Audiological assessment in patients with mitochondrial myopathy

Abstract

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.

Cochlear Dysfunction in Patients with Mitochondrial Myopathy

“…From the combination of all the results, it appears that in most of the subjects retrocochlear involvement seems possible, because of ABR abnormalities, while in 3 of them cochlear damage could also be hypothesized. These data agree with the results of our previous work and have been previously discussed in detail [7].…”

“…Sensorineural hearing loss is a common finding in patients with the disease, although in most of the extensive relevant literature it is only incidentally mentioned. In a previous study, Korres et al [7] investigated the audiological features in a group of 14 patients with mitochondrial disease. Audiological data in those patients was inconclusive regarding the site of lesion and in most cases a multiple involvement rather than a pure cochlear or retrocochlear damage was suspected, as supported from the diversity of the audiological findings.…”

“…Sensorineural hearing loss is mentioned in the literature as a common symptom of mitochondrial myopathies and several audiological studies have been performed, in order to confirm the clinical observations [4][5][6][7]. Recording of otoacoustic emissions is a diagnostic method widely used during the last decade, in order to study the detailed mechanical aspects of cochlear function, in a non-invasive and objective manner [8].…”

Increased nuclear factor-κB and loss of p53 are key mechanisms in Myalgic Encephalomyelitis/chronic fatigue syndrome (ME/CFS)

“…Audiological symptoms are quite common with tinnitus and sensorineural hearing loss [106,112,115,116]. Blurred vision, photophobia, nyctalopia, corneal edema, external ophthalmoplegia, visual impairment or even blindness due to cataracts or optic atrophy are some of the visual/ocular symptoms that are associated with mitochondrial aberrations [106,109,[117][118][119].…”