Cochlear implantation in patients with autoimmune inner ear disease including cogan syndrome: A comparison with age- and sex-matched controls

2010 ◽  
Vol 120 (12) ◽  
pp. 2478-2483 ◽  
Author(s):  
Jenny R. Wang ◽  
Heng W. Yuen ◽  
David B. Shipp ◽  
Suzanne Stewart ◽  
Vincent Y. W. Lin ◽  
...  
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Cogan Syndrome ◽  
Inner Ear Disease ◽  
Autoimmune Inner Ear Disease ◽  
Age And Sex

Cochlear Implantation in Autoimmune Inner Ear Disease

2008 ◽  
Vol 139 (2_suppl) ◽  
pp. P53-P53 ◽  
Author(s):  
Katrina R Stidham ◽  
Joseph B Roberson
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Immunosuppressive Therapy ◽  
Cochlear Implantation ◽  
Hearing Aid ◽  
Speech Discrimination ◽  
Inner Ear Disease ◽  
Potential Risks ◽  
The Impact ◽  
Autoimmune Inner Ear Disease

Objective 1) Evaluate the success of cochlear implantation (CI) in patients with autoimmune inner ear disease (AIED). 2) Discuss the impact of CI on consideration of immunosuppressive therapy for patients with fluctuating hearing loss due to AIED. Methods Retrospective chart review was conducted of patients undergoing CI with diagnosis of AIED between 6/02 and 6/07. Patients’ autoimmune and immunotherapy treatment history was explored. Non-operative ear hearing thresholds and speech discrimination scores (SDS) were documented pre- and postoperatively. Objective and subjective implant success in setting of fluctuating hearing in non-implant ear was evaluated. Results Four patients with AIED, aged 5–55, underwent CI. Two had isolated otologic symptoms and 2 had other systemic autoimmune diagnoses. All had been previously treated with steroids. Three had received other immunosuppressive therapy. One declined other treatment due to potential risks. At the time of implantation, 3 patients had functional hearing in the non-implant ear and received benefit from a hearing aid. Each continued to have usable hearing, with fluctuating improvements post-implant at times to much better SDS than would normally meet candidacy requirements for CI. All patients found the implant provided better hearing than the hearing-aid ear. Conclusions CI is a viable option for patients with AIED. Implanting a profoundly deafened ear where opposite ear has fluctuating but usable hearing does not negatively impact outcomes. With the success of CI for AIED, the potential risks of long-term immunosuppressive therapy may not warrant its use in patients with isolated hearing loss symptoms.

The pathogenesis of the secondary forms of autoimmune inner ear disease (AIED): advancing beyond the audiogram data

2021 ◽  
Author(s):  
Virginia Corazzi ◽  
Stavros Hatzopoulos ◽  
Chiara Bianchini ◽  
Magdalena B Skarżyńska ◽  
Stefano Pelucchi ◽  
...  
Keyword(s):  
Inner Ear ◽  
Inner Ear Disease ◽  
Autoimmune Inner Ear Disease

Cochlear Implantation Outcomes in Patients With Autoimmune and Immune-Mediated Inner Ear Disease

2010 ◽  
Vol 31 (8) ◽  
pp. 1337-1342 ◽  
Author(s):  
Saba Aftab ◽  
Maroun T. Semaan ◽  
Gail S. Murray ◽  
Cliff A. Megerian
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Immune Mediated ◽  
Inner Ear Disease

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.

scholarly journals Treatment of Corticosteroid-Responsive Autoimmune Inner Ear Disease With Methotrexate

JAMA ◽  
2003 ◽  
Vol 290 (14) ◽  
pp. 1875 ◽  
Author(s):  
Jeffrey P. Harris ◽  
Michael H. Weisman ◽  
Jennifer M. Derebery ◽  
Mark A. Espeland ◽  
Bruce J. Gantz ◽  
...  
Keyword(s):  
Inner Ear ◽  
Inner Ear Disease ◽  
Autoimmune Inner Ear Disease

scholarly journals Cogan's syndrome: An autoimmune inner ear disease

2013 ◽  
Vol 12 (3) ◽  
pp. 396-400 ◽  
Author(s):  
A. Greco ◽  
A. Gallo ◽  
M. Fusconi ◽  
G. Magliulo ◽  
R. Turchetta ◽  
...  
Keyword(s):  
Inner Ear ◽  
Cogan’S Syndrome ◽  
Cogan's Syndrome ◽  
Inner Ear Disease ◽  
Autoimmune Inner Ear Disease
Sign in / Sign up

Export Citation Format

Share Document