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Different clinical characteristics of aminoglycoside-induced profound deafness with and without the 1555 A-->G

T Tono1, K Kiyomizu, K Matsuda

  • 1Department of Otorhinolaryngology, Miyazaki Medical College, Miyazaki, Japan. tono@post1.miyazaki-med.ac.jp

ORL; Journal for Oto-Rhino-Laryngology and Its Related Specialties
|February 15, 2001
PubMed

Insights

The 1555 A-->G mitochondrial mutation causes hereditary hearing loss from aminoglycoside antibiotics. This mutation was found in 4 deaf patients, showing maternal transmission and distinct hearing loss characteristics.

Area of Science:

  • Genetics
  • Otolaryngology
  • Mitochondrial Medicine

Background:

  • Hereditary susceptibility to aminoglycoside antibiotics is linked to the 1555 A-->G mitochondrial mutation.
  • Aminoglycosides can cause profound, irreversible hearing loss.

Purpose of the Study:

  • To investigate the prevalence of the 1555 A-->G mitochondrial mutation in postlingual deaf patients undergoing cochlear implantation.
  • To characterize the neuro-otological findings in patients with the 1555 mutation compared to those without.

Main Methods:

  • Genetic screening for the 1555 A-->G mitochondrial mutation in 68 postlingual deaf patients.
  • Analysis of patient pedigrees for maternal transmission patterns.
  • Comparison of neuro-otological findings, including hearing loss progression, pure-tone thresholds, electrical promontory stimulation, and vestibular function.

Main Results:

  • The 1555 mutation was identified in 4 out of 68 patients, all of whom experienced bilateral profound hearing loss after aminoglycoside exposure.
  • Maternal transmission of hearing impairment was observed in all affected families.
  • Patients with the 1555 mutation exhibited progressive hearing loss, better residual pure-tone thresholds, lower electrical promontory stimulation thresholds, and preserved vestibular function compared to non-mutation carriers.

Conclusions:

  • The 1555 A-->G mitochondrial mutation is a significant genetic factor in aminoglycoside-induced hearing loss.
  • The distinct clinical presentation suggests a different pathogenic mechanism, potentially involving strial dysfunction rather than direct hair cell damage.

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