Literature DB >> 27557911

Cochlear Implantation in Patients With CHARGE Syndrome.

Yoon Chan Rah1, Ji Young Lee2, Myung-Whan Suh2, Moo Kyun Park2, Jun Ho Lee2, Sun O Chang3, Seung-Ha Oh4.   

Abstract

OBJECTIVE: To determine the optimal surgical approach for cochlear implantation (CI) preoperatively based on the spatial relation of a displaced facial nerve (FN) and middle ear structures and to analyze clinical outcomes of CHARGE syndrome.
METHODS: Facial nerve displacement and associated deviation of inner ear structures were analyzed in 13 patients (17 ears) with CHARGE syndrome who underwent CI. Surgical accessibility through the facial recess was assessed based on anatomical landmarks. Postoperative speech performance and associated clinical characteristics were analyzed.
RESULTS: The most consistently identified ear anomalies were semicircular canal aplasia (100%), ossicular anomaly (100%), and vestibular hypoplasia (88%). Facial nerve displacement was found in 77% of cases (anteroinferior: 47%, anterior: 24%, inferior: 6%). The width of available surgical space around facial recess was significantly greater in cases of facial recess approach (2.85 ± 0.9 mm) than those of alternative approach (0.12 ± 0.29 mm, P = .02). Postoperatively, 53% achieved better than category 4 on the categories of auditory perception (CAP) scale. The CAP category was significantly correlated with internal auditory canal diameter (P = .025) and did not differ according to the applied surgical approach.
CONCLUSION: Preoperative determination of surgical accessibility through facial recess would be useful for safe surgical approach, and successful hearing rehabilitation was achievable by applying appropriate surgical approaches.
© The Author(s) 2016.

Entities:  

Keywords:  CHARGE syndrome; anomaly; cochlear implantation; facial nerve; facial recess

Mesh:

Year:  2016        PMID: 27557911     DOI: 10.1177/0003489416665190

Source DB:  PubMed          Journal:  Ann Otol Rhinol Laryngol        ISSN: 0003-4894            Impact factor:   1.547


  2 in total

1.  Cochlear implantation in children with CHARGE syndrome: a report of eight cases.

Authors:  Vincenzo Vincenti; Filippo Di Lella; Maurizio Falcioni; Maurizio Negri; Diego Zanetti
Journal:  Eur Arch Otorhinolaryngol       Date:  2018-07-03       Impact factor: 2.503

2.  A Predictive Model for Cochlear Implant Outcome in Children with Cochlear Nerve Deficiency.

Authors:  Jae Joon Han; Myung-Whan Suh; Moo Kyun Park; Ja-Won Koo; Jun Ho Lee; Seung Ha Oh
Journal:  Sci Rep       Date:  2019-02-04       Impact factor: 4.379

  2 in total

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