Usher's syndrome, temporal bone pathology.
The histological findings in the right temporal bone of a 65-year-old deaf and blind man are presented. The subject suffered from the autosomal recessive Usher's syndrome, as did 2 of his 5 siblings. They are the offspring of a consanguineous marriage. This man died from an intra-abdominal hemorrhage. Within 3 h after death the temporal bones were donated for study and were processed for histopathological examination.
Cremers
C W
CW
Department of Otorhinolaryngology, University of Nijmegen, The Netherlands.
Delleman
W J
WJ
D000368:Aged; D001766:Blindness / Q000235:genetics* / Q000473:pathology; D002869:Chromosome Aberrations / Q000235:genetics* / Q000473:pathology; D025063:Chromosome Disorders; D003056:Cochlear Nerve / Q000473:pathology; D003638:Deafness / Q000235:genetics* / Q000473:pathology; D005808:Genes, Recessive; D006801:Humans; D008297:Male; D009412:Nerve Fibers / Q000473:pathology; D009925:Organ of Corti / Q000473:pathology; D010375:Pedigree; D012174:Retinitis Pigmentosa / Q000235:genetics* / Q000473:pathology; D013577:Syndrome; D013701:Temporal Bone / Q000473:pathology*