Vestibular and radiological characteristics of children affected by unilateral auditory neuropathy spectrum disorder.

OBJECTIVE: Auditory neuropathy spectrum disorders (ANSD) are defined by the association of a preserved outer hair cell function and an impaired auditory nerve neural response, and present mostly bilaterally. Unilateral ANSD are consequently only seldom described, and most frequently as isolated cases. This study aims to describe the audiological, vestibular and radiological characteristics of a population of children with unilateral ANSD. MATERIAL AND METHODS: We isolated 22 patients with unilateral ANSD, 12 boys and 10 girls from 0 to 95 months, in a database of auditory evoked potentials. We reviewed the audiological, radiological and vestibular assessments. The audiological assessment included tympanometry, otoacoustic emission recording and auditory evoked potential. Otolithic function was assessed by performing cervical vestibular evoked myogenic potential. The canal function was determined by video head impulse test and/or caloric test. The radiological evaluation consisted of an MRI of the internal auditory canal. RESULTS: Many patients with a type A tympanometry had no response to otoacoustic emission (53,8%), in the presence of a cochlear microphonic potential. Vestibular assessment was performed in 9 of the 22 patients. 4 children had impaired otolithic and/or canal function. MRI evaluation of the inner ear was performed in 18 patients. Aplasia or hypoplasia of the cochlear nerve was found in 17 of them. MRI showed additional vestibular or brainstem abnormalities in 7 of the 18 children. All children with impaired vestibular function had vestibular or brainstem radiological alterations in addition to cochlear branch aplasia or hypoplasia. CONCLUSIONS: Radiological and vestibular abnormalities are common in children with unilateral ANSD and suggest that a radiological and vestibular assessment is required.

Audiological benefit and subjective satisfaction of children with the ADHEAR audio processor and adhesive adapter.

OBJECTIVES: The main objective of this study was to evaluate the audiological benefit of the ADHEAR system in a group of children with a uni- or bilateral conductive hearing loss (CHL) during a short-term exposure of three weeks, and to compare it to a conventional bone conduction hearing aid (BCHA) on a softband. The secondary aim was to assess the quality of life improvement and patient satisfaction with the ADHEAR system. METHODS AND MATERIALS: The study was designed as a prospective study with repeated measures, where each subject served as his/her own control. Ten children (4-17 y/o) with a uni- or bilateral congenital or acquired CHL were included in this study. Pure tone audiometry and speech audiometry in quiet, both unaided and aided, were performed initially with the ADHEAR system and a BCHA on a softband, and after three weeks with the ADHEAR alone. Furthermore, patient satisfaction and quality of life were assessed using the SSQ12 and the ADHEAR questionnaire. RESULTS: The mean unaided free field hearing threshold of 50 dB HL (with 95%CI between 41.7 and 57.5 dB HL) expressed in 'Bureau International d'Audiophonologie' (BIAP), improved significantly by 22 dB (13.0-29.9) with the ADHEAR and by 23 dB (13.6-32.9) with the BCHA (p < 0.001). Furthermore, the mean unaided speech recognition threshold (SRT) in quiet improved significantly by 19 dB (10.3-28.1) with the ADHEAR and by 21 dB (12.6-29.4) with the BCHA (p < 0.001). For both audiological tests, there were no significant differences between the ADHEAR and the BCHA. After three weeks of use, the mean pure tone threshold of 28 dB HL (20.0-36.5) and the mean SRT of 47 dB SPL (41.9-51.5) with the ADHEAR system were comparable and not significantly different than the outcomes during the first visit. Speech understanding in noise and in multiple streams, sound localization and sound quality were rated significantly better with the ADHEAR, compared to the ratings without the ADHEAR system (p < 0.001). None of the children reported skin irritations or pain. CONCLUSIONS: The children included in our study had significantly improved hearing thresholds, speech perception in quiet and quality of life with the ADHEAR. The device can be an effective treatment method and a valuable alternative to other BCHA for children with a CHL, although the subjective experience of each child has to be taken into account. CLINICAL TRIAL REGISTRATION NUMBER: NCT03327194.