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. 2002 Sep;128(9):1033-8.
doi: 10.1001/archotol.128.9.1033.

Inner ear anomalies are frequent but nonobligatory features of the branchio-oto-renal syndrome

Martijn H Kemperman  1 , Sacha M P Koch, Frank B M Joosten, Shrawan Kumar, Patrick L M Huygen, Cor W R J Cremers
Affiliations

Inner ear anomalies are frequent but nonobligatory features of the branchio-oto-renal syndrome

Martijn H Kemperman et al. Arch Otolaryngol Head Neck Surg. 2002 Sep.

Abstract

Objective: To summarize the syndromic features and evaluate the presence of inner ear anomalies in 35 patients with branchio-oto-renal (BOR) syndrome from 6 families.

Design: Retrospective evaluation of magnetic resonance imaging of the temporal bones and clinical features in patients with BOR syndrome.

Setting: Tertiary referral center.

Patients: The study population comprised 35 clinically affected patients with BOR syndrome from 6 families. Most of these families were followed for over 25 years.

Main outcome measures: Twenty-four patients underwent high-resolution, heavily T2-weighted 3-dimensional magnetic resonance imaging of the temporal bones for evaluation of inner ear anomalies. Special attention was paid to the endolymphatic duct and sac.

Results: A total of 7 enlarged endolymphatic ducts and sacs (3 bilaterally and 4 unilaterally) and 5 enlarged endolymphatic ducts only (2 bilaterally and 3 unilaterally) were observed. Eight hypoplastic cochleas and 6 hypoplastic labyrinths were seen bilaterally. Seven family members had normal inner ears.

Conclusion: These findings suggest that inner ear anomalies are frequent but nonobligatory features of BOR syndrome.

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