Histopathological and neuroradiological features of Usher syndrome type II.
OBJECTIVE
To study the histopathological and neuroradiological features of Usher syndrome (USH), with particular focus on USH type II, an inherited disorder characterized by moderate to severe congenital hearing impairment and retinitis pigmentosa with onset in the late teens.


METHODS
A report of four cases and literature review.


CONCLUSIONS
Rare examples of histopathological and neuroradiological findings from four USH type II cases are presented. More studies like these are encouraged so that correlation studies between the morphological and clinical findings can be performed on the path to elucidate the pathogenesis of this heterogeneous disorder.
Ciorba
A
A
Audiology Department, University Hospital of Ferrara, Ferrara, Italy.
Schrott-Fisher
A
A
Berto
A
A
Glueckert
R
R
Janecke
A
A
Martini
A
A
D000328:Adult; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D011859:Radiography; D013701:Temporal Bone / Q000000981:diagnostic imaging / Q000473:pathology; D052245:Usher Syndromes / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology*