Hearing aid fitting for visual and hearing impaired patients with Usher syndrome type IIa

Abstract

ObjectivesUsher syndrome is the leading cause of hereditary deaf‐blindness. Most patients with Usher syndrome type IIa start using hearing aids from a young age. A serious complaint refers to interference between sound localisation abilities and adaptive sound processing (compression), as present in today's hearing aids. The aim of this study was to investigate the effect of advanced signal processing on binaural hearing, including sound localisation.Design and participantsIn this prospective study, patients were fitted with hearing aids with a nonlinear (compression) and linear amplification programs. Data logging was used to objectively evaluate the use of either program. Performance was evaluated with a speech‐in‐noise test, a sound localisation test and two questionnaires focussing on self‐reported benefit.ResultsData logging confirmed that the reported use of hearing aids was high. The linear program was used significantly more often (average use: 77%) than the nonlinear program (average use: 17%). The results for speech intelligibility in noise and sound localisation did not show a significant difference between type of amplification. However, the self‐reported outcomes showed higher scores on ‘ease of communication’ and overall benefit, and significant lower scores on disability for the new hearing aids when compared to their previous hearing aids with compression amplification.ConclusionsPatients with Usher syndrome type IIa prefer a linear amplification over nonlinear amplification when fitted with novel hearing aids. Apart from a significantly higher logged use, no difference in speech in noise and sound localisation was observed between linear and nonlinear amplification with the currently used tests. Further research is needed to evaluate the reasons behind the preference for the linear settings.

Sound localization with bilateral bone conduction devices

“…[20,21] Furthermore, in our experiences with bilaterally fitting conventional hearing aids, patients preferred linear amplification with a minimum set of sound processing features activated. [28] During the study, only minor gain corrections were applied upon request, while maintaining linear For each patient the gender, age in years at study participation, type of hearing rehabilitation prior to bone conduction device (BCD) implantation (hearing aid (HA)), age at hearing rehabilitation for right (AD) and left (AS) side, pre-or post-lingual hearing impairment, age at implantation of first and second BCD, duration of bilateral BCD use in years upon study participation, etiology (chronic otitis media (COM), congenital microtia/atresia/middle ear anomaly), atresia was classified according to Cremers et al [44], middle ear anomaly was classified according to Teunissen and Cremers [45], type of sound processor (5 p = 5 power), localization behaviour with bilateral BCDs at baseline, pure-tone average (PTA) at 0.5-, 1-, 2-, and 3 kHz for air conduction (AC), bone conduction (BC), and aided thresholds for right and left side are shown. n/a = not assessed a P2 underwent autologous ear reconstruction for microtia on the right side at the age of 11 (performed in another institution).…”

Usher Syndrome in the Inner Ear: Etiologies and Advances in Gene Therapy

“…The current treatment for USH1- and USH3-induced hearing loss is CI [ 23 , 75 ]. Hearing loss caused by USH2 is typically less severe, and the current intervention is hearing aids [ 76 ]. The existing interventions for Usher-related hearing loss are invasive and expensive, and gene therapy presents an opportunity to greatly improve outcomes through restoration of an individual’s endogenous hearing, ending the reliance on devices that do not faithfully recapitulate normal hearing [ 77 , 78 , 79 ].…”

Recent Advancements in the Regeneration of Auditory Hair Cells and Hearing Restoration

“…The most promising findings have been centered on the treatment of the rare genetic disease, Usher’s syndrome. Usher syndrome is the world’s most prominent cause of deaf-blindness (Friedman et al, 2011 ; Bonnet and El-Amraoui, 2012 ; El-Amraoui and Petit, 2014 ; Mathur and Yang, 2015 ; Hartel et al, 2017 ). The blindness that results from the disease is a form of retinitis pigmentosa, while the deafness is variable depending on the involved genes (Sun et al, 2016 ).…”