Search

Objective: To describe congenital anomalies of the incudostapedial joint (ISJ) and to discuss the possible unique embryogenesis of the ISJ based on the two anomalies that were encountered., Setting: Tertiary care medical center., Subjects and Methods: Retrospective review of the medical records of all patients with ISJ anomalies., Results: Four patients presented with congenital hearing loss. Upon further workup, we observed a preserved incudostapedial joint (ISJ) with deficiencies of the incus and stapes in three cases. Our fourth case demonstrated the inverse pattern of the congenital anomalies in which the ISJ was missing with an intact proximal incus and stapes crura. Three patients opted for surgical intervention with improvement in hearing. One case preferred hearing amplification over surgery., Conclusion: Isolated ISJ malformations are uncommon potential causes of congenital conductive hearing loss. Although numerous patterns of ossicular anomalies have been reported in the literature, our case series is the first to demonstrate both the absence of the ISJ in one patient and the presence of the ISJ in the presence of missing stapes crura and incus body in other patients. Though limited by the small number of cases, the inverse relationship of the single case compared to the three other cases, suggests a possible independent embryological development pathway for the ISJ. Therefore, an embryological explanation of the defects should be considered. Additionally, surgical intervention can improve hearing outcomes for patients with isolated ISJ anomalies., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Objective: Temporal bone imaging in children has several inherent limitations. Computed tomography has the disadvantage of ionizing radiation, possible sedation, cost and accessibility. Magnetic resonance imaging has most of these disadvantages, with the exception of radiation, and provides bone images of limited resolution. Recent advances in ultrasound have led to its increased application in numerous medical fields. The purpose of this study was to investigate the ability of four-dimensional ultrasound (4DUS) to image middle ear ossicles in vitro and determine if this technology should be adapted for future clinical use., Methods: Thirty cadaveric ossicles (10 malleus, 10 incus, and 10 stapes) were randomized and measured by two evaluators under a microscope. The ossicles were then immersed in a cold water bath and imaged, randomized, and measured using four-dimensional ultrasound by the same two evaluators. A separate cadaveric temporal bone, modified to allow the ultrasound probe to rest on the tympanic membrane, was imaged to visualize the ossicles in situ and evaluate whether or not the tympanic membrane and malleus would impede visualization of more medial structures., Results: Microscopic measurements were: malleus (h=8.0 mm+/-0.32, w=2.7 mm+/-0.20), incus (h=6.8 mm+/-0.41, w=5.3 mm+/-0.46), stapes (h=3.5 mm+/-0.34, w=2.4 mm+/-0.17). Inter-rater reliability was 0.8. Measurements were in agreement with previously published values. Ultrasound measurements were: malleus (h=8.0 mm+/-0.51, w=2.9 mm+/-0.27), incus (h=6.8 mm+/-0.49, w=5.5 mm+/-0.42), stapes (h=3.6 mm+/-0.41, w=2.5 mm+/-0.19). Inter-rater reliability was 0.7. Mean intra-class correlation coefficient for microscopic and ultrasound measurements was 0.7. Images of the ossicular chain in continuity in the temporal bone specimen were not as clear as images of individual ossicles that were disarticulated and imaged under water., Conclusions: 4DUS provides reasonable images of ossicles disarticulated and mounted in underwater medium. However, images of the intact ossicular chain in a modified cadaveric temporal bone were not as clear, making interpretation difficult. Further investigation into the development of a thinner ultrasound probe that can pass through the external auditory canal and into overcoming limitations of air in the middle ear cleft are warranted. This could allow for a clinically relevant, faster, lower cost and lower risk alternative to current imaging techniques., (Copyright 2010 Elsevier Ireland Ltd. All rights reserved.)

A 7-year-old boy with a history of purulent meningitis and watery rhinorrhea was studied using computed tomography (CT) and magnetic resonance imaging (MRI). He had a common cavity in the left inner ear. With high-resolution heavily T2-weighted MRI, leakage of the inner ear fluid into the middle ear at the oval window area through a congenital perilymphatic fistula could be visualized. Surgery to close the fistula showed a perforation in the stapes footplate.