Logo image
Phenotypic manifestations of branchiootorenal syndrome
Journal article   Peer reviewed

Phenotypic manifestations of branchiootorenal syndrome

Achih Chen, Mary Francis, Li Ni, Cor W. R. J Cremers, William J Kimberling, Yutaka Sato, Peter D Phelps, Susan C Bellman, Michael J Wagner, Marcus Pembrey, …
American journal of medical genetics, Vol.58(4), pp.365-370
09/25/1995
DOI: 10.1002/ajmg.1320580413
PMID: 8533848

View Online

Abstract

Branchiootorenal (BOR) syndrome is a variable, autosomal‐dominant disorder of the first and second embryonic branchial arches, kidneys, and urinary tract. We describe the phenotype in 45 individuals, highlighting differences and similarities reported in other studies. Characteristic temporal bone findings include cochlear hypoplasia (4/5 of normal size with only 2 turns), dilation of the vestibular aqueduct, bulbous internal auditory canals, deep posterior fossae, and acutely‐angled promontories. © 1995 Wiley‐Liss, Inc.
branchiootorenal syndrome syndromic hearing loss renal agenesis cochlear hypoplasia

Details

Metrics

Logo image