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Article dans une revue Neurology Année : 2018

Hearing impairment in patients with myotonic dystrophy type 2

Judith van Vliet
  • Fonction : Auteur
Alide Tieleman
  • Fonction : Auteur
Baziel G.M. van Engelen
  • Fonction : Auteur
Laurent Servais
  • Fonction : Auteur
Anthony Béhin
  • Fonction : Auteur
Tanya Stojkovic
  • Fonction : Auteur
Jan Meulstee
  • Fonction : Auteur
Joost A.M. Engel
  • Fonction : Auteur
George Lamas
  • Fonction : Auteur
Wim I.M. Verhagen
  • Fonction : Auteur
Elisabeth Mamelle
  • Fonction : Auteur

Résumé

Objective To systematically assess auditory characteristics of a large cohort of patients with genetically confirmed myotonic dystrophy type 2 (DM2). Methods Patients with DM2 were included prospectively in an international cross-sectional study. A structured interview about hearing symptoms was held. Thereafter, standardized otologic examination, pure tone audiometry (PTA; 0.25, 0.5, 1, 2, 4, and 8 kHz), speech audiometry, tympanometry, acoustic middle ear muscle reflexes, and brainstem auditory evoked potentials (BAEP) were performed. The ISO 7029 standard was used to compare the PTA results with established hearing thresholds of the general population according to sex and age. Results Thirty-one Dutch and 25 French patients with DM2 (61% female) were included with a mean age of 57 years (range 31–78). The median hearing threshold of the DM2 cohort was higher for all measured frequencies, compared to the 50th percentile of normal ( p < 0.001). Hearing impairment was mild in 39%, moderate in 21%, and severe in 2% of patients with DM2. The absence of an air–bone gap with PTA, concordant results of speech audiometry with PTA, and normal findings of BAEP suggest that the sensorineural hearing impairment is located in the cochlea. A significant correlation was found between hearing impairment and age, even when corrected for presbycusis. Conclusions Cochlear sensorineural hearing impairment is a frequent symptom in patients with DM2, suggesting an early presbycusis. Therefore, we recommend informing about hearing impairment and readily performing audiometry when hearing impairment is suspected in order to propose early hearing rehabilitation with hearing aids when indicated.
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Dates et versions

hal-03875067, version 1 (28-11-2022)

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Judith van Vliet, Alide Tieleman, Baziel G.M. van Engelen, Guillaume Bassez, Laurent Servais, et al.. Hearing impairment in patients with myotonic dystrophy type 2. Neurology, 2018, 90 (7), pp.e615-e622. ⟨10.1212/WNL.0000000000004963⟩. ⟨hal-03875067⟩
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