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Cranial hyperostosis and hearing loss (A new syndrome?)

Published online by Cambridge University Press:  29 June 2007

W. H. Moesker
Affiliation:
Amsterdam, The Netherlands
R. A. Tange*
Affiliation:
Amsterdam, The Netherlands
*
Dr R. A. Tange, Department of Otolaryngology, Academisch Ziekenhuis bij de Universiteit van Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam-Z.O.The Netherlands.

Summary

We present two patients, mother and daughter, with a skeletal disorder of the skull, hearing loss and in one of them recurrent facial paralysis. In one patient middle ear exploration was performed. The malleus and incus were found to be fixed in the epitympanum.

The differential diagnosis is discussed. We reviewed all known hereditary conditions with hearing loss and musculoskeletal disease but we could not accept any of these diagnoses for our patients.

Type
Clinical Records
Copyright
Copyright © JLO (1984) Limited 1986

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References

Beighton, P. and Hamersma, H. (1979) Sclerosteosis in South Africa. South African Medical Journal, 55: 783788.Google ScholarPubMed
Beighton, P., Barnard, A., Hamersma, H. and van der Wouden, A. (1984) The syndromic status of sclerosteosis and van Buchem disease. Clinical Genetics, 25: 175181.CrossRefGoogle ScholarPubMed
van Buchem, F. S. P., Hadders, H. N., Hansen, J. F. and Woldring, M. G. (1962) Hyperostosis corticalis generalisata, report of seven cases. American Journal of Medicine, 33: 387397.CrossRefGoogle ScholarPubMed
Cremers, C. W. R. J., Hageman, M. J. and Huizing, E. H. (1982) Erfelijke doofheid en slechthorendheid, p. 55Bohn, Scheltema & Holkema, Utrecht/Antwerpen.Google Scholar
Gorlin, R. J., Spranger, J. and Koszalka, M. F. (1969) In Birth Defects, original article series (Bergsma, D. ed.) Vol V, part 4, pp. 7989, The National Foundation-March of Dimes, New York.Google Scholar
Horan, F. T. and Beighton, P. (1978) Osteopathia striata with cranial sclerosis. An autosomal dominant entity. Clinical Genetics, 13: 201206.CrossRefGoogle ScholarPubMed
Kietzer, G. and Paparella, M. M. (1969) Otolaryngologic disorders in craniometaphyseal dysplasia. Laryngoscope, 79: 921941.CrossRefGoogle ScholarPubMed
Konigsmark, B. W. and Gorlin, R. J. (1976) Genetic and metabolic deafness. W. B. Saunders Company, Philadelphia.Google Scholar
Rimoin, D. L. and Hollister, D. W. (1979) in Birth Defects, Atlas and Compendium, 2nd ed. (Bersma, D. ed.) p. 838, The National Foundation-March of Dimes, New York.Google Scholar
Shea, J., Gerbe, R. and Ayani, N. (1981) Craniometaphyseal dysplasia: the first successful surgical treatment for associated hearing loss. Laryngoscope, 91: 13691374.CrossRefGoogle ScholarPubMed
Van Der Wouden, A. (1971) Botziekteninhet os temporale met gehoorstoornissen. pp. 3435, 6468, Mouton & Co-s' Gravenhage.Google Scholar