Cochlear Implantation in Children with Anomalous Cochleovestibular Anatomy

Abstract

Although we found that severe inner ear dysplasia was associated with increased surgical difficulty and lower speech perception, the lack of uniformity in published clinical data limited the strength of these results. Standardization of surgical and radiologic reporting as well as more consistent speech perception testing is needed to better determine the association between anomalous cochleovestibular anatomy and clinical outcomes.

Bilateral Sequential Cochlear Implantation in Patients With Enlarged Vestibular Aqueduct (EVA) Syndrome

“…This study demonstrates excellent audiometric outcomes after cochlear implantation in patients with a diagnosis of EVA in line with audiometric outcomes reported in the literature (8,14). Compared with a control group of pediatric CI patients with normal cochleovestibular anatomy, children with EVA showed comparable postoperative SRT and performance on word and sentence recognition tasks.…”

Facial nerve anomalies in paediatric cochlear implant candidates: radiological evaluation

“…[16][17][18][19][20] Various methods are reported to prevent facial nerve injury during cochlear implantation in patients with inner-ear anomalies. For cochleostomy, Sennaroglu and Saatci used a combined anteroposterior approach through the ear in which the facial nerve crossed over the oval and round windows.…”

Management of Children with Severe, Severe-profound, and Profound Sensorineural Hearing Loss

“…A diagnosis of cochlear nerve deficiency (CND) or significant cochlear or labyrinthine abnormalities may either preclude the use of a CI or predict a low chance of achieving open-set speech perception. 6,8,[19][20][21][22] CND is present in approximately 1% of children with bilateral SNHL, most often in the setting of severe to profound thresholds. 13,23,24 Labyrinthine abnormalities have been detected in up to 40% of children with SNHL 25 and can range in severity.…”

“…2). 26 High-resolution MRI with a 3-dimensional Fourier transformation constructive interference steady-state protocol is the most sensitive for diagnosing CND 19,24 and has the unique ability to assess for concurrent brain disease, 19,27 which may be present in up to 40% of pediatric CI recipients. 28 HRCT is complementary for assessing labyrinthine anomalies (Fig.…”