Diagnosis and management of conductive hearing loss in children with trisomy 21

Stewart, Robbie; Gallagher, Donna; Leyden, Peter

doi:10.1111/jpc.14065

Cited by 5 publications

(8 citation statements)

References 10 publications

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“…Furthermore, it is important to provide information on how they can limit the functional deficits of listening with one ear. Finally, it is essential to monitor the development of the child, especially the speech and language development, and start therapy in time, because children with one ear are at risk (52,53).…”

Section: Is Age At Intervention Important For Consistent Use Of Bcd In Uchl?mentioning

confidence: 99%

“…BCDs do not allow for processing of interaural time differences (33, 34), which is an important strategy for sound localization and hearing in background noise. Professionals might consider to advise parents of children with UCHL to wait with a more invasive hearing treatment until the children can make the decision regarding treatment themselves (52). Information for children, parents and teachers is important to further improve the care for these specific group of patients.…”

Section: What To Advise Parents Of Children With Uaa?mentioning

confidence: 99%

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Toward Optimal Care for Children With Congenital Unilateral Aural Atresia

2021

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Section: Is Age At Intervention Important For Consistent Use Of Bcd In Uchl?mentioning

confidence: 99%

Section: What To Advise Parents Of Children With Uaa?mentioning

confidence: 99%

Toward Optimal Care for Children With Congenital Unilateral Aural Atresia

2021

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“…These minimal findings suggest that the classification of CHL may be influenced by several patient characteristics relating to the cause, severity, and longevity of CHL. Given the number of aetiologies resulting in chronic middle ear dysfunction and resulting CHL [21,22], development of sub-classifications for CHL may be beneficial within UNHS. An absence of these subclassifications places children likely to develop chronic CHL at risk of further developmental delays due to delayed or inappropriate interventions.…”

Section: Are There Any Sub-classifications Of Chl That Guide Specific Interventions Within Unhs?mentioning

confidence: 99%

“…Similarly, craniofacial anomalies such as cleft palate and cleft lip are often linked to middle ear anomalies that typically result in CHL [18]. There is also a clear association between a number of syndromes present at birth and the occurrence of CHL throughout childhood [21][22].…”

Section: Introductionmentioning

confidence: 99%

Conductive Hearing Loss Within Universal Newborn Hearing Screening Programs: A Systematic Review

Collins¹,

Beswick²,

Driscoll³

et al. 2019

J Hear Sci

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Background: Universal Newborn Hearing Screening (UNHS) attempts to identify children with a permanent, bilateral, moderate or greater hearing loss at birth. However, children who are referred from UNHS programs may have conductive hearing loss (CHL), sensorineural, or mixed hearing loss. The aim of this review was to investigate the prevalence, sub-classifications, audiological diagnosis, and medical management of CHL within UNHS programs. Material and methods:A systematic literature search was completed in the scientific databases PubMed, CINAHL, and Embase. Studies were reviewed with reference to the inclusion criteria, then graded to assess the internal and external validity, leaving 25 studies for review. Results:The prevalence of conductive hearing loss ranged from 0.4% to 64.5%. 'Genetic' and 'Permanent' were the only two sub-classifications of CHL identified, with no uniform terminology evident. Given CHL is not a target condition of UNHS, audiological assessment was consistent with the diagnosis of Permanent Childhood Hearing Loss (PCHL). There was little evidence of audiological review, onward referrals, or medical management for CHL within UNHS programs. Of the evidence obtained, no alternative pathway was found for children identified with CHL through UNHS. Conclusions:In view of the limited evidence for CHL within UNHS, further investigation into the prevalence, sub-classification, and appropriate management of CHL within a UNHS program is recommended to better guide evidence-based assessment and management of these children.

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“…It can also reflect their listening environment, for example, if their device helps them in background noise or whether they are predominately in a quiet home and are able to position themselves for optimal listening. Counselling by a multidisciplinary team is advocated to avoid treatment bias for children with conductive UHL ( 14 ), and this can benefit children with either sensorineural UHL or aural atresia. However, it is critical to monitor speech and language development since they are at risk when listening with one ear and device trial should be implemented early ( 15 ).…”

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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Diagnosis and management of conductive hearing loss in children with trisomy 21

Cited by 5 publications

References 10 publications

Toward Optimal Care for Children With Congenital Unilateral Aural Atresia

Toward Optimal Care for Children With Congenital Unilateral Aural Atresia

Conductive Hearing Loss Within Universal Newborn Hearing Screening Programs: A Systematic Review

Characterisation of the treatment provided for children with unilateral hearing loss

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