Dr.

Supreet Singh Nayyar, AFMC

2012

Anomalies In Relation To Cochlear Implant

(for more topics & ppts in ENT, visit www.nayyarENT.com) I. Malformations limited to the membranous labyrinth A. Complete membranous labyrinthine dysplasia (Siebenmann-Bing) B. Limited membranous labyrinthine dysplasia 1. Cochleosaccular dysplasia (Scheibe) 2. Cochlear basal turn dysplasia (Alexander) II. Malformations of the osseous and membranous labyrinth A. Complete labyrinthine aplasia (Michel) B. Cochlear anomalies 1. Cochlear aplasia 2. Cochlear hypoplasia 3. Incomplete partition (Mondini) 4. Common cavity C. Labyrinthine anomalies 1. Semicircular canal dysplasia 2. Semicircular canal aplasia
Children with inner ear malformations can safely receive implants Adequate pre op imaging (HRCT/MRI) However, special attention should be paid to the occurrence of intraoperative o Facial nerve anomalies o CSF leaks through the cochleostomy o Difficulty in inserting electrodes Intra operative findings can be o FN partially or completely over RW/OW o FN inf to OW & post to RW o Dehiscent FN o Bifid FN o Perilymph gushers o Absent LSCC/ Absent SCCs o Flattened promontory o Absent RW & promontory o Persistant stapedial artery Total SCC aplasia and CC (common cavity) present particularly high risks for facial nerve anomaly, and surgical techniques should be modified appropriately In RW absence, electrodes put anteroinferior to RW expected location In terms of audiologic performance, children with inner ear malformations may perform very well Predictors of good performance include o Cochlear dysplasia o Enlarged Vestibular Aqueduct o Dilated vestibule
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Dr. Supreet Singh Nayyar, AFMC

2012

o Mondinis malformation o SCC aplasia or hypoplasia In contrast, poor performance is not as easily predicted by inner ear malformation CHARGE syndrome is commonly associated with inner ear anomalies o CHARGE (coloboma, congenital heart disease, atresia of choana, retardation, genital hypoplasia, ear anomalies) o Because of associated psychomotor retardation/developmental delay poor outcome Michel & IAC narrowing are contraindications for cochlear implantation

(for more topics & ppts in ENT, visit www.nayyarENT.com)

www.nayyarENT.com