Cochlear Implantation for Symptomatic Hereditary Deafness

Nishizaki, Kazunori; Fukushiama, Kunihiro; Oda, Yukinobu; Masuda, Akemi; Hayashi, S; Nagayasu, Norie; Yoshino, Tadashi; Kashihara, Kenichi; Takahashi, Koji; Masuda, Yu

doi:10.1080/00016489950181161

Cited by 6 publications

(2 citation statements)

References 20 publications

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“…Previous studies [22][23][24][25][26][27][28][29][30][31][32][33][34][35][36][37]40,41] and short-term observations in the present study indicate that patients with mtDNA mutations are good candidates for CI. Notably, however, the long-term observations in the present study also suggest that retrocochlear dysfunction may be responsible for the long-term deterioration of speech perception after CI in patients with mtDNA mutations.…”

Section: Discussionsupporting

confidence: 64%

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

Kanemoto

Kashio

Ogata

et al. 2022

Life

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Clinical evidence of the effectiveness of cochlear implantation for hearing loss with mitochondrial DNA mutation is limited. Most reports have only described short-term postoperative speech perception, which may not reflect the limitations of cochlear implantation caused by progressive retrocochlear dysfunction. The present study aimed to investigate long-term speech perception after cochlear implantation in patients with severe to profound hearing loss associated with mitochondrial DNA mutation. A retrospective chart review was performed on patients with mitochondrial DNA mutation who had undergone cochlear implantation at the Department of Otolaryngology and Head and Neck Surgery at the University of Tokyo Hospital. We extracted data on causative mutations, clinical types, clinical course, perioperative complications, and short-term and long-term postoperative speech perception. Nine patients with mitochondrial DNA mutation underwent cochlear implantation. The mean observation period was 5.5 ± 4.2 years (range, 1–13 years), and seven patients were followed for more than 3 years. Two of the seven patients who initially showed good speech perception exhibited deterioration during long-term follow-up. The absence of an acute progression of cognitive decline in patients, showing a gradual decrease in speech perception, suggests that the deterioration of speech perception was caused by progressive retrocochlear degeneration. Although most patients with mitochondrial DNA mutation maintained good speech perception for more than 3 years after cochlear implantation, retrocochlear degeneration could cause the deterioration of speech perception during long-term follow-up.

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Section: Discussionsupporting

confidence: 64%

“…Cochlear implantation (CI) for patients with severe to profound hearing loss associated with mtDNA mutations has been reported [19,[21][22][23][28][29][30][31][32][33][34][35]. Howes et al [36] reported a case of MIDD with a speech score of 67% at one-month follow-up.…”

Section: Introductionmentioning

confidence: 99%

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

Kanemoto

Kashio

Ogata

et al. 2022

Life

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Sensorineural hearing loss in patients with chronic progressive external ophthalmoplegia or Kearns–Sayre syndrome

et al. 2005

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In the present study we assessed the prevalence and nature of hearing loss in patients with chronic progressive external ophthalmoplegia (CPEO) or Kearns-Sayre syndrome (KSS) due to single large-scale mitochondrial DNA (mtDNA) deletion or mtDNA tRNA (Leu (UUR)) A3243G point mutation (A3243G PM). 14 patients with mtDNA deletion and three patients with A3243G PM underwent audiological evaluation comprising pure-tone and speech audiometry as well as transient evoked otoacoustic emissions (OAE). Audiological evaluation revealed hearing impairment in 10/17 patients. Hearing loss was mild to moderate predominantly affecting high frequencies in five patients with subjective hearing problems (three patients with mtDNA deletions, two patients with A3243G PM). Subclinical hearing deficits restricted to high frequencies were seen in further five asymptomatic patients (four patients with mtDNA deletions, one patients with A3243G PM). Audiological findings suggested a cochlear origin of hearing loss in all subjects. Our results demonstrate that CPEO or KSS patients due to mtDNA deletion or A3243G PM are at high risk of developing sensorineural hearing deficits.

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