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Query: UMLS:C0019045 (hemoglobinopathies)
 2,704 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In hereditary deafness a battery of audiological and medical investigations are needed to find out the aetiological factors. We tested the blood of children impaired hearing for hemoglobinopathies. Such anomalies might provide a clue to the aetiology. We found abnormal structures of hemoglobin in 5 children suffering from Fanconi's anemia. Four of these children had prenatal conductive hearing loss. The other children showed no abnormal hemoglobin.
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PMID:[Do hemoglobinopathies correlate with genetically-induced hearing disorders?]. 241 80

Extramedullary hematopoiesis occurs in children with hemoglobinopathy and chronic anemia. The liver and spleen are often affected first, but other foci can develop to support erythrocyte demand. We report a case of a nine-year-old with beta thalassemia and temporal bone extramedullary hematopoiesis causing ossicular fixation and bilateral conductive hearing loss. There is only one case in the literature describing this phenomenon in pediatric patients, and this is the first case report of bilateral hearing loss from this physiologic phenomenon. Otolaryngologists should consider this etiology in patients with chronic anemia and conductive hearing loss in the absence of otitis media.
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PMID:Temporal bone extramedullary hematopoiesis as a causeof pediatric bilateral conductive hearing loss:Case report and review of the literature. 2848 23