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Auditory and vestibular findings in Waardenburg's type II syndrome

  • M. Hildesheimer (a1), Z. Maayan (a1), C. Muchnik (a1), M. Rubinstein (a1) and R. M. Goodman (a2)...

A group of 33 patients with the autosomal dominant form of Waardenburg Type II syndrome underwent hearing and vestibular examination. A bilateral, symmetrical, sensorineural hearing loss was found in 51 per cent of the tested subjects. The severity of hearing loss varied from mild to severe. Strong evidence indicated the possible progressive nature of the hearing loss. Concerning the frequency of vestibular findings, the study highlights the frequent divergence between cochlear and vestibular involvement.

Corresponding author
M. Hildesheimer, PhD, Head, School of Communication Disorders, Speech Language and Hearing, Haim Sheba Medical Center, Tel Hashomer, Israel
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Arias, S. (1971). Genetic Heterogeneity in the Waardenburg Syndrome. Birth Defects: Original Article Series 7: 87101.
Anderson, H., Barr, B. and Wedenberg, E. (1969). Intraaural Reflexes in Retro-cochlear Lesions. In: Disorders of the Skull Base Region. (Eds. Hambarger, C. and Wersall, J.). pp. 4955. Almquist and Wikesell, Stockholm.
Bellotto, R., Costantini, F., Gaini, R. and Pignataro, O. (1985). Cochleo-vestibular features in Waardenburg's syndrome. Advances in Audiology 3: 7583.
Goodman, R. M., Levital, I., Solomon, A. and Klein, D. (1981). A Survey of the Klein Waardenburg Syndrome in Israel with Emphasis on Upper Extremity Involvement Evidence for Further Genetic Heterogeneity-Type III. In: Neurogenetics and Neuro-opthalmology. (Eds. Huber, A. and Klein, D. ), pp. 393406. Elsevier/North Holland Biomedical Press.
Goodman, R. M., Lewithal, I., Solomon, A. and Klein, D. (1982). Upper limb involvement in the Klein-Waardenburg Syndrome. American Journal of Medical Genetics 11: 425433.
Hageman, M. and Delleman, J. W. (1977). Heterogeneity in Waardenburg syndrome. American Journal of Human Genetics 29: 468485.
Jerger, J., Snedd, J. and Harford, E. (1959). On the detection of extremely small changes in sound intensity. Archives of Otolaryngology 69: 200211.
La Ferriere, K. A., Arenberg, I. K., Hawkins, J. E. Jr., and Johnsson, L. G. (1974). Melanocytes of the vestibular labyrinth and their relationship to the microvasculature. Annals of Otology, Rhinology and Laryngology 83: 685694.
Rosenberg, P. E. (1969). Tone Decay. Maico Audiologic Library Series, 7: Report 6.
Selters, W. A., Brackmann, D. E. (1977). Acoustic tumor detection with brain stem electric response audiometry. Archives of Otolaryngology 103: 181187.
Waardenburg, P. J. (1951). A new syndrome combining developmental anomalies of the eyelids, eyebrows and nose roof with pigmentary defects of the iris and head hair and with congenital deafness. American Journal of Human Genetics 3: 195255.
Wilber, L. (1976). Acoustic reflex measurement procedures interpretation and variables. In: Acoustic Impedance and Admittance. (Eds. Feldman, A. S., Wilber, L. A.) pp. 197216. Williams and Wilkins Company, Baltimore.
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The Journal of Laryngology & Otology
  • ISSN: 0022-2151
  • EISSN: 1748-5460
  • URL: /core/journals/journal-of-laryngology-and-otology
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