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Cochlear implantation in children with sickle cell disease
Thomas Ashfield1, Irumee Pai, Katherine Wilson
1Auditory Implant Centre, St Thomas' Hospital, London, UK.
Summary
Sickle cell disease (SCD) can cause severe hearing loss. Cochlear implants may help, but cochlear ossification presents challenges in SCD patients needing these devices.
Area of Science:
- Otolaryngology
- Hematology
- Genetics
Background:
- Sickle cell disease (SCD) is linked to sensorineural hearing loss (SNHL).
- While often mild, SNHL in SCD can become severe-to-profound, necessitating interventions like cochlear implants (CI).
Observation:
- Two children with SCD experienced bilateral severe-to-profound SNHL.
- One patient developed profound deafness post-dizziness, with imaging revealing cochlear ossification complicating CI surgery.
- The second patient developed SNHL after vaso-occlusive crises, undergoing successful CI surgery.
Findings:
- SCD can lead to variable SNHL progression, impacting management.
- Cochlear ossification is a potential complication in SCD patients with SNHL, posing surgical challenges for cochlear implantation.
- Prompt audiological assessment is crucial after acute events in children with SCD.
Implications:
- Regular audiological screening is recommended for children with SCD.
- Clinicians must recognize the risk of cochlear fibrosis and ossification in SCD.
- Timely evaluation is essential following vaso-occlusive crises or vestibulocochlear events in these patients.
