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Familial systemic amyloidosis associated with bilateral sensorineural hearing loss and bilateral facial palsies

Published online by Cambridge University Press:  26 July 2006

R Hornigold
Affiliation:
Department of Otolaryngology, Guy's and St Thomas' Hospitals NHS Trust, London, UK
A V Patel
Affiliation:
Department of Otolaryngology, Guy's and St Thomas' Hospitals NHS Trust, London, UK
V M M Ward
Affiliation:
Department of Otolaryngology, Guy's and St Thomas' Hospitals NHS Trust, London, UK
A F O'Connor
Affiliation:
Department of Otolaryngology, Guy's and St Thomas' Hospitals NHS Trust, London, UK

Abstract

The Finnish type of familial amyloid polyneuropathy due to variant gelsolin is a rare form of familial amyloidosis. The subtype was first described in 1969 and is characterized by progressive cranial neuropathies, corneal lattice dystrophy and distal sensorimotor dysfunction. It is extremely uncommon, with only two families known to be affected in the UK. We discuss the case of a 70-year-old woman who presented with bilateral facial nerve palsies, bilateral sensorineural hearing loss and Finnish type familial hereditary amyloidosis. A literature search of the Medline database (1966–2005) was performed, using the keywords ‘amyloid’, ‘hearing loss’ and ‘facial palsy’; however, this association appears to be a novel finding. We review the current literature and discuss otorhinolaryngological presentations of amyloidosis.

Type
Clinical Records
Copyright
2006 JLO (1984) Limited

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