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Bilateral profound sudden sensorineural hearing loss presenting a diagnostic conundrum in a child with sickle cell anaemia

Published online by Cambridge University Press:  17 September 2008

A D Mace ,
M S Ferguson ,
M Offer ,
K Ghufoor  and
M J Wareing
A D Mace*
Affiliation:
Department of Otolaryngology, St Bartholomew's and The Royal London Hospitals NHS Trust, London, UK
M S Ferguson
Affiliation:
Department of Otolaryngology, St Bartholomew's and The Royal London Hospitals NHS Trust, London, UK
M Offer
Affiliation:
Department of Haematology, St George's Healthcare NHS Trust, London, UK
K Ghufoor
Affiliation:
Department of Otolaryngology, St Bartholomew's and The Royal London Hospitals NHS Trust, London, UK
M J Wareing
Affiliation:
Department of Otolaryngology, St Bartholomew's and The Royal London Hospitals NHS Trust, London, UK
*
Address for correspondence: Mr Alasdair Mace, 2 Whitelands Avenue, Chorleywood WD3 5RD, UK. E-mail: alasdairmace@hotmail.com
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Abstract

Objective:

To present the first published case of a child with bilateral profound sudden sensorineural hearing loss found in association with sickle cell anaemia, and to demonstrate the importance of early recognition, investigation and empirical treatment of sudden sensorineural hearing loss.

Method:

Case report and review of world literature.

Case report:

The authors present the case of a seven-year-old child with known sickle cell anaemia, who presented with bilateral profound sensorineural hearing loss developing over a period of five days. There was a history of ophthalmological disease in the preceding weeks, and inflammatory markers were raised. The differential diagnosis included a vaso-occlusive or inflammatory aetiology such as Cogan's syndrome, and treatment for both was instigated. Hearing thresholds did not recover, and the patient underwent cochlear implantation 12 weeks later.

Conclusion:

Sudden sensorineural hearing loss has a variable aetiology and is rare in children. Immediate treatment for all possible aetiologies is essential, along with targeted investigations and early referral for cochlear implantation if no recovery is demonstrated.

Keywords

Sickle Cell AnaemiaSensorineural Hearing LossCogan's SyndromeChild
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 123 , Issue 7 , July 2009 , pp. 811 - 816
Copyright
Copyright © JLO (1984) Limited 2008

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