This Article  • References  • Full text PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

A Variant of Branchio-oto Dysplasia

Alan F. Lipkin, MD; Newton J. Coker, MD; Herman A. Jenkins, MD

Arch Otolaryngol Head Neck Surg. 1986;112(10):1097-1100.

Abstract
• A mother and daughter both presented at age 5 years with the triad of right-sided congenital cholesteatoma, right preauricular pits, and bilateral sensorineural hearing loss. Twenty-six years apart, both were treated with middle ear exploration and removal of a cholesteatoma that filled the sinus tympani, facial recess, and middle ear. The sensorineural hearing losses were nonprogressive, and the preauricular pits were asymptomatic. These two cases may represent a unique variant of branchio-oto dysplasia. The mechanism of formation of these anomalies and the possible modes of inheritance are conjectural. This triad, however, supports genetic predisposition rather than aberrant epithelial rests during morphogenesis as a possible cause in congenital cholesteatoma.

(Arch Otolaryngol Head Neck Surg 1986;112:1097-1100)

Author Affiliations
From the Department of Otorhinolaryngology and Communicative Sciences, Baylor College of Medicine, Houston. Dr Lipkin is now in private practice, in Denver.

Footnotes
Accepted for publication Feb 7, 1986.

Presented in part at the annual meeting of the American Academy of Otolaryngology-Head and Neck Surgery (Scientific Poster), Atlanta, Oct 21, 1985.

Reprint requests to Department of Otorhinolaryngology and Communicative Sciences, Baylor College of Medicine, 1 Baylor Plaza, Houston, TX 77030 (Dr Coker).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?