child Adolesc Psychiatry 6 (Suppl l): 5–7.
  • Hagberg B, Aicardi J, Dias Κ, Ramos Ο. (1983). A progressive syndrome of autism, dementia, ataxia and loss of purposeful hand use in girls: Rett syndrome: report of 35 cases. Ann Neurol 14: 471–479.
  • Haggard M, Hughes E. (1991). Screening Children's Hearing: A Review of the Literature and the Implications of Otitis Media. London: HMSO.
  • Hanks SB. (1990). Motor disabilities in the Rett syndrome and physical therapy strategies. Brain Dev 12: 157–161.
  • Harris FP, Lonsbury-Martin BL, Stagner BB, Coats AC, Martin GK. (1989). Acoustic distortion products in humans: systematic changes in amplitudes as a function of f2/f1 ratio. J Acoust Soc Am 85: 220–229.
  • Hotz MA, Harris FP, Probst R. (1994). Otoacoustic emissions: an approach for monitoring aminoglycoside-induced ototoxicity. Laryngoscope 104: 1130–1134.
  • Jacobson JT, Jacobson CA. (1994). The effects of noise in transient EOAE newborn hearing screening. Int J Pediatr Otorhinolaryngol 29: 235–248.
  • Jerger J. (1970). Clinical experience with impedance audiometry. Arch Otolaryngol Head Neck Surg 92: 311–324.
  • Kei J, McPherson B, Smyth V, Latham S, Loscher J. (1997). Transient evoked otoacoustic emissions in infants: effects of gender, ear asymmetry and activity status. Audiology 36: 61–71.
  • Kemp DT. (1997). Otoacoustic emissions in perspective. In: Robinette MS, Glattke TJ, eds. Otoacoustic Emissions. New York: Thieme, 1–21.
  • Lenn NJ, Olsho LW, Turk WR. (1986). Auditory processing deficit in a patient with Rett syndrome. Am J Med Genet 24: 153–156.
  • Naidu S, Murphy M, Moser HW, Rett A. (1986). Rett syndrome: natural history in 70 cases. Am J Med Genet 24(Suppl l): 61–72.
  • Norton SJ, Rubel EW. (1990). Active and passive ADP components in mammalian and avian ears. In: Dallos P, Geisler CD, Matthews JW, Ruggero MA, Steele CR, eds. The Mechanics and Biophysics of Hearing. Berlin: Springer-Verlag, 219–226.
  • Nozza RJ, Sabo DL, Mandel EM. (1997). A role for otoacoustic emissions in screening for hearing impairment and middle ear disorders in school-aged children. Ear Hear 18: 227–239.
  • Owens JJ, McCoy MJ, Lonsbury-Martin BL, Martin GK. (1992). Influence of otitis media on evoked otoacoustic emissions in children. Semin Hear 13: 53–66.
  • Pelson R, Budden S. (1987). Auditory brainstem response findings in Rett syndrome. Brain Dev 9: 514–516.
  • Rett A. (1966). Ueber ein eigen artiges hirnatrophisches Syndrom bei Hyperammonaemie im Kindersalter. Weiner Medizinische Wochenschrift 116: 723–738.
  • Rhoades K, McPherson Β, Smyth V, Kei J. (1998). Effects of background noise on click-evoked otoacoustic emissions. Ear Hear 19: 450–462.
  • Salamy A, Eldredge L, Sweetow R. (1996). Transient evoked otoacoustic emissions: feasibility in the nursery Ear Hear 17: 42–48.
  • Schanen C, Francke U. (1998). A severely affected male born into a Rett syndrome kindred supports X-linked inheritance and allows extension of the exclusion map. Am J Hum Genet 63: 267–269.
  • Stach Β, Stoner W, Smith S, Jerger J. (1994). Auditory evoked potentials in Rett syndrome. J Am Acad Audiol 5: 226–230.
  • van der Drift JFC, Brocaar MP, van Zanten GA. (1987). The relationship between the pure-tone audiogram and the click auditory brainstem response threshold in cochlear hearing loss. Audiology 26: 1–10.
  • Vernon M. (1970). Clinical phenomenon of cerebral palsy and deafness. Except Child 36: 743–751.
  • Woodyatt GC, Murdoch B. (1996). The effect of the presentation of visual and auditory stimuli on the breathing patterns of two girls with Rett syndrome. J Intellect Disabil Res 40: 252–259.
  • Woodyatt GC, Ozanne AE. (1993). A longitudinal study of cognitive skills and communication behaviours in children with Rett syndrome. J Intellect Disabil Res 37: 419–435.