Association between hearing loss and hereditary ATTR amyloidosis. (2nd October 2019)

• Record Type: Journal Article
• Authors: Bartier, Sophie; Bodez, Diane; Kharoubi, Mounira; Guellich, Aziz; Canouï-Poitrine, Florence; Chatelin, Véronique; Coste, André; Damy, Thibaud; Béquignon, Emilie
• Is Part Of: Amyloid. Volume 26:Number 4(2019)
• Page Start: 234
• Page End: 242
• Publication Date: 2019-10-02
• Subjects: cardiac amyloidosis -- hearing loss -- screening -- transthyretin -- hereditary Transthyretin related amyloidosis (ATTRv); Amyloidosis -- Periodicals; 616.3995
• DOI: 10.1080/13506129.2019.1663814
• Languages: English
• ISSNs: 1350-6129

Abstract

Abstract: Background: Hereditary transthyretin (TTR) related amyloidosis (ATTRv) is a life-threatening condition, which can potentially affect all organs. The objective was to identify the hearing status of patients with cardiac ATTRv and describe their audiological pattern. Methods: Nineteen patients with confirmed diagnosis of ATTRv cardiac amyloidosis (CA) underwent otoscopy and audiological tests, including pure tone and speech audiometry. Results : 74% were male, with a mean age of 72 ± 1.8 years. The main mutations were Val122Ile ( n = 7) and Val30Met ( n = 6). Objective hearing loss was detected in 17 patients (89%), whereas only 37% complained of hearing loss. ATTRv patients presented a different audiometric profile compared to patients of the same age with presbycusis: a higher prevalence and worse hearing thresholds compared to age-related expectations (ISO). Hearing loss affected all frequencies with, unexpectedly, mixed or conductive hearing loss (35%). According to the type of mutation, there was an increased rate of sensorineural or mixed/conductive hearing loss. Conclusions: the present study indicates that hearing loss is more prevalent and worse in patients with ATTRv amyloidosis than in the general population, while mostly clinically under-estimated. It suggests that ATTRv deposits could infiltrate the various anatomical structures of the inner and mild ear.