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Cochlear implantation in children with internal ear malformations
N Loundon1, I Rouillon, N Munier
1ENT Department, Armand Trousseau Children's Hospital, Paris, France. natalie.loundon@trs.ap-hop-paris.fr
Insights
Cochlear implantation in children with inner ear malformations showed good surgical outcomes and variable hearing and speech results. Progressive deafness generally leads to better outcomes than congenital deafness.
Area of Science:
- Otolaryngology
- Pediatric Audiology
- Neurosurgery
Background:
- Inner ear malformations present unique challenges for cochlear implantation.
- Understanding surgical aspects and outcomes is crucial for managing hearing loss in these children.
Purpose of the Study:
- To evaluate the surgical techniques and clinical outcomes of cochlear implantation in pediatric patients with inner ear malformations.
- To compare results between children with progressive versus congenital deafness.
Main Methods:
- Retrospective cohort study of 18 children with inner ear malformations undergoing cochlear implantation.
- Surgical complications, word recognition (CSW, OSW), and speech production were assessed.
- Patients were categorized into progressive (G1) and congenital (G2) deafness groups.
Main Results:
- No major surgical complications like facial injury or infection were observed; 50% experienced intraoperative gusher.
- Significant improvements in word recognition (CSW) were noted post-implantation (83% vs 16%).
- Good oral language development was achieved by 76% at 2 years, with better outcomes in the progressive deafness group (G1).
Conclusions:
- Cochlear implantation is surgically safe in children with inner ear malformations.
- Perceptive and linguistic outcomes are variable, influenced by the type and onset of deafness.
- Progressive deafness is associated with more favorable outcomes compared to congenital deafness.
Objective:
To evaluate surgical aspects and results of cochlear implantation in inner ear malformations.
Study Design:
Retrospective cohort study.
Setting:
Ear, nose, and throat department of a tertiary referral hospital.
Patients:
Out of 260 implanted children, 18 (6.9%) had inner ear malformations: complex cochleovestibular malformation (n = 11), common cavity (n = 1), and enlarged vestibular aqueduct (EVA) (n = 6). Deafness was progressive in 12 cases (G1) and congenital in 6 cases (G2). Genetics lead to diagnosis in 12 of 13 cases: PSD mutation (n = 11), Waardenburg syndrome (n = 1), negative (1). Mean age at implant was 7.8 years. Mean follow-up period was 48 months.
Main Outcome Measures:
Medical and surgical outcomes were reported. Closed (CSW) and open (OSW) set word perception and level of speech production were evaluated each year. The results were compared pre- and postoperatively and between the two groups.
Results:
Gusher at surgery was observed in 50% of cases, with a persistent leak in one case. No facial injury or infectious complications were observed. At 12 months, 83% of the population had achieved more than 75% recognition in CSW, versus 16% before implant (p = 0.001). After 2 years, 64% of patients had more than 50% recognition in OSW. Good oral language was seen in 76% at 2 years and 100% at 3 years, versus 55% before implant (respectively, p > 0.05 and p = 0.03). At 1 year after implant, 83% of the G1 and 20% of the G2 achieved more than 50% recognition in OSW (p = 0.02). After 24 months, 83% of G1 and 40% of G2 had more than 50% in OSW (p > 0.05). Before implant, 75% in G1 and 0% in G2 had good oral language (p = 0.01). At 1 year, 83% in G1 and 16% in G2 had good oral language (p = 0.02). At 2 years, 100% in G1 and 20% in G2 had good oral language (p = 0.02). One child in G1 had no improvement after implantation.
Conclusions:
No major complication was seen. Perceptive and linguistic results were variable and depended on the type of the deafness. In progressive deafness, the perceptive and linguistic result are expected to be good. In congenital deafness, the results are more variable.