OBJECTIVE: To examine the audiometric patterns of @entity393 due to the @entity14505 mutation in the mitochondrial DNA. @entity1 AND METHODS: We include 55 subjects with the @entity14505 mutation from 6 unrelated families, affected by nonsyndromic @entity1274 and residing in Cantabria. The @entity14505 mutation was found in homoplasmy in all the families, except in one family, in which it was in heteroplasmy. Aside from standard history taking and general otolaryngological examination, pure tone audiometry was carried out in all @entity1 . RESULTS: @entity393 was developed by most of the @entity1 . The auditory defect was a slowly progressive bilateral symmetrical @entity1274 , affecting mainly the high frequencies. In @entity1 in which @entity4028 @entity2167 could be excluded, @entity393 usually ranged from mild to moderate, with a late onset. In 17 cases there were previous history of treatment with a @entity2167 drugs, and most of them developed severe @entity393 . One of them was deaf-mute. No audometric differences between families with the homoplasmic and the heteroplasmic @entity14505 mutation were observed. CONCLUSIONS: @entity1 with the @entity14505 mutation generally present bilateral symmetrical @entity1274 , ranging from mild to moderate, slowly progressive, which is obvious approximately in the second decade of life and affects specially the high frequencies. @entity393 severity is increased by treatment with @entity581 .

[Audiometric features of XXXX transmitted by mitochondrial inheritance ( @entity14505 )].

@entity1 :: ('9606', 'Species') :: ['Patients', 'patients', 'PATIENTS'] @entity2167 :: ('MESH:D006311', 'Disease') :: ['ototoxic', 'ototoxicity'] @entity1274 :: ('MESH:D006319', 'Disease') :: ['sensorineural hearing loss'] @entity581 :: ('MESH:D000617', 'Chemical') :: ['aminoglycosides'] @entity14505 :: ('c|SUB|A|1555|G', 'DNAMutation') :: ['A1555G'] @entity393 :: ('MESH:D034381', 'Disease') :: ['familial hearing impairment', 'hearing loss', 'Hearing loss'] @entity4028 :: ('CHEBI:47779', 'Chemical') :: ['aminoglycoside']
@entity393 :: (MESH:D034381,Disease) :: ['familial hearing impairment']
