Usher's syndrome type 3 in Finland.
Usher's syndrome, type 3 (USH3) is characterized by progressive hearing loss. Usher's syndrome, type 3 has been supposed to be rare, occurring in 2% to 4% of all patients with Usher's syndrome. In a nationwide study we collected data on 229 patients with Usher's syndrome in Finland. Definite cases of USH3 were found in 30 (13%) of the 229 patients. An additional 61 patients had clinical evidence of earlier progression of their hearing impairment. We suggest that 91 (40%) of the 229 patients with Usher's syndrome represent cases of USH3.
Pakarinen
L
L
Department of Otorhinolaryngology and Phoniatrics, Tampere University Hospital, Finland.
Karjalainen
S
S
Simola
K O
KO
Laippala
P
P
Kaitalo
H
H
D000328:Adult; D018450:Disease Progression; D005260:Female; D005387:Finland / Q000453:epidemiology; D005500:Follow-Up Studies; D005808:Genes, Recessive; D006319:Hearing Loss, Sensorineural / Q000151:congenital / Q000453:epidemiology / Q000235:genetics*; D006320:Hearing Tests; D006801:Humans; D008297:Male; D011446:Prospective Studies; D012174:Retinitis Pigmentosa / Q000453:epidemiology / Q000235:genetics*; D013577:Syndrome; D013997:Time Factors