Nancy M Young1, Elizabeth Tournis, Jenelle Sandy, Stephen R Hoff, Maura Ryan. 1. *Division of Otolaryngology-Head and Neck Surgery, Ann and Robert H Lurie Children's Hospital of Chicago †Department Otolaryngology-Head and Neck Surgery, Northwestern University Feinberg School of Medicine, Chicago ‡Knowles Hearing Center, Northwestern University School of Communication, Evanston §Department of Audiology ||Department of Medical Imaging, Ann and Robert H Lurie Children's Hospital of Chicago ¶Department of Medical Imaging, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
Abstract
OBJECTIVE: Review perioperative complications, benefits, and the timeframe over which auditory skills develop in children with CHARGE syndrome who receive a cochlear implant (CI). STUDY DESIGN: IRB-approved retrospective chart review of children with CHARGE syndrome who had at least 12 months of cochlear implant use. SETTING: Tertiary care children's hospital. PATIENTS: Twelve children, seven males and five females. Mean age implant = 3.5 years (1.7-8.2 yr); mean duration follow-up = 4.7 years (1.5-10.1 yr). INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: Auditory skills categorized into four levels, temporal bone imaging findings, perioperative complications, time to emergence of speech perception, expressive communication mode. RESULTS: All children imaged with magnetic resonance imaging had cochlear nerve deficiency in at least one ear. Speech awareness threshold improved with the CI compared with aided preoperative in 83% of children, with means of 51.7 dB SAT preoperative and 27.1 dB with the CI (p ≤ 0.002). Overall, four children improved to auditory Level 2 (improved detection), three obtained Level 3 (closed-set speech perception), and three had open-set speech perception with their CIs (Level 4) that was first evident at 3.5, 3.3, and 0.8 years postimplant testing. Two children had minimal or limited improvement. One child with hypoplasia of the cochlear nerve obtained open-set levels. CONCLUSION: Auditory skills may develop slowly in children with CHARGE syndrome who receive a CI but most can achieve at least improved detection. In our series, half acquired some speech perception ability. Cochlear nerve deficiency is frequent, but should not be a contraindication to implantation.
OBJECTIVE: Review perioperative complications, benefits, and the timeframe over which auditory skills develop in children with CHARGE syndrome who receive a cochlear implant (CI). STUDY DESIGN: IRB-approved retrospective chart review of children with CHARGE syndrome who had at least 12 months of cochlear implant use. SETTING: Tertiary care children's hospital. PATIENTS: Twelve children, seven males and five females. Mean age implant = 3.5 years (1.7-8.2 yr); mean duration follow-up = 4.7 years (1.5-10.1 yr). INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: Auditory skills categorized into four levels, temporal bone imaging findings, perioperative complications, time to emergence of speech perception, expressive communication mode. RESULTS: All children imaged with magnetic resonance imaging had cochlear nerve deficiency in at least one ear. Speech awareness threshold improved with the CI compared with aided preoperative in 83% of children, with means of 51.7 dB SAT preoperative and 27.1 dB with the CI (p ≤ 0.002). Overall, four children improved to auditory Level 2 (improved detection), three obtained Level 3 (closed-set speech perception), and three had open-set speech perception with their CIs (Level 4) that was first evident at 3.5, 3.3, and 0.8 years postimplant testing. Two children had minimal or limited improvement. One child with hypoplasia of the cochlear nerve obtained open-set levels. CONCLUSION: Auditory skills may develop slowly in children with CHARGE syndrome who receive a CI but most can achieve at least improved detection. In our series, half acquired some speech perception ability. Cochlear nerve deficiency is frequent, but should not be a contraindication to implantation.
Authors: Jenny X Chen; Anahita Nourmahnad; Jennifer O'Malley; Katherine Reinshagen; Joseph B Nadol; Alicia M Quesnel Journal: Laryngoscope Investig Otolaryngol Date: 2020-01-13
Authors: Carolyn M Jenks; Stephen R Hoff; Jennifer Haney; Elizabeth Tournis; Denise Thomas; Nancy M Young Journal: Otol Neurotol Date: 2022-03-01 Impact factor: 2.311