How can we best support children with limited usable hearing in one ear? This article discusses the latest approaches.
Limited usable hearing unilaterally (LUHU) is an emerging term and has been suggested to replace the historical term ‘single-sided deafness (SSD)’. LUHU is described as a unilateral sensorineural hearing loss, often of severe to profound degree, which is characterised by poor speech recognition abilities resulting in the apparent or predicted lack of benefit from an air conduction (AC) hearing aid [1].
This audiological configuration poses challenging management considerations, particularly for the paediatric population. The status of the auditory nerve on the affected side, developmental considerations in the assessment of speech perception skills, limitations with speech access provided by assistive technologies, and the increased potential for signal crossover to the normal hearing ear are some considerations.
Determining LUHU
The integrity of the cochlear nerve plays a critical role in speech perception abilities. Cochlear nerve deficiency (CND) has been reported to be as high as 63% in children with unilateral severe to profound hearing loss, as identified using magnetic resonance imaging (MRI) [2]. Additionally, CND cannot be predicted from hearing thresholds alone and may be experienced by those with lesser degrees of hearing loss [3]. Ideally, imaging and speech perception assessment would be completed as soon as possible in children with suspected LUHU. Imaging, combined with a functional assessment of speech perception, would support the determination of LUHU which would subsequently aid management recommendations. Unfortunately, infants, young children and those with developmental differences are often not able to participate in speech perception testing. In these instances, clinicians must rely on imaging alone, which may not be available for months to years after hearing loss confirmation.
"Cochlear nerve deficiency (CND) has been reported to be as high as 63% in children with unilateral severe to profound hearing loss, as identified using magnetic resonance imaging (MRI)"
With these limitations in mind, the proportion of infants and young children who have LUHU has been difficult to determine in the literature. Clinically, flexibility is required along the management journey. Counselling and managing family expectations should consider what information is available at the time and should be re-evaluated as diagnostic information becomes available.
LUHU management pathway.
Reproduced from: Brown CL, Bagatto M. Limited usable hearing unilaterally (LUHU) in infants and young children: a scoping review of technology considerations and developmental outcomes © 2025 The Author(s). Originally published in International Journal of Audiology © 2025 BSA; ISA; NAS. Reprinted with permission of Taylor & Francis Ltd. Licensed under CC BY-NC-ND 4.0 (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Aidable hearing and auditory access
Infants and children who have unilateral mild to moderately severe sensorineural hearing loss often have intact cochlear nerves and good speech perception abilities on the affected side. As such, a behind-the-ear (BTE) air conduction hearing aid is a suitable recommendation. BTEs can provide the necessary output to support appropriate speech access to the affected side without concern for crossover to the normal hearing cochlea. Additionally, there is evidence that developmental risk is higher, quality of life is lower, and listening effort is increased for children with unilateral hearing loss [4,5]. As such, best practice indicates that infants and children with UHL be provided with a hearing aid.
"Cochlear implantation is a promising intervention option for eligible LUHU candidates and can support binaural listening development when accessed in the early years"
Due to output limitations of BTEs, infants and young children with severe to profound hearing loss are not suitable candidates for this technology. In these cases, it is likely that the chosen device insufficiently amplifies speech. Additionally, for children with apparent or predicted LUHU, poor aided speech perception and sound quality may be experienced and result in lack of benefit and/or device rejection. Paediatric amplification protocols recommend device use be discontinued if there is lack of benefit [6,7]. In these cases, evidence suggests that alternative management strategies be considered.
Alternative management strategies for children with LUHU
LUHU management options may include cochlear implantation, contralateral routing of signal (CROS) amplification, a bone conduction device (BCD; non-surgical for those under five years of age), remote microphone system (RMS) or no technology. Cochlear implantation (CI) is a promising intervention option for eligible LUHU candidates and is the only treatment option which has the potential to support binaural listening development when accessed in the early years. CI is often contraindicated when CND has been identified, and non-surgical alternatives should be considered [8].
The outcomes for non-surgical options such as a CROS or BCD are not well understood for infants and young children with LUHU. These devices result in a rerouting of the signal from the affected side to the normal hearing ear. Binaural input is not possible and, as a result, binaural listening development is not supported. While sound awareness on the affected side may be improved, these rerouting devices have been shown to be a detriment in noisy situations [9]. This is because these rerouting devices require the child to monitor and adjust their listening environment so the affected side is positioned away from noise. In some instances, the device may need to be removed if competing signals cannot be avoided through positioning alone. These decisions require an understanding of complex listening environments and communication strategies that infants and young children have not yet learned. In contrast, an ear-level RMS, fitted to the normal hearing ear, has demonstrated improved performance when compared to rerouting technologies [9]. The timing for the provision of an ear-level RMS must consider whether the size of the child’s ear canal can support an open fitting so natural sounds are not blocked, while at the same time achieving good device retention.
Considering these variables, there is not a single technology that is best for infants and young children in the early stages of managing LUHU. This is a challenging clinical scenario because early intervention is a goal. In addition to considering which technology may be best for an individual child and family, non-technological strategies should be implemented. Some examples include enriching the listening environment through proximity to the child, limiting background noise, and the use of age-appropriate language to support cognitive development until such time as technology can be revisited.
Conclusion
Management considerations for infants and young children who have LUHU involves many variables. The child’s age and developmental status combined with diagnostic information such as the degree and configuration of the loss and knowledge about the integrity of the cochlear nerve guide management decisions. There are several technology options that can be considered and it is recommended that they be revisited as the child grows. A guideline for paediatric audiologists who work with children who have LUHU offers an evidence-informed approach for the variety of scenarios they will encounter when considering management [10].
References
1. Picou EM, Davis H, Lewis D, Tharpe AM. Contralateral Routing of Signal Systems Can Improve Speech Recognition and Comprehension in Dynamic Classrooms. J Speech Lang Hear Res 2020;63(7):2468–82.
2. Ward KM, Coughran AJ, Lee M, et al. Prevalence of Cochlear Nerve Deficiency and Hearing Device Use in Children With Single‐Sided Deafness. Otolaryngol Head Neck Surg 2023;169(2):390–6.
3. Vos TG, Park LR, Noxon AS, Brown KD. Cochlear Nerve Deficiency in Pediatric Unilateral Hearing Loss and Asymmetric Hearing Loss. Audiol Neurootol 2022;27(4):328–35.
4. McCreery RW, Walker EA, Spratford M, et al. Longitudinal Predictors of Aided Speech Audibility in Infants and Children. Ear Hear 2015;36(Supplement 1):24S–37S.
5. Nicolas S, Gallois Y, Calmels MN, et al. Quality of life of children treated for unilateral hearing loss: A systematic review and meta-analysis. Arch Dis Child 2021;106(11):1102–10.
6. American Academy of Audiology Clinical Practice Guidelines Pediatric Amplification.
https://audiology-web.s3.amazonaws.
com/migrated/PediatricAmplificationGuidelines.pdf
_539975b3e7e9f1.74471798.pdf. 2013.
[Link last accessed November 2025].
7. Bagatto MP, Scollie S, Moodie STF. Protocol for the Provision of Amplification (Version 2023.01). Ontario Infant Hearing Program / National Centre for Audiology. 2023.
8. Park LR, Griffin AM, Sladen DP, et al. American Cochlear Implant Alliance Task Force Guidelines for Clinical Assessment and Management of Cochlear Implantation in Children with Single-Sided Deafness. Ear Hear 2022;43(2):255–67.
9. Griffin AM, Atri A, Licameli G, Stiles DJ. Effect of Hearing Device Use on Speech-in-Noise Performance in Children with Severe-to-Profound Unilateral Hearing Loss. Ear Hear 2023;44(3):588–602.
10. Brown CL, Bagatto M. The development of a clinical practice guideline for the audiological management of infants and young children with permanent unilateral hearing loss using an integrated knowledge translation approach. Int J Audiol 2025;64(11):1155–63.
Declaration of competing interests: MB has received research funding from the Ministry of Children, Community and Social Services.
