Malignant external otitis and mastoiditis associated with an IgG4 subclass deficiency in a child

We have presented the first child in Delaware with malignant external otitis associated with IgG4 deficiency. Our patient needed three courses of intravenous antibiotics and twice required mastoidectomy, but has recovered completely following the restoration of the natural barrier between the internal and external ear, using a fascial graft from the large temporalis muscle. Some hearing deficit remains.