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


METHODS
Case report and review of world literature.


METHODS
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.


CONCLUSIONS
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.
Mace
A D
AD
Department of Otolaryngology, St Bartholomew's and The Royal London Hospitals NHS Trust, London, UK. alasdairmace@hotmail.com
Ferguson
M S
MS
Offer
M
M
Ghufoor
K
K
Wareing
M J
MJ
D000755:Anemia, Sickle Cell / Q000150:complications*; D001301:Audiometry, Pure-Tone; D002648:Child; D019929:Cochlear Implantation / Q000379:methods; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000209:etiology* / Q000601:surgery; D006801:Humans; D012720:Severity of Illness Index; D016896:Treatment Outcome; D014606:Uveitis, Anterior / Q000188:drug therapy