作者
Judith van Vliet, Alide A Tieleman, Baziel GM van Engelen, Guillaume Bassez, Laurent Servais, Anthony Béhin, Tanya Stojkovic, Jan Meulstee, Joost AM Engel, George Lamas, Bruno Eymard, Wim IM Verhagen, Elisabeth Mamelle
发表日期
2018/2/13
期刊
Neurology
卷号
90
期号
7
页码范围
e615-e622
出版商
Lippincott Williams & Wilkins
简介
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 …
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J van Vliet, AA Tieleman, BGM van Engelen, G Bassez… - Neurology, 2018