Hearing impairment in patients with myotonic dystrophy type 2
| Autor: | G. Bassez, Wim I. M. Verhagen, A.A. Tieleman, Judith van Vliet, George Lamas, Joost A.M. Engel, Elisabeth Mamelle, Baziel G.M. van Engelen, Tanya Stojkovic, Anthony Béhin, Bruno Eymard, Laurent Servais, Jan Meulstee |
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| Přispěvatelé: | Département de pathologie [CHU Henri Mondor], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor, Institut de Myologie, Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Association française contre les myopathies (AFM-Téléthon)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS) |
| Jazyk: | angličtina |
| Rok vydání: | 2018 |
| Předmět: |
0301 basic medicine
Adult Male medicine.medical_specialty [SDV]Life Sciences [q-bio] Population Presbycusis 030105 genetics & heredity Audiology Severity of Illness Index Cohort Studies 03 medical and health sciences 0302 clinical medicine All institutes and research themes of the Radboud University Medical Center otorhinolaryngologic diseases Evoked Potentials Auditory Brain Stem Medicine Humans Myotonic Dystrophy education Hearing Loss Aged education.field_of_study Absolute threshold of hearing medicine.diagnostic_test business.industry Hearing Tests Age Factors Tympanometry Middle Aged medicine.disease Disorders of movement Donders Center for Medical Neuroscience [Radboudumc 3] Cross-Sectional Studies Cohort Female Neurology (clinical) Pure tone audiometry Audiometry business 030217 neurology & neurosurgery Cohort study |
| Zdroj: | Neurology Neurology, 2018, 90 (7), pp.e615-e622. ⟨10.1212/WNL.0000000000004963⟩ Neurology, 90, 7, pp. E615-E622 Neurology, 90, E615-E622 |
| ISSN: | 0028-3878 1526-632X |
| DOI: | 10.1212/WNL.0000000000004963⟩ |
| Popis: | ObjectiveTo systematically assess auditory characteristics of a large cohort of patients with genetically confirmed myotonic dystrophy type 2 (DM2).MethodsPatients 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.ResultsThirty-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.ConclusionsCochlear 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. |
| Databáze: | OpenAIRE |
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