rss
J Neurol Neurosurg Psychiatry 75:626-630 doi:10.1136/jnnp.2003.017673
  • Paper

Progressive auditory neuropathy in patients with Leber’s hereditary optic neuropathy

  1. B Ćeranić,
  2. L M Luxon
  1. Department of Neuro-otology, The National Hospital for Neurology and Neurosurgery, Queen Square, London WC1, UK
  1. Correspondence to:
 Dr Borka Ćeranić
 Department of Neuro-otology, Box 127, The National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG; borka.ceranicuclh.org
  • Received 28 April 2003
  • Accepted 6 August 2003
  • Revised 31 July 2003

Abstract

Objective: To investigate auditory neural involvement in patients with Leber’s hereditary optic neuropathy (LHON).

Methods: Auditory assessment was undertaken in two patients with LHON. One was a 45 year old woman with Harding disease (multiple-sclerosis-like illness and positive 11778mtDNA mutation) and mild auditory symptoms, whose auditory function was monitored over five years. The other was a 59 year old man with positive 11778mtDNA mutation, who presented with a long standing progressive bilateral hearing loss, moderate on one side and severe to profound on the other. Standard pure tone audiometry, tympanometry, stapedial reflex threshold measurements, stapedial reflex decay, otoacoustic emissions with olivo-cochlear suppression, auditory brain stem responses, and vestibular function tests were undertaken.

Results: Both patients had good cochlear function, as judged by otoacoustic emissions (intact outer hair cells) and normal stapedial reflexes (intact inner hair cells). A brain stem lesion was excluded by negative findings on imaging, recordable stapedial reflex thresholds, and, in one of the patients, olivocochlear suppression of otoacoustic emissions. The deterioration of auditory function implied a progressive course in both cases. Vestibular function was unaffected.

Conclusions: The findings are consistent with auditory neuropathy—a lesion of the cochlear nerve presenting with abnormal auditory brain stem responses and with normal inner hair cells and the cochlear nucleus (lower brain stem). The association of auditory neuropathy, or any other auditory dysfunction, with LHON has not been recognised previously. Further studies are necessary to establish whether this is a consistent finding.

Footnotes

  • Competing interests: none declared

Responses to this article

Podcasts
Visit the full archive of podcasts for JNNP here >>

Free sample
This recent issue is free to all users to allow everyone the opportunity to see the full scope and typical content of JNNP.
View free sample issue >>

Don't forget to sign up for content alerts so you keep up to date with all the articles as they are published.

Navigate This Article