Hearing Preservation during Cochlear Implantation and Electroacoustic Stimulation in Patients with SLC26A4 Mutations

Kyung Jin Roh, Sera Park, Jin Se Jung, In Seok Moon, Sung Huhn Kim, Mi Young Bang, Jae Young Choi

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Background and Objectives: Patients with SCL26A4 mutations presenting with Mondini deformity and enlarged vestibular aqueduct (EVA) tend to have comparable residual hearing. Although cochlear implantation (CI) produces good results in this group, deterioration of residual hearing can be an adverse event after surgery due to accompanying cochlear malformation and perilymph leakage during cochleostomy. The purpose of this study was to investigate if CI in patients with SCL26A4 mutations via the round window (RW) approach could achieve preservation of residual hearing, and to evaluate their speech reception with electroacoustic stimulation (EAS). Subjects and Methods: This is a retrospective chart review of eight patients with bilateral EVA, who were bi-allelic patients with SCL26A4 mutations. CI was performed in all patients by a single surgeon using the RW approach. Audiological results were compared before and after implantation. Results: Additional hearing loss after CI was less than 10 dBHL in five out of eight patients. Average hearing deterioration after CI was 8.75 dB (range, 0-26). Six out of eight patients used EAS mode after CI. The acoustic stimulation frequency ranged from 271 to 438 Hz. Patients showed better speech recognition in quiet and in noise using EAS mode compared with electrical stimulation alone. Conclusions: Preservation of residual hearing could be achieved after CI in patients with the SLC26A4 mutation via the RW approach. For successful preservation of residual hearing, application of newly-developed soft electrode and meticulous surgical is necessary. Our study showed that patients with the SLC26A4 mutation can be good candidates for EAS surgery.

Original languageEnglish
Pages (from-to)1262-1267
Number of pages6
JournalOtology and Neurotology
Volume38
Issue number9
DOIs
Publication statusPublished - 2017 Oct 1

Fingerprint

Cochlear Implantation
Hearing
Mutation
Perilymph
Acoustic Stimulation
Cochlea
Hearing Loss
Electric Stimulation
Noise
Electrodes

All Science Journal Classification (ASJC) codes

  • Otorhinolaryngology
  • Sensory Systems
  • Clinical Neurology

Cite this

Roh, Kyung Jin ; Park, Sera ; Jung, Jin Se ; Moon, In Seok ; Kim, Sung Huhn ; Bang, Mi Young ; Choi, Jae Young. / Hearing Preservation during Cochlear Implantation and Electroacoustic Stimulation in Patients with SLC26A4 Mutations. In: Otology and Neurotology. 2017 ; Vol. 38, No. 9. pp. 1262-1267.
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Hearing Preservation during Cochlear Implantation and Electroacoustic Stimulation in Patients with SLC26A4 Mutations. / Roh, Kyung Jin; Park, Sera; Jung, Jin Se; Moon, In Seok; Kim, Sung Huhn; Bang, Mi Young; Choi, Jae Young.

In: Otology and Neurotology, Vol. 38, No. 9, 01.10.2017, p. 1262-1267.

Research output: Contribution to journalArticle

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T1 - Hearing Preservation during Cochlear Implantation and Electroacoustic Stimulation in Patients with SLC26A4 Mutations

AU - Roh, Kyung Jin

AU - Park, Sera

AU - Jung, Jin Se

AU - Moon, In Seok

AU - Kim, Sung Huhn

AU - Bang, Mi Young

AU - Choi, Jae Young

PY - 2017/10/1

Y1 - 2017/10/1

N2 - Background and Objectives: Patients with SCL26A4 mutations presenting with Mondini deformity and enlarged vestibular aqueduct (EVA) tend to have comparable residual hearing. Although cochlear implantation (CI) produces good results in this group, deterioration of residual hearing can be an adverse event after surgery due to accompanying cochlear malformation and perilymph leakage during cochleostomy. The purpose of this study was to investigate if CI in patients with SCL26A4 mutations via the round window (RW) approach could achieve preservation of residual hearing, and to evaluate their speech reception with electroacoustic stimulation (EAS). Subjects and Methods: This is a retrospective chart review of eight patients with bilateral EVA, who were bi-allelic patients with SCL26A4 mutations. CI was performed in all patients by a single surgeon using the RW approach. Audiological results were compared before and after implantation. Results: Additional hearing loss after CI was less than 10 dBHL in five out of eight patients. Average hearing deterioration after CI was 8.75 dB (range, 0-26). Six out of eight patients used EAS mode after CI. The acoustic stimulation frequency ranged from 271 to 438 Hz. Patients showed better speech recognition in quiet and in noise using EAS mode compared with electrical stimulation alone. Conclusions: Preservation of residual hearing could be achieved after CI in patients with the SLC26A4 mutation via the RW approach. For successful preservation of residual hearing, application of newly-developed soft electrode and meticulous surgical is necessary. Our study showed that patients with the SLC26A4 mutation can be good candidates for EAS surgery.

AB - Background and Objectives: Patients with SCL26A4 mutations presenting with Mondini deformity and enlarged vestibular aqueduct (EVA) tend to have comparable residual hearing. Although cochlear implantation (CI) produces good results in this group, deterioration of residual hearing can be an adverse event after surgery due to accompanying cochlear malformation and perilymph leakage during cochleostomy. The purpose of this study was to investigate if CI in patients with SCL26A4 mutations via the round window (RW) approach could achieve preservation of residual hearing, and to evaluate their speech reception with electroacoustic stimulation (EAS). Subjects and Methods: This is a retrospective chart review of eight patients with bilateral EVA, who were bi-allelic patients with SCL26A4 mutations. CI was performed in all patients by a single surgeon using the RW approach. Audiological results were compared before and after implantation. Results: Additional hearing loss after CI was less than 10 dBHL in five out of eight patients. Average hearing deterioration after CI was 8.75 dB (range, 0-26). Six out of eight patients used EAS mode after CI. The acoustic stimulation frequency ranged from 271 to 438 Hz. Patients showed better speech recognition in quiet and in noise using EAS mode compared with electrical stimulation alone. Conclusions: Preservation of residual hearing could be achieved after CI in patients with the SLC26A4 mutation via the RW approach. For successful preservation of residual hearing, application of newly-developed soft electrode and meticulous surgical is necessary. Our study showed that patients with the SLC26A4 mutation can be good candidates for EAS surgery.

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