Longitudinal auditory data of children with prelingual single-sided deafness managed with early cochlear implantation

Arras, Tine; Boudewyns, An; Swinnen, Freya; Zarowski, Andrzej; Philips, Birgit; Desloovere, Christian; Wouters, Jan; Wieringen, Astrid Van

doi:10.1038/s41598-022-13247-5

Cited by 13 publications

(14 citation statements)

References 60 publications

(62 reference statements)

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“…Individuals with single-sided deafness (SSD) face many challenges that can profoundly affect their quality of life. In particular, the loss of binaural hearing compromises sound localization [22] and the comprehension of speech in noisy environments, leading to communication barriers. Moreover, those with SSD struggle with immediate hear- [11,12,[14][15][16]18].…”

Section: Discussionmentioning

confidence: 99%

Cochlear Implantation in Children Affected by Single-Sided Deafness: A Comprehensive Review

Santopietro,

Fancello,

Fancello

et al. 2024

Audiology Research

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Children with single-sided deafness (SSD) may experience delays in language and speech development. Reduced speech discrimination and poor sound localization abilities in young SSD patients may result in greater cognitive efforts required to focus and process auditory information, as well as increased listening-related fatigue. Consequently, these children can have a higher risk of academic failure and are often in need of extra help at school. Recently, cochlear implants (CIs) have been introduced as a rehabilitative option for these children, but their effectiveness is still a topic of debate. A literature review was performed according to PRISMA guidelines, searching the Medline database from inception to October 2023. The research identified nine papers that met the inclusion criteria. Data extracted from the selected studies included 311 children affected by SSD and cochlear implants. The reported audiological outcomes were further analyzed. Overall, a high level of satisfaction was described by parents of children with SSD and CI, and those who received a CI under the age of 3 presented better results. However, a proportion of patients did not use the device daily. Our review highlights the possible, and still controversial, role of CI for the hearing rehabilitation of children with unilateral deafness, underlining the need for further research in this field. To date, careful and comprehensive counseling with the child and the family is necessary before considering this option.

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Section: Discussionmentioning

confidence: 99%

Cochlear Implantation in Children Affected by Single-Sided Deafness: A Comprehensive Review

Santopietro,

Fancello,

Fancello

et al. 2024

Audiology Research

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“…In addition, the risk of hearing-related fatigue in children with UHL was higher than that in children with normal hearing reported in the literature. 19 Children need adequate rest, visual information to supplement hearing, and other intervention measures to alleviate fatigue, and these children might also show defects in sound source localization, voice discrimination in noise, and binaural processing. 1,5 Therefore, it is necessary for patients with unilateral or bilateral microtia to conduct an early diagnostic hearing assessment and appropriate intervention to minimize the impact of HL on language and cognitive development.…”

Section: Discussionmentioning

confidence: 99%

Correlations Among Clinical Phenotypes, Radiological Examination Indexes, and Hearing Status in Congenital Microtia

Wang,

Jiang,

Pan

et al. 2023

Journal of Craniofacial Surgery

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Microtia is a congenital malformation of the external ear that often presents with other anatomical abnormalities and ipsilateral hearing loss (HL). The aim of this study was to present the correlation among important phenotypic abnormalities in microtia and their relationship with HL in a clinical population in China. In this study, a retrospective analysis was conducted on 307 patients diagnosed with microtia who visited the Department of Auricular Reconstruction of the Plastic Surgery Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, for surgical auricle reconstruction from April 2021 to April 2022. Standardized classification of ear malformations, craniofacial CT scans, and pure tone audiometric data were collected, and statistical analyses were performed using the rank sum test, Kruskal-Wallis test, and Spearman’s rank correlation coefficient. The results showed that group differences between ear malformation and variations in the development of mandible, external auditory canal (EAC), and mastoid pneumatization were statistically significant and each had a positive correlation. Among them, the correlation between development of ear and EAC was the most significant (Ρ=0.72). Besides, the severity of HL (97% were conductive) was positively correlated with ear and EAC dysplasia with or without mandibular hypoplasia. Based on the statistical analysis of the correlation between ear malformation and HL, the authors strongly recommend that facial phenotype reconstruction and hearing improvement of microtia should be considered comprehensively, regardless of whether children with microtia show HL or not, early diagnosis of audiology evaluation and appropriate intervention measures should be implemented.

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“…Where there is a lack of auditory input, this will impede binaural summation of loudness, the head shadow effect, and binaural release from masking which affects their ability to decode speech in noise and localise sound in space (15)(16)(17)(18)(19). In a recent study with children who have no access to sound in one ear (SSD) and who had a cochlear implant (CI), the implanted group exhibited an improved speech perception in noise and better sound localisation skills, compared with their nonimplanted peers (20).…”

Section: Introductionmentioning

confidence: 99%

Characterisation of the treatment provided for children with unilateral hearing loss

Patel,

Hoare,

Willis

et al. 2023

Front. Pediatr.

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BackgroundChildren with permanent unilateral hearing loss (UHL) are an understudied population, with limited data to inform the guidelines on clinical management. There is a funding gap in healthcare provision for the children with UHL in the United Kingdom, where genetic screening, support services, and devices are not consistently provided or fully funded in all areas. They are a disparate population with regard to aetiology and their degree of hearing loss, and hence their device choice and use. Despite having one “good ear”, some children with UHL can have similar outcomes, socially, behaviourally, and academically, to children with bilateral hearing loss, highlighting the importance of understanding this population. In this longitudinal cohort study, we aimed to characterise the management of the children with UHL and the gaps in the support services that are provided for the children in Nottingham, United Kingdom.MethodsA cohort study was conducted collecting longitudinal data over 17 years (2002–2019) for 63 children with permanent congenital confirmed UHL in a large tertiary regional referral centre for hearing loss in Nottingham, United Kingdom. The cases of UHL include permanent congenital, conductive, mixed, or sensorineural hearing loss, and the degree of hearing loss ranges from mild to profound. The data were taken from their diagnostic auditory brainstem responses and their two most recent hearing assessments. Descriptors were recorded of the devices trialled and used and the diagnoses including aetiology of UHL, age of first fit, degree of hearing loss, when and which type of device was used, why a device was not used, the support services provided, concerns raised, and who raised them.ResultsMost children (45/63; 71%) trialled a device, and the remaining 18 children had no device trial on record. Most children (20/45; 44%) trialled a bone-conduction device, followed by contralateral routing of signal aid (15/45; 33%) and conventional hearing aids (9/45; 20%). Most children (36/45; 80%) who had a device indicated that they wore their device “all day” or every day in school. Few children (8/45; 18%) reported that they wore their device rarely, and the reasons for this included bullying (3/8), feedback from the device (2/8), and discomfort from the device (2/8). Only one child reported that the device was not helping with their hearing. The age that the children were first fitted with their hearing device varied a median of 2.5 years for hearing aids and bone-conduction devices and 7 years for a contralateral routing of signal aid. The length of time that the children had the device also varied widely (median of 26 months, range 3–135 months); the children had their bone-conduction hearing aid for the longest period of time (median of 32.5 months). There was a significant trend where more recent device fittings were happening for children at a younger age. Fifty-one children were referred by the paediatric audiologist to a support service, 72.5% (37/51) were subsequently followed up by the referred service with no issue, whilst the remaining 27.5% (14/51) encountered an issue leading to an unsuccessful provision of support. Overall, most children (65%, 41/63) had no reported concerns, and 28.5% (18/63) of the children went on to have a documented concern at some point during their audiological care: five with hearing aid difficulties, five with speech issues, four with no improvement in hearing, three facing self-image or bullying issues, and one case of a child struggling to interact socially with friends. Three of these children had not trialled a device. We documented every concern reported from the parents, clinicians, teachers of the deaf, and from the children themselves. Where concerns were raised, more than half (58.6%, 10/18) were by schools and teachers, the remaining four concerns were raised by the family, and further four concerns were raised by the children themselves.ConclusionTo discover what management will most benefit which children with permanent UHL, we first must characterise their treatment, their concerns, and the support services available for them. Despite the children with UHL being a highly disparate population—in terms of their aetiology, their device use, the degree of hearing loss, and the age at which they trial a device—the majority report they use their device mostly in school. In lieu of available data and in consideration of the devices that are available to them, it could be useful to support families and clinicians in understanding the devices which are most used and where they are used. Considering the reasons for cessation of regular device use counselling and support services would be vital to support the children with UHL.

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Longitudinal auditory data of children with prelingual single-sided deafness managed with early cochlear implantation

Cited by 13 publications

References 60 publications

Cochlear Implantation in Children Affected by Single-Sided Deafness: A Comprehensive Review

Cochlear Implantation in Children Affected by Single-Sided Deafness: A Comprehensive Review

Correlations Among Clinical Phenotypes, Radiological Examination Indexes, and Hearing Status in Congenital Microtia

Characterisation of the treatment provided for children with unilateral hearing loss

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