Sensorineural Hearing Loss and the 1555G Mitochondrial DNA Mutation

1999 ◽  
Vol 119 (1) ◽  
pp. 48-52 ◽  
Author(s):  
Tim Hutchin
2009 ◽  
Vol 390 (3) ◽  
pp. 755-757 ◽  
Author(s):  
Haris Kokotas ◽  
Maria Grigoriadou ◽  
George S. Korres ◽  
Elisabeth Ferekidou ◽  
Eleftheria Papadopoulou ◽  
...  

2003 ◽  
Vol 46 (6) ◽  
pp. 595-600
Author(s):  
Shunsuke Miyamoto ◽  
Hajime Sano ◽  
Yuichi Ono ◽  
Mitsukuni Nitta ◽  
Makito Okamoto

2005 ◽  
Vol 120 (3) ◽  
pp. 230-232 ◽  
Author(s):  
S Arif Ulubil ◽  
Alexis D Furze ◽  
Simon I Angeli

The A1555G mitochondrial deoxyribonucleic acid (mtDNA) point mutation has classically been associated with sensorineural hearing loss in patients following aminoglycoside exposure. More recently, the mutation has been implicated in sensorineural hearing loss in patients without previous aminoglycoside use. In addition, cochlear implantation has been shown to be effective in the group of patients with prior aminoglycoside exposure but, to date, no case of cochlear implantation in a patient with the A1555G mutation and no prior exposure to aminoglycosides has been explicitly described in the literature.We report the case of an 80-year-old woman with the A1555G mtDNA mutation, a 35-year history of bilateral progressive hearing loss and no history of aminoglycoside exposure who underwent successful implantation of a Nucleus 24 Contour device at our institution. Post-operatively, the patient exhibited marked improvement in tests of auditory performance.We conclude that cochlear implantation can be an effective method to restore some sense of hearing in patients with the A1555G mtDNA mutation and sensorineural hearing loss.


1998 ◽  
Vol 6 (6) ◽  
pp. 563-569 ◽  
Author(s):  
Satoko Abe ◽  
Shin-ichi Usami ◽  
Hideichi Shinkawa ◽  
MikeD Weston ◽  
LarryD Overbeck ◽  
...  

2005 ◽  
Vol 132 (6) ◽  
pp. 890-895 ◽  
Author(s):  
Simon I. Angeli ◽  
Denise Yan ◽  
Fred Telischi ◽  
Thomas J. Balkany ◽  
Xiao M. Ouyang ◽  
...  

OBJECTIVE: To determine the etiology of adult-onset sensorineural hearing loss. STUDY DESIGN AND SETTING: This is a prospective cohort study of 60 adult subjects with bilateral sensorineural hearing loss of no obvious etiology by medical history and physical examination. These patients were evaluated at an academic medical center and underwent evaluation by high-resolution computed tomography of the temporal bone, autoimmune panel, and DNA testing for mutations of both the GJB2 gene and the mitochondrial DNA (1555A>G and 7445A>G). RESULTS: An etiologic diagnosis was achieved in 6 patients: cochlear otosclerosis, 1 case; dilated vestibular aqueduct, 1 case; a mitochondrial DNA 7445A>G mutation, 3 cases; and a mitochondrial DNA 1555A>G mutation, 1 case. CONCLUSION: This result underscores the importance of a search for the etiology of a hearing deficit in adult patients. There are specific interventions now available for the management of hearing-impaired patients with cochlear otosclerosis and mitochondrial DNA mutations.


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