Familial neural mitochondrial deafness
Marangos, N.; Mausolf, A.
Hno 38(9): 322-325
1990
ISSN/ISBN: 0017-6192 PMID: 2262349 Document Number: 356108
Mitochondrial abnormalities are known to cause several neurological syndromes that often include hearing loss as one of their features. We present two brothers with mitochondrial cytopathy and hearing loss. The audiological and electrocochleographic findings suggest a neural origin for the hearing impairment. Muscle biopsy is an important tool for the diagnosis of these syndromes in patients with audiological evidence of a bilateral neural hearing loss and neurological abnormalities.
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