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Bilateral congenital cholesteatoma in branchio-oto-renal syndrome

Published online by Cambridge University Press:  29 June 2007

George A. Worley ,
Archana Vats ,
Jonathan Harcourt  and
David M. Albert
George A. Worley*
Affiliation:
Department of Otolaryngology, Head and Neck Surgery, Great Ormond Street Hospital for Children, London, UK.
Archana Vats
Affiliation:
Department of Otolaryngology, Head and Neck Surgery, Great Ormond Street Hospital for Children, London, UK.
Jonathan Harcourt
Affiliation:
Department of Otolaryngology, Head and Neck Surgery, Great Ormond Street Hospital for Children, London, UK.
David M. Albert
Affiliation:
Department of Otolaryngology, Head and Neck Surgery, Great Ormond Street Hospital for Children, London, UK.
*
Address for correspondence: Mr George A. Worley, F.R.C.S., 43 St Peter's Lane, Canterbury, Kent CT1 2BP.
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Abstract

Branchio-oto-renal syndrome is a rare autosomal dominant condition characterized by hearing loss, branchial arch abnormalities and renal tract malformations. We present the first reported case of branchio-oto-renal syndrome associated with bilateral congenital cholesteatoma and ossicular chain abnormalities. The pathogenesis of this syndrome is described and the literature is reviewed.

Keywords

Urinary tractCholesteatoma, middle earEar ossicles, abnormalitiesHearing loss, bilateral

Information

Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 113 , Issue 9 , September 1999 , pp. 841 - 843
Copyright
Copyright © JLO (1984) Limited 1999

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References

Arnold, W., Weidaur, H., Seelig, H. P. (1976) Expermenteller beweis einer gemeinsamen antigenizitaet zwischen Innenohr und niere. Archives of Oto-Rhino-Laryngology 212: 99–117.CrossRefGoogle Scholar
Chen, A., Francis, M., Ni, L., Cremers, C. W. R. J., Kimberling, W. J., Sato, Y., Phelps, P. D., Bellman, S. C., Wagner, M. J., Pembrey, M., Smith, R. J. H. (1995) Phenotypic manifestations of branchiootorenal syndrome. American Journal of Medical Genetics 58: 365–370.CrossRefGoogle Scholar
Cremers, C. W. R. J., Fikkers-Van Noord, M. (1980) The earpits-deafness syndrome. International Journal of Paediatric Otorhinolaryngology 2: 309–322.CrossRefGoogle ScholarPubMed
Fitch, N., Lindsay, J. R., Srolovitz, H. (1976) The temporal bone in the preauricular pit, cervical fistula, hearing loss syndrome. Annals of Otology, Rhinology and Laryngology 85: 268–275.CrossRefGoogle ScholarPubMed
Fraser, F. C., Sproule, J. R., Halal, F. (1980) Frequency of the branchio-oto renal syndrome in children with profound hearing loss. American Journal of Medical Genetics 7: 341–349.CrossRefGoogle ScholarPubMed
Gorlin, R. J., Cohen, M. M., Levin, L. S. (1990) In Syndromes of the Head and Neck. 3rd Edition. (Gorlin, R. J., Cohen, M. M., Levin, L. S., eds.), Oxford University Press, New York, NY, pp 657–659.Google Scholar
Peron, D. L., Schuknecht, H. F. (1975) Congenital cholesteatoma with other anomalies. Archives of Otolaryngology 101: 498–505.CrossRefGoogle ScholarPubMed
Quick, C. A., Fish, A., Brown, C. (1973) The relationship between the cochlea and kidney. Larygnoscope 83: 1469–1482.CrossRefGoogle ScholarPubMed
Smith, P. G., Dyches, T. J., Loomis, R. A. (1984) Clinical aspects of the branchio-oto-renal syndrome. Otolaryngology – Head and Neck Surgery 92: 468–475.CrossRefGoogle ScholarPubMed
Suetake, M., Kobayashi, T., Takasaka, T. (1991) Bilateral congenital cholesteatomas associated with ossicular anomalies: A case report. American Journal of Otology 12: 132–134.Google ScholarPubMed
Wang, R., Zubick, H. H., Vernick, D. M. (1984) Bilateral congenital middle ear cholesteatomas. Laryngoscope 94: 1461–1463.CrossRefGoogle ScholarPubMed