RESUMO
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.
RESUMO
BACKGROUND: This study aimed to evaluate the role of landmarks for proper round window electrode insertion in cochlear implantation surgery. METHODS: This is a case series study. We included 150 patients undergoing cochlear implantation in a tertiary medical center during the period from January to December 2019. Patients with inner ear malformations or ossification or revision surgery were excluded. Three surgeons partici- pated in the study. During surgery, the round window electrode insertion was marked using 5 surgical landmarks: oval window, pyramid, fustis, round window membrane, and arborization of intracochlear blood vessels. Each surgeon reported on the identification of each landmark and its reliability for round window electrode insertion. RESULTS: Oval window and round window membrane were clearly seen by the 3 surgeons in all cases. Pyramid was seen in 94% of cases, fustis in 85%, and intracochlear wall in 90% of cases. The postoperative transorbital x-ray confirmed the intracochlear position of electrodes in all cases. CONCLUSION: Round window electrode insertion can be precisely performed using these 5 surgical landmarks in straight forwards cases as well as in difficult cases. These landmarks can also assist in teaching young surgeons, in a step-wise manner, how to properly do round window electrode insertion in cochlear implantation surgery.