Bilateral auditory ossicular expansions in a child with beta-thalassemia major: Case report and literature review

Publication date: September 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 112
Author(s): Melisha Sirisena, Catherine S. Birman, Amy J. McKibbin, Katie J. O'Brien
Marrow proliferation of the ossicular chain is a rare phenomenon. To date, only two other cases have described this rarity. We report a third paediatric case from Australia. A seven-year-old with thalassemia major demonstrated conductive impairment during surveillance for Deferasirox ototoxicity. Otitis media was assumed, however, CT scan of the petrous temporal bone revealed extramedullary haematopoiesis causing bilateral ossicular expansions and fixed conductive deficit. Reports of hearing loss in the thalassemia population focus on sensorineural impairment from iron chelation therapies. Clinicians should suspect ossicular deformation where treatment has been delayed, poorly controlled or conductive deficit persists without effusion.



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