Literature DB >> 15236904

Hearing evaluation in two sisters with a T8993G point mutation of mitochondrial DNA.

Yuki Sakai1, Kimitaka Kaga, Kazuo Kodama, Asako Higuchi, Junko Miyamoto.   

Abstract

We report on two sisters with a T8993G point mutation of mitochondrial DNA, and their hearing evaluation. Considering auditory function, hearing in the elder sister remains almost normal. However, in the younger sister, the auditory brainstem response (ABR) threshold has fluctuated remarkably during a 3-year follow-up. The threshold changes of ABR in the younger sister suggest that her hearing problems may well be caused by both cochlear nerves and retrocochlear lesions. Our experience is clinically important because there have been only a few reports on hearing evaluation in patients with a T8993G point mutation of mitochondrial DNA.

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Year:  2004        PMID: 15236904     DOI: 10.1016/j.ijporl.2004.03.015

Source DB:  PubMed          Journal:  Int J Pediatr Otorhinolaryngol        ISSN: 0165-5876            Impact factor:   1.675


  2 in total

1.  Cochlear Implantation in Patients with Mitochondrial Gene Mutation: Decline in Speech Perception in Retrospective Long-Term Follow-Up Study.

Authors:  Kai Kanemoto; Akinori Kashio; Erika Ogata; Yusuke Akamatsu; Hajime Koyama; Tsukasa Uranaka; Yujiro Hoshi; Shinichi Iwasaki; Tatsuya Yamasoba
Journal:  Life (Basel)       Date:  2022-03-26

Review 2.  ATP Synthase Diseases of Mitochondrial Genetic Origin.

Authors:  Alain Dautant; Thomas Meier; Alexander Hahn; Déborah Tribouillard-Tanvier; Jean-Paul di Rago; Roza Kucharczyk
Journal:  Front Physiol       Date:  2018-04-04       Impact factor: 4.566

  2 in total

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