Abstract
The use and utility of cochlear implantation has rapidly increased in recent years as technological advances in the field have expanded both the efficacy and eligible patient population for implantation. This review aims to serve as a general overview of the most common hearing disorders that have favorable auditory outcomes with cochlear implants (CI). Hearing loss in children caused by congenital cytomegalovirus infection, syndromic conditions including Pendred Syndrome, and non-syndromic genetic conditions such as hearing impairment associated with GJB2 mutations have shown to be successfully managed by CI. Furthermore, cochlear implantation provides the auditory rehabilitation for the most common etiology of hearing loss in adults and age-related hearing loss (ARHL) or presbycusis. However, in some cases, cochlear implantation have been associated with some challenges. Regarding implantation in children, studies have shown that sometimes parents seem to have unrealistic expectations regarding the ability of CI to provide auditory rehabilitation and speech improvement. Given the evidence revealing the beneficial effects of early intervention via CI in individuals with hearing disorders especially hearing loss due to genetic etiology, early auditory and genetic screening efforts may yield better clinical outcomes. There is a need to better understand genotype-phenotype correlations and CI outcome, so that effective genetic counseling and successful treatment strategies can be developed at the appropriate time for hearing impaired individuals.
Highlights
Hearing loss is a common neurosensory disorder affecting humans
cochlear implant (CI) seems to be highly effective in the majority of cases of hearing loss
Some patients with hearing loss due to mutations in specific, genes especially those expressed in spiral ganglion neurons (SGNs), do not benefit adequately from CI
Summary
Hearing loss is a common neurosensory disorder affecting humans. Studies have long documented the significant prevalence, financial burden, and societal ramifications of hearing loss (Mathers et al, 2002; Dalton et al, 2003; Nelson et al, 2005; Agrawal et al, 2008; Vos et al, 2017; Graydon et al, 2019; Ronner et al, 2020). There are several other genetic causes of congenital hearing loss, and the most common are outlined in Table 1 along with their CI outcomes Many of these mutations follow a relatively consistent pattern regarding good CI performance outcomes. Associated with improved results in Usher syndrome but age is most prognostic factor in CI performance with early CI achieving satisfactory auditory and speech outcomes (Xiong et al, 2019) Case study: CI significantly improved speech perception tests [monosyllable: 77%; word: 84%, sentence: 100% (Usami et al, 2020)]. – Cause of post-lingual deafness – Cochlear implantation may prove to be a viable treatment option for the hearing loss characteristic of Alport syndrome (Bittencourt et al, 2012) – Children under 2 years of old had an average score of 33 of 35 on the littlEARS test. – Open-Set Word Ability via the Phonetically Based Kindergarten Test: 6 of the 7 were assessed and scores of 84, 80, 80, 60, 52, and 40% (patient had a wound seroma) were reported
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