The WFS1 gene, responsible for low frequency sensorineural hearing loss and Wolfram syndrome, is expressed in a variety of inner ear cells

2003 ◽  
Vol 119 (3) ◽  
pp. 247-256 ◽  
Author(s):  
Kim Cryns ◽  
Sofie Thys ◽  
Lut Van Laer ◽  
Yoshitomo Oka ◽  
Markus Pfister ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Low Frequency ◽  
Wolfram Syndrome ◽  
Sensorineural Hearing ◽  
Wfs1 Gene

A mutation in Wolfram syndrome type 1 gene in a Japanese family with autosomal dominant low-frequency sensorineural hearing loss

2005 ◽  
Vol 125 (11) ◽  
pp. 1189-1194 ◽  
Author(s):  
Yoshihiro Noguchi ◽  
Takatoshi Yashima ◽  
Akio Hatanaka ◽  
Masamichi Uzawa ◽  
Michio Yasunami ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Autosomal Dominant ◽  
Low Frequency ◽  
Wolfram Syndrome ◽  
Sensorineural Hearing ◽  
Syndrome Type ◽  
Japanese Family

Mutations in the WFS1 gene that cause low-frequency sensorineural hearing loss are small non-inactivating mutations

2002 ◽  
Vol 110 (5) ◽  
pp. 389-394 ◽  
Author(s):  
Kim Cryns ◽  
Markus Pfister ◽  
Ronald J. Pennings ◽  
Steven J. Bom ◽  
Kris Flothmann ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Low Frequency ◽  
Sensorineural Hearing ◽  
Wfs1 Gene ◽  
Inactivating Mutations

Drug Delivery to the Inner Ear: Strategies and Their Therapeutic Implications for Sensorineural Hearing Loss

2012 ◽  
Vol 9 (3) ◽  
pp. 231-242 ◽  
Author(s):  
Teresa Rivera ◽  
Lorena Sanz ◽  
Guadalupe Camarero ◽  
Isabel Varela-Nieto
Keyword(s):  
Drug Delivery ◽  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sensorineural Hearing ◽  
Therapeutic Implications

Azathioprine in Combination with Steroids in the Treatment of Autoimmune Inner-Ear Disease

1993 ◽  
Vol 21 (4) ◽  
pp. 192-196 ◽  
Author(s):  
Aytac Saraçaydin ◽  
Sedat Katircioğlu ◽  
Sami Katircioğlu ◽  
M Can Karatay
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sensorineural Deafness ◽  
Sensorineural Hearing ◽  
Once Daily ◽  
Uncommon Disease ◽  
Inner Ear Disease ◽  
Pure Tones ◽  
Autoimmune Inner Ear Disease

A total of twelve patients with a relatively uncommon form of progressive sensorineural deafness (autoimmune innerear disease) were treated orally with 1 mg/kg azathioprine, once daily, and with 30 mg prednisolone, every other day, for 4 weeks. Statistically significant increases in the ability to hear pure tones or in discrimination on audiometry took place in 10/12 patients. This condition was initially described as ‘sensorineural hearing loss', but it is now clear that the term ‘autoimmune inner-ear disease’ is more appropriate since the vestibular compartment as well as the cochlear compartment is involved. This relatively uncommon disease is one of the few forms of sensorineural deafness that can be successfully treated.

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