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Children with congenital unilateral hearing loss

This presentation focuses on children with congenital unilateral hearing loss. Drawing on current evidence, Dr. Teresa Ching addresses three questions:
  1. What is the impact of unilateral hearing loss?
  2. Does hearing change?
  3. Does a hearing device help?

Dr Teresa YC Ching is Professorial Fellow at NextSense Institute (formerly the research arm of the Royal Institute for Deaf and Blind Children) in Australia, and Conjoint Professor in Special Education and Disability Studies at Macquarie University. She is also an Honorary Professor at The University of Queensland. She has led population-based research on speech, language, psychosocial and quality of life outcomes of children who are deaf or hard of hearing in one ear or both ears, including those with or without additional disabilities. The research findings have generated evidence-based guidelines for clinical management to improve outcomes of children with hearing loss.

Regardless of whether you consider a hearing device for the child who is affected with unilateral hearing loss, the two pieces of advice that are most important to help the child grow.

  1. Provide a rich language environment 
    Provide a rich language environment for your child, regardless of whether they were wearing a device or not. There is emerging evidence to suggest that a rich language exposure is critical for normal language development – not only for children with hearing loss but also for typically-developing children. It is particularly important for children who have some hearing impairment to have that rich language exposure. This isn’t turning the TV on and watching videos on iPads. It is that interactive exchange of language that helps the child to make sense of the environment and to learn speech and language.

  2. Position the speaker to the good ear of the child.
    Remember to position the speaker to the good ear of the child. Even if the child is wearing a device in the ear that is affected, the normal functioning ear is still the ear that is going to provide the best possible input.

Q: What audiology test/s is/are recommended for assessing hearing ability in noisy situations e.g. a classroom.

Dr Ching: Speech perception in noise tests. One method to assess hearing ability in noisy situations was shown in the presentation when performance was compared between two listening conditions: in one condition, speech and noise were presented from the same location in front; and in a second condition, speech was presented from the frontal loudspeaker and noise from loudspeakers on both sides. Children who achieved better performance when the location of speech was separated from that of the noise than when speech and noise were collocated demonstrated an ability to use binaural cues and would have less difficulties listening in noisy situations. Griffin et al (2023) Doi: 10.1097/AUD.0000000000001310 reported on the performance of school-aged children with unilateral hearing loss listening to speech in noise when they were using different hearing devices.

Q: Are there studies showing impact of sign language intervention for children with UHL? With/without listening devices

Dr Ching: This is a good question! As spoken language is accessible via the normally functioning ear in a child with unilateral hearing loss and to some extent via the affected ear (depending on the severity and whether hearing devices are used), the desired mode of communication is generally spoken language. If that is the case, emphasize spoken language. Enrich their language environment, read aloud, sing songs and chant nursery rhymes. Develop the auditory brain by speaking, reading and singing to support learning. Always orientate the child’s normally functioning ear (good ear) to the dominant sound source. Reinforce head turning to the sound source.

I am not aware of high-quality studies that investigated the effectiveness of sign language intervention for children with unilateral hearing loss. For deaf and hard of hearing children, a systematic review in 2016 (Fitzpatrick et al (2016) Pediatrics. 2016;137(1):e20151974) concluded that there was insufficient high-quality evidence that sign language in combination with oral language therapy is more effective than oral language therapy alone. For children who required cochlear implantation, Geers et al (2017) (Pediatrics 140(1): e20163489; doi: 10.1542/peds.2016-3489) showed that there was no advantage to parents’ use of sign language either before or after cochlear implantation.

Q: Have any studies looked at remote mic on poorer ear vs. better ear? And unilateral remote mic vs. bilateral?

Dr Ching: Schafer et al (2020) provides a comprehensive description of remote microphone technology for children with hearing loss or auditory processing issues (Seminars in Hearing 2020, 41(4):277-290. Doi: 10.1055/s-0040-1718713).

Q: With regard to loss by frequency – how do low frequencies fare as I heard these are more difficult to diagnose in an ABR and variance can be greater (+/- 20db)?

Dr Ching: Electrophysiological measurements such as ABR records brain responses to electrical stimulation. These are used for diagnosis to provide an estimate of hearing. The gold standard for assessing hearing thresholds is behavioural audiometry, typically from 250 Hz to 8000 Hz. Frequencies from 500 to 4000 Hz are important for understanding speech.

Q: I’m confused, was told it’s best to talk to the aided ear not the good ear. Only talk to good ear when unaided. Thank you.

Dr Ching: The good ear functions normally. So, it sends the best quality input to the brain. As the affected ear may not hear sounds well, aiding enables sounds to be audible in that ear, albeit at a lower quality compared to sounds in the good ear. Aiding also allows the potential use of binaural hearing cues for localisation and for listening to speech in noise.

  1. Anne, S., Lieu, J. E. C., & Cohen, M. S. (2017). Speech and language consequences of unilateral hearing loss: A systematic review. Otolaryngology–Head and Neck Surgery, 157, 572-579. DOI: 10.1177/0194599817726326
  2. Appachi, S., Specht, J. L., Raol, N., Lieu, J. E. C., Cohen, M. S., Dedhia, K., & Anne, S. (2017). Auditory outcomes with hearing rehabilitation in children with unilateral hearing loss: A systematic review. Journal of Otolaryngology – Head & Neck Surgery, 157, 565-571. DOI: 10.1177/0194599817726757
  3. Arras, T., Boudewyns, A., Dhooge, I., et al. (2021). Assessment of Receptive and Expressive Language Skills Among Young Children With Prelingual Single-Sided Deafness Managed With Early Cochlear Implantation. JAMA Netw Open, 4(8), e2122591. DOI: 10.1001/jamanetworkopen.2021.22591
  4. Bussé, A. M. L., Hoeve, H. L. J., Nasserinejad, K., Mackey, A. R., Simonsz, H. J., & Goedegebure, A. (2020). Prevalence of permanent neonatal hearing impairment: systematic review and Bayesian meta-analysis. International Journal of Audiology, 59(6), 475-485. DOI: 10.1080/14992027.2020.1716087
  5. Cañete, O. M., Purdy, S. C., Brown, C. R. S., Neeff, M., & Thorne, P. R. (2021). Behavioural performance and self-report measures in children with unilateral hearing loss due to congenital aural atresia. Auris Nasus Larynx, 48(1), 65-74. DOI: 10.1016/j.anl.2020.07.008
  6. Fitzpatrick, E. M., Gaboury, I., Durieux-Smith, A., Coyle, D., Whittingham, J., & Nassrallah, F. (2019, Feb). Auditory and language outcomes in children with unilateral hearing loss. Hear Res, 372, 42-51. DOI: 10.1016/j.heares.2018.03.015
  7. Griffin, A. M., Atri, A., Licameli, G., & Stiles, D. J. (2023). Effect of Hearing Device Use on Speech-in-Noise Performance in Children with Severe-to-Profound Unilateral Hearing Loss. Ear and Hearing, 44(3), 588-602. DOI: 10.1097/AUD.0000000000001310.
  8. Griffin, A. M., Poissant, S. F., & Freyman, R. L. (2020). Auditory Comprehension in School-Aged Children With Normal Hearing and With Unilateral Hearing Loss. Language, Speech, and Hearing Services in Schools, 51(1), 29-41. DOI: 10.1044/2019_LSHSS-OCHL-19-0020
  9. Golub, J. S., Lin, F. R., Lustig, L. R., & Lalwani, A. K. (2018). Prevalence of adult unilateral hearing loss and hearing aid use in the United States. Laryngoscope, 128(7), 1681-1686. DOI: 10.1002/lary.27017
  10. Huttunen, K., Erixon, E., Lofkvist, U., & Maki-Torkko, E. (2019, Feb 19). The impact of permanent early-onset unilateral hearing impairment in children – A systematic review. International Journal of Pediatric Otorhinolaryngology, 120, 173-183. DOI: 10.1016/j.ijporl.2019.02.029
  11. McSweeny, C., Cushing, S. L., Campos, J. L., Papsin, B. C., & Gordon, K. A. (2021). Functional Consequences of Poor Binaural Hearing in Development: Evidence From Children With Unilateral Hearing Loss and Children Receiving Bilateral Cochlear Implants. Trends Hear, 25, 23312165211051215. DOI: 10.1177/23312165211051215
  12. Nassrallah, F., Fitzpatrick, E. M., Whittingham, J., Sun, H., Na, E., & Grandpierre, V. (2018). A descriptive study of language and literacy skills of early school-aged children with unilateral and mild to moderate bilateral hearing loss. Deafness & Education International, 1-19DOI: 10.1080/14643154.2018.1555119
  13. Purcell, P. L., Shinn, J. R., Davis, G. E., & Sie, K. C. (2016, Mar). Children with unilateral hearing loss may have lower intelligence quotient scores: A meta-analysis. Laryngoscope, 126(3), 746-754. DOI: 10.1002/lary.25524
  14. Ross, D. S., Visser, S. N., Holstrum, W. J., Qin, T., & Kenneson, A. (2010). Highly variable population-based prevalence rates of unilateral hearing loss after the application of common case definitions. Ear & Hearing, 31(1), 126-133. DOI: 10.1097/AUD.0b013e3181bb69db

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