Mitochondrial DNA Mutation at Nucleotide 1555 in a Patient with Bilateral Sensorineural Hearing Loss of Unknown Etiology

The A1555G mitochondrial deoxyribonucleic acid (mtDNA) point mutation has classically been associated with sensorineural hearing loss in patients following aminoglycoside exposure. More recently, the mutation has been implicated in sensorineural hearing loss in patients without previous aminoglycoside use. In addition, cochlear implantation has been shown to be effective in the group of patients with prior aminoglycoside exposure but, to date, no case of cochlear implantation in a patient with the A1555G mutation and no prior exposure to aminoglycosides has been explicitly described in the literature.We report the case of an 80-year-old woman with the A1555G mtDNA mutation, a 35-year history of bilateral progressive hearing loss and no history of aminoglycoside exposure who underwent successful implantation of a Nucleus 24 Contour device at our institution. Post-operatively, the patient exhibited marked improvement in tests of auditory performance.We conclude that cochlear implantation can be an effective method to restore some sense of hearing in patients with the A1555G mtDNA mutation and sensorineural hearing loss.

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Biological age and rates of aging of patients with vibration disease and bilateral sensorineural hearing loss

Russian Journal of Occupational Health and Industrial Ecology ◽

10.31089/1026-9428-2019-59-9-624-625 ◽

2020 ◽

pp. 624-624

Author(s):

S. I. Ereniev ◽

O. V. Plotnikova

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Sensorineural Hearing ◽

Biological Age ◽

Biological Aging ◽

Vibration Disease ◽

Bilateral Sensorineural Hearing Loss

Biological age and rates of aging of patients with vibration disease and bilateral sensorineural hearing loss were studied. The biological age of patients exceeded the calendar age by an average of 7.36±0.36 years and the proper biological age by 10.79±0.72 years. The rate of biological aging of the examined patients was 1.14±0.08 times higher than the rate of aging of their healthy peers.

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Sudden bilateral sensorineural hearing loss associated with urticarial vasculitis

The Journal of Laryngology & Otology ◽

10.1017/s0022215113001047 ◽

2013 ◽

Vol 127 (7) ◽

pp. 708-711 ◽

Cited By ~ 3

Author(s):

A C Hall ◽

A C Leong ◽

D Jiang ◽

A Fitzgerald-O'Connor

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Cochlear Implantation ◽

Skin Lesions ◽

Sudden Hearing Loss ◽

Sudden Onset ◽

Sensorineural Hearing ◽

Urticarial Vasculitis ◽

Wells Syndrome ◽

Bilateral Sensorineural Hearing Loss

AbstractBackground:Bilateral sensorineural hearing loss associated with recurrent urticarial skin lesions may be signs of underlying Muckle–Wells syndrome. Previous reports have described the hearing loss to be progressive in nature.Method:To our knowledge, this paper presents the first published case of sudden onset, bilateral sensorineural hearing loss associated with urticarial vasculitis due to underlying Muckle–Wells syndrome.Results:The patient underwent a cochlear implantation with a modest outcome.Conclusion:Cochlear implantation may help to rehabilitate sudden hearing loss associated with this condition, but early diagnosis may allow treatment with interleukin-1β inhibitors such as anakinra.

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