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

Melisha Sirisena, Catherine S Birman, Amy J McKibbin, Katie J O'Brien

Journal: International journal of pediatric otorhinolaryngology 2018;112():126-131

PMID: 30055721

Full Text on Elsevier Science View on PubMed

Abstract

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.

Address: Department of Audiology, The Sydney Children's Hospital Network (Westmead), Level 2, Locked Bag 4001, Westmead, NSW, 2145, Australia. Electronic address: [email protected].; Department of Otolaryngology (ENT), The Sydney Children's Hospital Network (Westmead), Sydney Medical School, The University of Sydney, The Sydney Cochlear Implant Centre, PO Box 188, Gladesville, NSW, 1675, Australia. Electronic address: [email protected].; Department of Audiology, The Sydney Children's Hospital Network (Westmead), Level 2, Locked Bag 4001, Westmead, NSW, 2145, Australia. Electronic address: [email protected].; Department of Audiology, The Sydney Children's Hospital Network (Westmead), Level 2, Locked Bag 4001, Westmead, NSW, 2145, Australia. Electronic address: [email protected].

DOI: 10.1016/j.ijporl.2018.06.046

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MeSH Terms: Child, Ear Ossicles, Hearing Loss, Conductive, Hematopoiesis, Extramedullary, Humans, Male, beta-Thalassemia