Cochlear implantation in a pediatric patient with thalassemia major

We report the first known cochlear implantation in a pediatric patient with thalassemia major. After bilateral profound hearing loss was diagnosed a cochlear implantation was performed in the right ear when he was 24 months old. No complications occurred during the surgery and the postoperative period. Free-field audiometry and evaluation of auditory responses to speech (EARS) battery tests were used to evaluate perceptual auditory abilities, speech recognition and language growth. The patient demonstrated encouraging improvement in threshold levels that reached similar values to those of his peers. In contrast his level of language growth and recognition stayed at low levels. Results are discussed in the light of the specificity of this hereditary disease and its complications as we share our experience for better management of those type of patients in future.