Temporal bone histopathologic features in Fanconi's anemia syndrome.

A histopathologic study of the temporal bones from a 7-year-old girl with Fanconi's anemia syndrome demonstrated (1) hemorrhage in the submucosal layer and the cavity of the middle ear and mastoid, massive in the right ear; (2) hypocellularity of the bone marrow; (3) minor but multiple anomalies of the middle ear; and (4) hypodevelopment of the hook portion of the cochlea and reduced overall length of the cochlear duct. The histopathologic features of these temporal bones appears to suggest that congenital anomalies of the inner ear, as well as those of the external and middle ears, would be possible causes of the deafness that accompanies Fanconi's anemia syndrome.