Facioscapulohumeral dystrophy associated with sensorineural hearing loss, tortuosity of retinal arterioles, and an early onset and rapid progression of respiratory failure

1988 ◽  
Vol 10 (5) ◽  
pp. 319-324 ◽  
Author(s):  
Satoshi Yasukohchi ◽  
Yoshio Yagi ◽  
Taro Akabane ◽  
Akiko Terauchi ◽  
Kimiko Tamagawa ◽  
...  
2019 ◽  
Vol 162 (1) ◽  
pp. 114-120 ◽  
Author(s):  
Vanessa Torrecillas ◽  
Chelsea M. Allen ◽  
Tom Greene ◽  
Albert Park ◽  
Winnie Chung ◽  
...  

Objective To describe the progression of sensorineural hearing loss (SNHL) in the better- and poorer-hearing ears in children with asymptomatic congenital cytomegalovirus (CMV) infection with isolated SNHL. Study Design Longitudinal prospective cohort study. Setting Tertiary medical center. Subjects and Methods We analyzed hearing thresholds of the better- and poorer-hearing ears of 16 CMV-infected patients with isolated congenital/early-onset or delayed-onset SNHL identified through hospital-based CMV screening of >30,000 newborns from 1982 to 1992. Results By 12 months of age, 4 of 7 patients with congenital/early-onset SNHL developed worsening thresholds in the poorer-hearing ear, and 1 had an improvement in the better-hearing ear. By 18 years of age, all 7 patients had worsening thresholds in the poorer-hearing ear and 3 patients had worsening thresholds in the better-hearing ear. Hearing loss first worsened at a mean age of 2 and 6 years in the poorer- and better-hearing ears, respectively. Nine patients were diagnosed with delayed-onset SNHL (mean age of 9 years vs 12 years for the poorer- and better-hearing ears), 6 of whom had worsening thresholds in the poorer-hearing ear and 1 in both ears. Conclusion In most children with congenital CMV infection and isolated SNHL, the poorer-hearing ear worsened earlier and more precipitously than the better-hearing ear. This study suggests that monitoring individual hearing thresholds in both ears is important for appropriate interventions and future evaluation of efficacy of antiviral treatment.


1998 ◽  
Vol 124 (8) ◽  
pp. 886 ◽  
Author(s):  
Robbert J. H. Ensink ◽  
Kristien Verhoeven ◽  
Henri A. M. Marres ◽  
Patrick L. M. Huygen ◽  
Georges W. Padberg ◽  
...  

2010 ◽  
Vol 74 (7) ◽  
pp. 825-827 ◽  
Author(s):  
Yuval Ramot ◽  
Koji Sayama ◽  
Ruth Sheffer ◽  
Victoria Doviner ◽  
Nurith Hiller ◽  
...  

Stem Cells ◽  
2011 ◽  
Vol 29 (4) ◽  
pp. 670-677 ◽  
Author(s):  
Sonali R. Pandit ◽  
Jeremy M. Sullivan ◽  
Viktoria Egger ◽  
Alexander A. Borecki ◽  
Sharon Oleskevich

2010 ◽  
Vol 268 (2) ◽  
pp. 197-201 ◽  
Author(s):  
T. S. Ibekwe ◽  
P. O. Okokhere ◽  
D. Asogun ◽  
F. F. Blackie ◽  
M. M. Nwegbu ◽  
...  

2011 ◽  
Vol 114 (6) ◽  
pp. 557-561 ◽  
Author(s):  
Yuko Kataoka ◽  
Kunihiro Fukushima ◽  
Yukihide Maeda ◽  
Akiko Sugaya ◽  
Rie Nagayasu ◽  
...  

2012 ◽  
Vol 18 (4) ◽  
pp. 549-557 ◽  
Author(s):  
Israel Lerman-Garber ◽  
Daniel Cuevas-Ramos ◽  
Samantha Valdés ◽  
Lorena Enríquez ◽  
Marlette Lobato ◽  
...  

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