Audiological assessment in patients with mitochondrial myopathy.
Mitochondrial myopathies comprise a heterogeneous group of disorders characterized by the presence of ragged red fibres in muscle biopsy. The present study investigates the audiological features in a group of 14 patients in whom diagnosis was histologically confirmed. A complete ENT, neurological and audiological work-up was performed for every patient, including measurement of brainstem auditory evoked potentials (BAEPs). Most patients presented with a varying degree of hearing loss and occasional abnormalities of the latencies and interpeak latencies of the BAEPs, but only the increased latencies of waves I and V have been statistically confirmed. Audiological data suggest non-specific involvement at one or more sites along the auditory pathway.
Korres
S G
SG
Department of Neurology of Athens National University, Eginition Hospital, Athens, Greece.
Manta
P B
PB
Balatsouras
D G
DG
Papageorgiou
C T
CT
D000328:Adult; D000368:Aged; D001301:Audiometry, Pure-Tone / Q000379:methods; D001306:Auditory Pathways / Q000473:pathology; D001309:Auditory Threshold / Q000502:physiology; D001706:Biopsy; D003051:Cochlea / Q000000981:diagnostic imaging / Q000473:pathology; D004569:Electroencephalography; D004576:Electromyography; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D005260:Female; D006311:Hearing Disorders / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D017240:Mitochondrial Myopathies / Q000150:complications* / Q000175:diagnosis; D018482:Muscle, Skeletal / Q000473:pathology; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed