The auditory function of 75 children affected by homozygous β°-thalassemia, managed with a low transfusion scheme and treated irregularly with low doses of desferrioxamine, and of 75 controls were examined. In 12 patients a mild bilateral conductive hearing impairment due to bony hypertrophy and/or adenoid hypertrophy was found. In 43 cases a moderate monolateral or bilateral sensory-neural hearing loss at high frequencies with recruitment phenomenon was observed. Ferritin levels were determined in a randomly chosen group of these patients with (14) and without hearing loss (11). In the subjects with sensory-neural hearing loss the mean ferritin levels were significantly higher than in those with no hearing defect. There was no obvious relation between sensory-neural damage on the one hand and Hb levels and unit of blood transfused on the other. The results of this study suggest that iron overload could be a cause of damage in the high frequency elements of the auditory mechanism. Intermittent hypoxia and slow 8th nerve compression due to bony hypertrophy as causes of auditory involvement are also discussed.
Muscle and serum CPK electrophoretic pattern and serum GOT electrophoretic pattern were studied in Duchenne type, limb-girdle type and distal congenital type muscular dystro- phies. Muscle CPK isoenzyme pattern usually did not show significant changes. In 2 cases a fetal-like pattern was observed. Serum CPK isoenzyme pattern showed in some cases only the MM type. In other cases MB type was found in addition to MM type. Serum electrophoresis zone of GOT displayed in some cases only the cytoplasmic isoenzyme. Other cases showed mitochondrial isoenzyme in addition to the cytoplasmic type. The latter was predominant. GOT mitochondrial isoenzyme and CPK MB isoenzyme were found in serum in the early stages of the disease. These results suggest that a relationship may intercur between the pathological changes in the muscle in early muscular dystrophy and the presence in serum of GOT mitochondrial isoenzyme and CPK MB isoenzyme.
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