Inner ear dysfunction in myotonic dystrophy type 1
Article first published online: 5 NOV 2012
© 2012 John Wiley & Sons A/S
Acta Neurologica Scandinavica
Volume 127, Issue 5, pages 337–343, May 2013
How to Cite
Inner ear dysfunction in myotonic dystrophy type 1. Acta Neurol Scand: DOI: 10.1111/ane.12020. © 2012 John Wiley & Sons A/S., , , , , , .
- Issue published online: 13 APR 2013
- Article first published online: 5 NOV 2012
- Manuscript Accepted: 21 SEP 2012
- hearing loss;
- myotonic dystrophy;
- otoacoustic emissions;
Myotonic dystrophy type 1 is associated with various oculomotor, vestibular, and auditory abnormalities. However, auditory system investigation has been mainly performed with the subjective method of pure-tone audiometry. In this study, a detailed vestibular and audiological evaluation was undertaken, including the objective and more sensitive method of transiently evoked otoacoustic emissions (TEOAEs).
Materials and methods
Twenty-four patients with genetically diagnosed myotonic dystrophy type 1 and 21 controls were studied. Audiological and vestibular investigations included pure-tone audiometry, tympanometry, auditory brainstem responses (ABRs), TEOAEs, and electronystagmography.
Hearing impairment was evident in 15 (62.5%) patients and in nine of them (37.5%) ABR abnormalities were found. However, subclinical cochlear damage was found in all patients, as evidenced by absent emissions or lower otoacoustic emission amplitude. Vestibular hypesthesia was found in nine patients (37.5%), accompanied by spontaneous nystagmus in four of them (15.6%).
Auditory and vestibular abnormalities are quite common in patients with myotonic dystrophy type 1. However, it appears that subclinical cochlear damage is an ubiquitous finding of the disease.