Two Down Syndrome Patients with Bilateral Profound Hearing Loss: Case Report and Literature Review

Yu Zheng,Meng Zhao,Fang-Lu Chi,Juan-Mei Yang,Xiao-Qing Qian

doi:10.3390/ohbm1020008

Abstract

Hearing loss is not uncommon among patients with Down syndrome (DS). It has been reported in 38–78% of the Down syndrome population. However, profound hearing loss in DS patients is rarely noticed due to its low incidence. In this article, we reported two Down syndrome patients with bilateral profound hearing loss in two cases. The first case involved an eight-year-old DS child experiencing extremely severe defects in terms of language and severe defects in terms of gross motor function, adaptability, and sociability. The second case revolved around another DS child with bilateral cochlear nerve absence. We review literature on the DS patients with hearing loss and conclude that profound sensorineural hearing loss in those patients has not received enough attention so far. We also recommend that cochlear implantation (CI) suitability assessment and timely intervention via cochlear implantation are necessary in DS patients. Besides, benefits from CI would be limited and hearing rehabilitation process could be much slower when compared with children without additional inabilities.

Highlights

Thanks to evidences stockpiled in in past 30 years, it is crystal clear that the incidence of hearing loss in Down syndrome (DS) patients is much higher past 30 years, it is crystal clear that the incidence of hearing loss in DS patients is much higher than than general population, estimated at 38–78% in comparison with 2.5% [2]
CTThe and/or management of profound patients has not drawn enough attention so far, and there is of magnetic resonance imaging (MRI) to identify inner ear anomalies in DS patients and uncovered a much higher incidence little reported evidence in respect to the outcomes of management of severe or profound inner ear anomalies in this population, which may partly explain profound SNHL in DS patients
A child with the longest history of implant use impairment and intellectual disability in DS patients should not be considered as contraindications had the best outcomes in all four children reported by Hans

Summary

Case 1

An eight-year-old boy, who failed in hearing screening test and was diagnosed with Down syndrome post-natally, attended our clinic over his inability to respond to sound and to talk. Auditory brainstem response (ABR) and multiple-frequency auditory steady-state evoked responses (ASSR) were performed when he was 29-month-old at the second affiliated hospital of. The ABR results showed the bilateral hearing thresholds were greater than. The ASSR results showed the bilateral hearing thresholds were greater than 110 dB. Balance Med. 2018, 1, 8 gross motor function, he could stand independently for only dozens of seconds and he could walk only when others held one of his hands; he was unable to identify objects in terms of cognitive level;. Could walk only when others held one of his hands; he was unable to identify objects in terms of cognitive level; Table he could only pronounce the “ah”

Schedules

Case 2

Findings

Discussion

Conclusions