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Inner ear dysfunction in myotonic dystrophy type 1
Author(s) -
Balatsouras D. G.,
Felekis D.,
Panas M.,
Xenellis J.,
Koutsis G.,
Kladi A.,
Korres S. G.
Publication year - 2013
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/ane.12020
Subject(s) - audiology , medicine , myotonic dystrophy , tympanometry , audiometry , pure tone audiometry , subclinical infection , electronystagmography , vestibular system , otoacoustic emission , auditory neuropathy , vestibular evoked myogenic potential , hearing loss
Objectives 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 ( TEOAE s). 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 ( ABR s), TEOAE s, and electronystagmography. Results 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%). Conclusions 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.