title: Inner ear dysfunction in myotonic dystrophy type 1.
abstract: OBJECTIVE
  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).
  
  
  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.
  
  
  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.
authors.affiliation: ENT Department, Tzanion General Hospital, Piraeus, Greece. dbalats@hotmail.com
authors.forename: D G
authors.initials: DG
authors.lastname: Balatsouras
authors.forename: D
authors.initials: D
authors.lastname: Felekis
authors.forename: M
authors.initials: M
authors.lastname: Panas
authors.forename: J
authors.initials: J
authors.lastname: Xenellis
authors.forename: G
authors.initials: G
authors.lastname: Koutsis
authors.forename: A
authors.initials: A
authors.lastname: Kladi
authors.forename: S G
authors.initials: SG
authors.lastname: Korres
mesh_terms: D000158:Acoustic Impedance Tests; D000328:Adult; D001301:Audiometry, Pure-Tone; D003051:Cochlea / Q000503:physiopathology; D007758:Ear, Inner / Q000503:physiopathology*; D004584:Electronystagmography; D016057:Evoked Potentials, Auditory, Brain Stem; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000209:etiology* / Q000503:physiopathology; D006801:Humans; D006987:Hypesthesia / Q000209:etiology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000503:physiopathology*; D009759:Nystagmus, Pathologic / Q000209:etiology* / Q000503:physiopathology; D017084:Otoacoustic Emissions, Spontaneous; D015995:Prevalence; D014722:Vestibule, Labyrinth / Q000503:physiopathology