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Thyroid dyshormonogenesis is usually inherited in an autosomal recessive pattern. Thyroid enlargement may be the only manifestation and dyshormonogenesis should be considered in young patients presenting with euthyroid goitre. The most common abnormalities affect TPO activity and thyroglobulin synthesis. A classic example of dyshormonogenesis due to TPO deficiency is Pendred syndrome, in which goiter is associated with severe sensorineural hearing impairment and abnormality of the bony labyrinth observed on CT examination of the temporal bones.