A new cochlear implant electrode with a “cork”-type stopper for inner ear malformations

Objective: To determine the prevalence and features of inner ear anomalies in children with congenital profound hearing loss who presented at our cochlear implant center based on imaging studies. Methods: This retrospective study reviewed charts of children with congenital SNHL, who presented to Department of Otolaryngology & Auditory Implant Centre, Capital Hospital Islamabad over a period of 2 years from 1st May 2017 to 30th April 2019. These included 481 cases of both genders aged between 1 to 12 years. After gathering demographic data, audiological data, computed tomography findings of the temporal bone were analyzed. Data was analyzed using SPSS 22. Results: The Inner Ear Malformations were identified in 48(10%) children including 28 (58.33%) males and 20 (41.67%) female. Most 20(41.67%) presented at >3-5 years of age followed by 19(39.58%) at 2-3 years. However, no significant association of gender (p=0.57, p=0.076) and age of presentation (p=0.344, p=0.697) for right and left ears was noted with inner ear malformations. The most common anomaly noted were CLA, CH-III and CH-II in decreasing order of frequency in both ears. Conclusion: The prevalence of IEM’s was found to be 48(10%). Commonest anomalies noted were CLA, CH-III and CH-II. No significant association of gender and age of presentation was noted with type of anomaly in both ears. doi: https://doi.org/10.12669/pjms.36.7.3134 How to cite this:Ahmed J, Saqulain G, Khan MIJ, Kausar M. Prevalence & features of inner ear malformations among children with congenital sensorineural hearing loss: A Public Cochlear Implant Centre Experience . Pak J Med Sci. 2020;36(7):---------. doi: https://doi.org/10.12669/pjms.36.7.3134 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Analysis of inner ear malformations associated with a facial nerve anomaly in 653 children fitted with a cochlear implant

Clinical Otolaryngology ◽

10.1111/coa.13246 ◽

2018 ◽

Vol 44 (1) ◽

pp. 96-101 ◽

Cited By ~ 4

Author(s):

Aurelie Coudert ◽

Stephanie Vigier ◽

Aurelien Scalabre ◽

Ruben Hermann ◽

Sonia Ayari-Khalfallah ◽

...

Keyword(s):

Facial Nerve ◽

Cochlear Implant ◽

Inner Ear ◽

Inner Ear Malformations ◽

Ear Malformations

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Cochlear Implantation in Children with Inner Ear Malformations

Annals of Otology Rhinology & Laryngology ◽

10.1177/000348949810700607 ◽

1998 ◽

Vol 107 (6) ◽

pp. 492-500 ◽

Cited By ~ 48

Author(s):

Audie L. Woolley ◽

Rodney P. Lusk ◽

Virginia Jenison ◽

Robert S. Bahadori ◽

Barbara S. Stroer ◽

...

Keyword(s):

Speech Perception ◽

Cochlear Implant ◽

Inner Ear ◽

Cochlear Implantation ◽

Csf Leak ◽

Closed Set ◽

Inner Ear Malformations ◽

Open Set ◽

Ear Malformations ◽

Csf Leaks

We performed a case study and intervention study, with follow-up of 1 to 5 years, in 4 children with inner ear malformations who underwent implantation of a multichannel cochlear implant (Nucleus, Cochlear Corporation) at ages 3 to 12 years. Malformations included a common cavity deformity, 2 incomplete partitions, and 1 case of isolated bilateral vestibular aqueduct enlargement. One child had a single-channel implant placed at 3 years of age, and this was exchanged for a 22-channel implant at age 9. One child had her implant placed at age 4.5 years, but due to complications from a cerebrospinal fluid (CSF) leak had the initial implant removed and replaced at age 5 years during repair of the CSF leak. Intraoperative findings included a CSF leak at the time of surgery in 3 patients. One patient contracted bacterial meningitis 7 months postimplantation that was thought to be secondary to acute otitis media in the unoperated ear. Bilateral CSF leaks were noted in the middle ear by a lumbar puncture radionuclide and fluorescein dye study. Successful repair of the CSF leaks and reimplantation of the cochlear implant was carried out in this patient. Mapping and programing of the implant was found to be challenging in each of these patients. All patients demonstrated improved performance after implantation. Two patients demonstrated some open-set speech perception. One patient demonstrates improved use of temporal cues in a structured closed set. One patient has achieved no significant speech recognition at this time, but does have improved sound detection and awareness. Cochlear implantation in children with congenital inner ear abnormalities can be a successful method of rehabilitation. It should be recognized that the postoperative speech perception results may be highly variable among patients, and that intraoperative complications may occur.

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