Usher Syndrome: A Temporal Bone Report

Usher Syndrome A Temporal Bone Report

Usher syndrome. A temporal bone report.

The bilateral temporal bones of a deceased 84-year-old man who had been suffering from Usher syndrome were examined using light microscopy. Histopathologic examination disclosed degeneration of the organ of Corti that was most profound in the basal turn, degeneration of cochlear neurons in all of the turns, and severe loss of spiral ganglia in both cochleas. Endolymphatic hydrops of unknown cau...

USHER\'S SYNDROME REVISITED

Usher's syndrome is a genetically inherited autosomal recessive disorder resulting in the double handicap of deafness and progressive blindness, known as retinitis pigmentosa. The disease is also associated with psychoses, mental retardation, and other major neurophysiological changes. It appears to be more common among Jewish individuals and consanguinous marriages. While it is rare in th...

Metastatic Adenocarcinoma of Temporal Bone with Collet-Sicard Syndrome

Introduction: Metastatic tumors of the temporal bone are extremely rare. Collet-Sicard syndrome is an uncommon condition characterized by unilateral palsy of the lower four cranial nerves. The clinical features of temporal bone metastasis are nonspecific and mimic infections such as chronic otitis media and mastoiditis.   Case Report: This report describes a rare case of metastatic adenocarcino...

Bilateral uveitis and Usher syndrome: a case report

INTRODUCTION Usher syndrome is a genetically heterogeneous condition and represents the most common cause of inherited combined vision and hearing loss. Deficits manifest as sensorineural hearing loss that typically develops at a young age and retinitis pigmentosa that can lead to peripheral vision loss and night blindness. As a result, this syndrome can have a significant impact on a patient's...