scholarly journals Pre‐emptive intra‐auricular electrosurgical resurfacing of cylindromas to prevent conductive deafness in CYLD cutaneous syndrome

2020 ◽  
Vol 183 (6) ◽  
Author(s):  
J. Langtry ◽  
N. Rajan
Keyword(s):  
1977 ◽  
Vol 70 (11) ◽  
pp. 807-815 ◽  
Author(s):  
Ruth E M Bowden
Keyword(s):  

2020 ◽  
pp. 014556132094463
Author(s):  
Huiying Sun ◽  
Yufei Qiao ◽  
Na Chen ◽  
Hua Yang ◽  
Zhiqiang Gao ◽  
...  

We report a 6-year-old girl with progressive bilateral conductive hearing loss for 2 years. She passed the newborn hearing screening conducted with otoacoustic emissions testing and had a normal development of speech and language, which indicated that her deafness was delayed-onset. She also had congenital proximal interphalangeal joints. Proximal symphalangism was confirmed by genetic testing ( NOG gene: c.406C > T, p.R136C). Bilateral stapes ankyloses were proved by surgery and her hearing was improved after stapedotomy by over 30 dB. Besides, this case should remind clinicians to carefully distinguish NOG gene-related deafness from congenital ossicular malformation and pediatric otosclerosis.


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