We report a case of superior semicircular canal dehiscence, which radiological diagnosis was confirmed by multiplanar reformatted CT images, associated to omolateral enlarged vestibular aqueduct in a 16-year-old female who presented with congenital hearing loss without vestibular symptoms. Both inner ear malformations act as a third mobile window into the labyrinth and cochleo-vestibular symptoms can result from loud sounds or pressure changes. An early diagnosis should be strived for preserve inner ear functions.
Superior semicircular canal dehiscence and lack of the tympano-ossicular system: Favourable functional aspects of a coincidental finding
Objectives: Our objectives were to report the functional conditions of superior semicircular canal dehiscence (SSCD) associated with the post-inflammatory lack of tympano-ossicular system. Methods: A 40-year-old male, initially referred for bilateral ear discharge due to a left cholesteatoma and a right unstable ‘intact bridge’ tympanoplasty, underwent a left open mastoidectomy without a tympano-ossicular reconstruction. Two years later, he had a conversion of the ‘intact bridge’ tympanoplasty into an open mastoidectomy without reconstruction on the right side. Moreover, he had a bilateral SSCD diagnosed by means of vestibular evoked myogenic potentials (VEMPs) and high-resolution computed tomography (HRCT). Results: Combination of the absence of the tympano-ossicular system and SSCD resulted in surprisingly good hearing function. Conclusions: The reported bilateral near-to-normal hearing function outlines the possibility of a favourable outcome deriving from the association of two different adverse conditions. This presents an opportunity for reflection on the pathophysiology of sound transmission, and indicates the usefulness of including the radiological investigation of the bone overlying the superior semicircular canal even when investigating inflammatory middle ear diseases.<p><p>from <a href=”Audiological” _mce_href=”http://informahealthcare.com/doi/abs/10.3109/1651386X.2011.559381″><em>Audiological”>http://informahealthcare.com/doi/abs/10.3109/1651386X.2011.559381″><em>Audiological Medicine</em></a></p>