Abstract
PurposeThe purpose of this retrospective study was to investigate the outcome and critical age of cochlear implantation in congenital single-sided deafness (SSD).Methods11 children with congenital SSD were implanted with a cochlear implant (CI). Auditory performance was measured through the results of speech discrimination, subjective assessment by the Categories of auditory performance (CAP) score, the Speech, Spatial and Qualities scale questionnaire (SSQ) and the German version of the IOI-HA [Internationales Inventar zur Evaluation von Hörgeräten (IIEH, version for CI)].ResultsLong-term follow-up [median: 3 years and 5 months (3;5 years)] revealed that nine children use their CI (> 8 h/day) and two became nonusers. In children aged below 3;2 years at surgery, there was a substantial long-term increase in speech discrimination and subjective benefit. Children over 4;4 years of age at CI surgery improved partially in audiological/subjective measurements. Among children above 5 years, the SSQ score did not improve despite further slight improvement in speech discrimination long-term.ConclusionOur data suggest a critical age for CI surgery below 3 years in children with congenital SSD for successful hearing rehabilitation. It is mandatory to identify children with SSD as early as bilaterally deaf children.
Highlights
Indication for cochlear implantation in single-sided deafness (SSD) has been established for adults with a reduction in tinnitus burden, benefit in localisation, speech discrimination in noise and quality of life [1,2,3]
Most pediatric cochlear implant (CI) candidates (n = 8/11) with congenital SSD were identified through the bilateral newborn hearing screening (NHS); obligatory in Germany since 2009
The deafness period was calculated from their birth to the age where they underwent surgery, since all subjects were diagnosed with congenital SSD
Summary
Indication for cochlear implantation in single-sided deafness (SSD) has been established for adults with a reduction in tinnitus burden, benefit in localisation, speech discrimination in noise and quality of life [1,2,3]. Evidence exists for an “aural preference syndrome” in which the developing auditory pathway in SSD children reorganises with dominance towards the NH ear and weaker central representation of the hearing-impaired ear, involving areas for spatial processing [9, 13,14,15,16]. Representation of the deaf ear does not entirely vanish, but with prolonged period of deafness integration of new auditory input to the deaf ear changes due to central reorganisation and crossmodal plasticity [9, 12]. These changes may be long-lasting and not reversible in development.
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