TY - JOUR

T1 - Congenital Nonprofound Bilateral Sensorineural Hearing Loss in Children

T2 - Comprehensive Characterization of Auditory Function and Hearing Aid Benefit

AU - Berninger, Erik

AU - Drott, Maria

AU - Romanitan, Mircea

AU - Tranebjærg, Lisbeth

AU - Hellström, Sten

N1 - 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.

PY - 2022

Y1 - 2022

N2 - 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.

AB - 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.

KW - children

KW - congenital

KW - electrophysiology

KW - hearing

KW - hearing aids

KW - imaging

KW - otoacoustic emissions

KW - sensorineural hearing loss

KW - speech recognition

U2 - 10.3390/audiolres12050054

DO - 10.3390/audiolres12050054

M3 - Journal article

C2 - 36285911

AN - SCOPUS:85140391997

VL - 12

SP - 539

EP - 563

JO - Audiology Research

JF - Audiology Research

SN - 2039-4330

IS - 5

ER -