1 Chapter 32 Hearing Loss in Osteogenesis Imperfecta Copyright © 2014 Elsevier Inc. All rights reserved.

2 FIGURE 32.1 A hematoxylin and eosin-stained cross-section of a temporal bone in a patient with type I OI. This patient had a conductive hearing loss that progressed to a mixed hearing loss during late adulthood. The cochlea is surrounded by a darker blue stain corresponding to the otosclerotic lesion. Beneath the cochlea there is an area of cavitation. The asterix indicates the silhouette of a surgically placed stapes prosthesis to correct the conductive hearing loss. This figure is reproduced in color in the color plate section.

3 Copyright © 2014 Elsevier Inc. All rights reserved. FIGURE 32.2 A hematoxylin and eosin-stained temporal bone of a patient with type II OI. The cochlea and structures of the inner ear are normal. In this section delayed patterns of ossicle formation are noted. The stapes footplate is separated into two layers and the incus has a higher content of marrow (asterix) than predicted for age. This figure is reproduced in color in the color plate section.

4 Copyright © 2014 Elsevier Inc. All rights reserved. FIGURE 32.3 Audiological fi ndings are shown for a 43-year-old male with OI. This individual has a mixed hearing impairment with a conductive hearing loss in the low frequency region from 250 to 1000 Hz with sensorineural hearing loss at 2000 Hz and above. This figure is reproduced in color in the color plate section.

5 Copyright © 2014 Elsevier Inc. All rights reserved. FIGURE 32.4 Findings for a 35-year-old female with OI are shown. This patient has a sensorineural hearing loss for the left ear and a conductive hearing loss for the right ear. This figure is reproduced in color in the color plate section.