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Sudden hearing loss due to fibromuscular dysplasia

Published online by Cambridge University Press:  17 October 2008

E Kunstmann ,
A Eickelmann ,
H Sudhoff ,
M Pearson  and
D Brors
E Kunstmann*
Affiliation:
Department of Human Genetics, Ruhr University Bochum, Germany Praxis für Humangenetik, University of Wuerzburg, Germany
A Eickelmann
Affiliation:
Department of Human Genetics, Ruhr University Bochum, Germany
H Sudhoff
Affiliation:
Department of Otolaryngology, Städt Kliniken Bielefeld, Germany
M Pearson
Affiliation:
Department of Otolaryngology and Head and Neck Surgery, St Elisabeth Hospital, Ruhr University Bochum, Germany
D Brors
Affiliation:
Department of Otolaryngology and Head and Neck Surgery, St Elisabeth Hospital, Ruhr University Bochum, Germany
*
Address for correspondence: Dr Erdmute Kunstmann, Praxis für Humangenetik, Theodor Boveri Weg 11, 97074 Wuerburg, Germany. Fax: +49 931 45265859 E-mail: erdmute.kunstmann@rub.de
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Abstract

Objective:

This case is reported in order to demonstrate the importance of detailed clinical analysis, including evaluation of personal and family history, in the differential diagnosis of sudden sensorineural hearing loss.

Case report:

A 50-year-old woman presented with a sudden onset of sensorineural hearing loss in her right ear. She had experienced three previous episodes of sudden sensorineural hearing loss in her left ear, at the ages of 35, 48 and 50 years. She also reported suffering two strokes with left hemiparesis due to fibromuscular dysplasia of her right internal carotid artery. A positive family history of stroke among maternal relatives suggested autosomal dominant inheritance. The patient's personal and family history suggested a rare cause of sudden sensorineural hearing loss, for which alternative therapeutic modalities may be applicable in selected cases.

Conclusions:

Careful follow up of any patient with sudden sensorineural hearing loss and evaluation of their personal and family history is essential, in order to uncover evidence of rare underlying causes of sudden sensorineural hearing loss. For patients with such rare diagnoses, alternative therapy and surveillance modalities may be useful in disease management, depending on pre-existing pathology. Those patients should be managed via a multidisciplinary approach, including genetic counselling, in order to achieve the best possible outcome.

Keywords

Fibromuscular DysplasiaSensorineural DeafnessSudden Deafness
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 123 , Issue 3 , March 2009 , pp. 359 - 362
Copyright
Copyright © JLO (1984) Limited 2008

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