Cochlear origin of hearing loss in MELAS syndrome

No abstract is available for this paper.

• Publication year

  1998

• Venue

  Annals of Neurology

• Publication date

  1998-03-01

• Fields of study

  Medicine

• Identifiers
  DOI 10.1002/ana.410430313PMID 9506552
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

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  1996cited by this paper
• Seminars in medicine of the Beth Israel Hospital, Boston. Mitochondrial DNA and disease.

  1995cited by this paper
• Rapid and noninvasive screening of patients with mitochondrial myopathy

  1994cited by this paper
• Maternally inherited diabetes and deafness is a distinct subtype of diabetes and associates with a single point mutation in the mitochondrial tRNA Leu(UUR) gene

  1994cited by this paper
• New techniques of hearing assessment.

  1994cited by this paper
• Distortion product otoacoustic emissions and sensorineural hearing loss.

  1994cited by this paper
• Screening all newborns for hearing loss using transient evoked otoacoustic emissions.

  1994cited by this paper
• Adult‐onset diabetes mellitus and neurosensory hearing loss in maternal relatives of MELAS patients in a family with the tRNALeu(UUR) mutation

  1993cited by this paper
• Mitochondrial gene mutations and diabetes mellitus.

  1993cited by this paper
• Mitochondrial gene mutations and diabetes mellitus.

  1993cited by this paper
• Mitochondrial encephalomyopathy: variable clinical expression within a single kindred.

  1993cited by this paper
• Maternally transmitted diabetes and deafness associated with a 10.4 kb mitochondrial DNA deletion

  1992cited by this paper
• Distortion-product and click-evoked otoacoustic emissions of normally-hearing adults.

  1992cited by this paper
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  1992cited by this paper
• Distortion-product emissions and auditory sensitivity in human ears with normal hearing and cochlear hearing loss.

  1992cited by this paper
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  1991cited by this paper
• Audiologic findings in a family with mitochondrial disorder.

  1991cited by this paper
• Immunocytochemical and enzyme histochemical localization of Na+,K(+)-ATPase in normal and ischemic porcine myocardium.

  1990cited by this paper
• Distribution of immunoreactive Na+,K+-ATPase in gerbil cochlea.

  1989cited by this paper
• Myoclonus epilepsy and ragged-red fibres (MERRF). 1. A clinical, pathological, biochemical, magnetic resonance spectrographic and positron emission tomographic study.

  1989cited by this paper
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  1989cited by this paper
• Evoked otoacoustic emissions and sensorineural hearing loss.

  1989cited by this paper
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  1989cited by this paper
• Positive endocochlear potential: mechanism of production by marginal cells of stria vascularis.

  1987cited by this paper
• Evoked potentials in clinical medicine.

  1986cited by this paper
• The clinical features of mitochondrial myopathy.

  1986cited by this paper
• Genetic factors affecting hearing development.

  1985cited by this paper
• Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes: A distinctive clinical syndrome

  1984cited by this paper
• Diagnostic Significance of Transtympanic Electrocochleography in Menière's Disease

  1983cited by this paper
• An Introduction to the Physiology of Hearing

  1982cited by this paper
• Essentials of clinical electric response audiometry

  1978cited by this paper
• Stimulated acoustic emissions from within the human auditory system.

  1978cited by this paper
• Lumping or splitting? “ophthalmoplegia‐plus” or kearns‐sayre syndrome?

  1977cited by this paper
• Histopathologic features of the inner ear associated with Kearns-Sayre syndrome.

  1976cited by this paper
• Postnatal development of endocochlear potential and stria vascularis in the cat.

  1974cited by this paper
• Sensorineural Hearing Loss

  1972cited by this paper
• Evaluation of adenosine triphophatase activity in the stria vascularis and spiral ligament of normal guinea pigs.

  1967cited by this paper
• Stria vascularis as source of endocochlear potential.

  1959cited by this paper
• Para-acetaminobenzaldehyde thiosemicarbazone in the treatment of leprosy.

  1952cited by this paper

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• [Research progress on hereditary endocrine and metabolic diseases associated with sensorineural hearing loss].

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• [Maternally inherited diabetes and deafness: a case report].

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  2004cites this paper
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  2004cites this paper
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  2004cites this paper
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  2003cites this paper
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  2003cites this paper
• Mitochondrial Hearing Loss

  2003cites this paper
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  2003influential citation
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  2003cites this paper
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  2003cites this paper
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  2003cites this paper
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  2003influential citation