Congenital Nonprofound Bilateral Sensorineural Hearing Loss in Children: Comprehensive Characterization of Auditory Function and Hearing Aid Benefit

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A prospective cross-sectional design was used to characterize congenital bilateral sensorineural hearing loss (SNHL). The underlying material of >30,000 consecutively screened newborns comprised 11 subjects with nonprofound, alleged nonsyndromic, SNHL. Comprehensive audiological testing was performed at ≈11 years of age. Results showed symmetrical sigmoid-like median pure-tone thresholds (PTTs) reaching 50–60 dB HL. The congenital SNHL revealed recruitment, increased upward spread of masking, distortion product otoacoustic emission (DPOAE) dependent on PTT (≤60 dB HL), reduced auditory brainstem response (ABR) amplitude, and normal magnetic resonance imaging. Unaided recognition of speech in spatially separate competing speech (SCS) deteriorated with increasing uncomfortable loudness level (UCL), plausibly linked to reduced afferent signals. Most subjects demonstrated hearing aid (HA) benefit in a demanding laboratory listening situation. Questionnaires revealed HA benefit in real-world listening situations. This functional characterization should be important for the outline of clinical guidelines. The distinct relationship between DPOAE and PTT, up to the theoretical limit of cochlear amplification, and the low ABR amplitude remain to be elucidated. The significant relation between UCL and SCS has implications for HA-fitting. The fitting of HAs based on causes, mechanisms, and functional characterization of the SNHL may be an individualized intervention approach and deserves future research.

Original languageEnglish
JournalAudiology Research
Volume12
Issue number5
Pages (from-to)539-563
ISSN2039-4330
DOIs
Publication statusPublished - 2022

Bibliographical note

Funding Information:
This project was supported by the regional agreement on medical training and clinical research (ALF) between Stockholm County Council and Karolinska Institutet, the Tysta Skolan Foundation, Stingerfonden, Foundation of the Swedish Order of Freemasons (Konung Gustaf VI Adolfs frimurarefond, 2022), Karolinska University Hospital, and Karolinska Institutet.

    Research areas

  • children, congenital, electrophysiology, hearing, hearing aids, imaging, otoacoustic emissions, sensorineural hearing loss, speech recognition

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