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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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References

1 Byl, FM. Seventy-six cases of presumed sudden hearing loss occurring in 1973: prognosis and incidence. Laryngoscope 1977;87:817–25CrossRefGoogle ScholarPubMed
2 Hughes, GB, Freedman, MA, Haberkamp, TJ, Guay, ME. Sudden sensorineural hearing loss. Otolaryngol Clin North Am 1996;29:393–405CrossRefGoogle ScholarPubMed
3 Lee, H, Baloh, RW. Sudden deafness in vertebrobasilar ischemia: clinical features, vascular topographical patterns and long-term outcome. J Neurol Sci 2005;228:99–104CrossRefGoogle ScholarPubMed
4 Gluth, MB, Baratz, KH, Matteson, EL, Driscoll, CL. Cogan syndrome: a retrospective review of 60 patients throughout a half century. Mayo Clin Proc 2006;81:483–8CrossRefGoogle ScholarPubMed
5 Perdu, J, Gimenez-Roqueplo, AP, Boutouyrie, P, Beaujour, S, Laloux, B, Nau, V et al. Alpha1-antitrypsin gene polymorphisms are not associated with renal arterial fibromuscular dysplasia. J Hypertens 2006;24:705–10CrossRefGoogle Scholar
6 Sang, CN, Whelton, PK, Hamper, UM, Connolly, M, Kadir, S, White, RI et al. Etiologic factors in renovascular fibromuscular dysplasia. A case-control study. Hypertension 1989;14:472–9CrossRefGoogle ScholarPubMed
7 Gladstien, K, Rushton, AR, Kidd, KK. Penetrance estimates and recurrence risks for fibromuscular dysplasia. Clin Genet 1980;17:115–16CrossRefGoogle ScholarPubMed
8 Mettinger, KL, Ericson, K. Fibromuscular dysplasia and the brain. I. Observations on angiographic, clinical and genetic characteristics. Stroke 1982;13:46–52CrossRefGoogle Scholar
9 Kempf, HG. Ear involvement in Wegener's granulomatosis. Clin Otolaryngol 1989;14:451–6CrossRefGoogle ScholarPubMed
10 Rowe-Jones, JM, Macallan, DC, Sorooshian, M. Polyarteritis nodosa presenting as bilateral sudden onset cochleo-vestibular failure in a young woman. J Laryngol Otol 1990;104:562–4CrossRefGoogle ScholarPubMed
11 Jagiello, P, Gross, WL, Epplen, JT. Complex genetics of Wegener granulomatosis. Autoimmun Rev 2005;4:42–7CrossRefGoogle ScholarPubMed
12 Phillips, JS, King, JA, Chandran, S, Prinsley, PR, Dick, D. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) presenting with sudden sensorineural hearing loss. J Laryngol Otol 2005;119:148–51CrossRefGoogle ScholarPubMed