AI Article Synopsis
- The study aimed to evaluate the prevalence of syndromes among children receiving cochlear implants, focusing on inner and middle ear malformations, as well as the surgical challenges faced.
- Out of 1024 children studied, 4.3% were diagnosed with syndromes, with Jervell and Lange Nielsen syndrome and Waardenberg syndrome being the most common, and most cases showing no inner ear malformations.
- Despite some inner and middle ear anomalies noted during surgery, the overall outcomes for syndromic patients were comparable to those without syndromes, emphasizing the need for individualized assessments in managing hearing loss.
Article Abstract
Objective: Identify the prevalence of syndromes in a cohort of patients who underwent cochlear implantation, to report on the presence of inner and middle ear malformations and highlight the surgical difficulties encountered.
Study Design: Observational, retrospective study.
Setting: Tertiary referral children's hospital pediatric cochlear implant program.
Material & Methods: An IRB-approved retrospective chart review of children undergoing cochlear implantation at a tertiary academic medical center, from 2018 to 2023. Preoperative imaging data of syndromic patients in that cohort with special attention to the presence of inner ear or middle ear malformations were collected. Abnormal intraoperative findings and difficulties reported by the surgeons were also noted.
Results: 1024 children were unilaterally implanted for bilateral profound hearing loss. There were 45 cases diagnosed with associated syndromes (4.3%). The commonest syndromes were Jervell and Lange Nielsen (JLN) syndrome followed by Waardenberg syndrome (WS), in a prevalence of 34% and 32% respectively. These syndromes had no associated inner ear malformations (IEM). Less common syndromes included Branchio-oto-renal (BOR) syndrome, CHARGE association and Treacher Collins syndrome, 3 cases each, and keratosis icthyosis deafness syndrome (KID), Usher syndrome and Albino, 2 cases each and an H syndrome case. There were 9 cases (20%) with IEM, with 6 cases of perilymph gusher. Two cases had middle ear anomalies and one case had a facial nerve course abnormality. The outcome of these cases was similar to non-syndromic cases.
Conclusion: Children with syndromic HL should be dealt with on a case by case scenario to diagnose inner and middle ear malformations. Additional disabilities can affect the rehabilitation procedures. All children with congenital hearing loss should undergo pediatric, cardiologic, ophthalmologic and nephrologic examination in order to exclude the syndromic etiology of hearing loss.
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|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11735558 | PMC |
| http://dx.doi.org/10.1007/s00405-024-08897-2 | DOI Listing |
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