Cochlear Implantation in a Patient with Mitochondrial Disease-Kearns-Sayre Syndrome: A Case Report

Author(s):  
T. Yamaguchi ◽  
T. Himi ◽  
Y. Harabuchi ◽  
M. Hamamoto ◽  
A. Kataura
1996 ◽  
Vol 89 (10) ◽  
pp. 1195-1199 ◽  
Author(s):  
Tomo YAMAGUCHI ◽  
Tetsuo HIMI ◽  
Yasuaki HARABUCHI ◽  
Makoto HAMAMOTO ◽  
Yuji YOKOYAMA ◽  
...  

2020 ◽  
Vol 6 (6) ◽  
pp. 271-272
Author(s):  
Reshmi Mishra ◽  

Kearns–Sayre syndrome (KSS) is a rare mitochondrial disease was first described in 1958. The characteristic triad is age of onset less than 20 years, progressive external ophthalmoplegia, pigmentary retinopathy, The prevalence rate of KSS is nearly 1–3 per 100 000 individuals. Here, we report a rare case of a 11-year-old male with KSS.


2012 ◽  
Vol 126 (11) ◽  
pp. 1172-1175 ◽  
Author(s):  
Y M Feng ◽  
Y Q Wu ◽  
J Wang ◽  
S K Yin

AbstractObjective:We report the case of a successful cochlear implantation in a patient with severe cochlear hypoplasia.Case report:The outcome of cochlear implantation is generally less favourable for patients with cochlear hypoplasia than for those with a normal cochlear structure. In the reported patient, part of the electrode array was inserted into the internal auditory canal. Nevertheless, the benefits following cochlear implantation seemed to outweigh the risks for this patient.Conclusion:Cochlear hypoplasia is not necessarily a contraindication for cochlear implantation.


Author(s):  
D.J. Mawman ◽  
R.T. Ramsden ◽  
M. O�Driscoll ◽  
T. Adams ◽  
S.R. Saeed

2008 ◽  
Vol 123 (7) ◽  
pp. 811-816 ◽  
Author(s):  
A D Mace ◽  
M S Ferguson ◽  
M Offer ◽  
K Ghufoor ◽  
M J Wareing

AbstractObjective:To present the first published case of a child with bilateral profound sudden sensorineural hearing loss found in association with sickle cell anaemia, and to demonstrate the importance of early recognition, investigation and empirical treatment of sudden sensorineural hearing loss.Method:Case report and review of world literature.Case report:The authors present the case of a seven-year-old child with known sickle cell anaemia, who presented with bilateral profound sensorineural hearing loss developing over a period of five days. There was a history of ophthalmological disease in the preceding weeks, and inflammatory markers were raised. The differential diagnosis included a vaso-occlusive or inflammatory aetiology such as Cogan's syndrome, and treatment for both was instigated. Hearing thresholds did not recover, and the patient underwent cochlear implantation 12 weeks later.Conclusion:Sudden sensorineural hearing loss has a variable aetiology and is rare in children. Immediate treatment for all possible aetiologies is essential, along with targeted investigations and early referral for cochlear implantation if no recovery is demonstrated.


2002 ◽  
Vol 21 (5) ◽  
pp. 411-414 ◽  
Author(s):  
A. Corrado ◽  
F. P. Cantatore ◽  
L. Serlenga ◽  
A. Amati ◽  
V. Petruzzella ◽  
...  

2011 ◽  
Vol 125 (7) ◽  
pp. 741-744 ◽  
Author(s):  
Y-M Feng ◽  
Y-Q Wu ◽  
H-Q Zhou ◽  
H-B Shi

AbstractObjective:We report a patient who underwent cochlear implantation in an ear with long-term deafness, after an acoustic neuroma had been removed surgically from the other, hitherto good ear and the cochlear nerve had subsequently been resected to relieve severe tinnitus.Method:Case report.Results:The patient could not tolerate the cochlear implant, because of a moderate headache due to the stimulation level necessary for environmental sound discrimination.Conclusion:Cochlear implantation in patients with long-term deafness should be considered carefully, even if deafness is monaural.


2012 ◽  
Vol 126 (4) ◽  
pp. 414-417 ◽  
Author(s):  
E Muzzi ◽  
P Boscolo-Rizzo ◽  
R Santarelli ◽  
M A Beltrame

AbstractObjective:To report a series of pitfalls and complications in a case of cochlear implantation.Method:Case report.Results:An 11-year-old boy affected by auditory neuropathy underwent cochlear implantation. Intra-operative assessment was apparently consistent with correct insertion of the electrode array into the cochlea. However, subsequent high resolution computed tomography revealed that the entire electrode array was curled up within the vestibule. Revision surgery was complicated by cerebrospinal fluid leakage. A straight probe was repeatedly inserted into the internal auditory canal, before conversion to a canal wall down procedure and appropriate positioning of the electrode array.Conclusion:In this case, mild anteriorisation of the facial nerve created an awkward insertion angle for the electrode array via the retro-facial route, which may have triggered the described series of adverse events.


Sign in / Sign up

Export Citation Format

Share Document