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Updated: Jun 30, 2026
Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
Bilateral profound sudden sensorineural hearing loss presenting a diagnostic conundrum in a child with sickle cell
A D Mace1, M S Ferguson, M Offer
1Department of Otolaryngology, St Bartholomew's and The Royal London Hospitals NHS Trust, London, UK. alasdairmace@hotmail.com
Insights
This case report highlights a child with sickle cell anaemia who experienced sudden sensorineural hearing loss. Early recognition and treatment are crucial for managing this rare complication in children.
Area of Science:
- Otolaryngology
- Pediatrics
- Hematology
Background:
- Sudden sensorineural hearing loss (SSNHL) is uncommon in pediatric populations.
- Sickle cell anaemia is a known risk factor for various vascular complications.
Observation:
- A seven-year-old child with sickle cell anaemia presented with bilateral profound SSNHL over five days.
- The patient had a history of ophthalmological disease and elevated inflammatory markers.
- Differential diagnoses included vaso-occlusive events and inflammatory conditions like Cogan's syndrome.
Findings:
- Despite empirical treatment for potential vaso-occlusive and inflammatory causes, hearing thresholds did not improve.
- The child ultimately underwent cochlear implantation 12 weeks after symptom onset.
Implications:
- This case underscores the importance of considering SSNHL in children with sickle cell anaemia.
- Prompt investigation and empirical treatment are vital for potential hearing salvage.
- Early cochlear implantation referral is indicated if hearing recovery is not observed.
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.
