Outcome of cochlear implantation in children with enlarged vestibular aqueduct (EVA) and mondini dysplasia (incomplete partition type II)

Abstract

Background Congenital inner ear abnormality is a major cause of sensorineural hearing loss in children, about 20% of children with congenital sensorineural hearing loss (SNHL) have associated malformations of the temporal bone, and increased experience in cochlear implantation has led to more children with abnormal cochleo-vestibular anatomy submitted to this procedure. Aim To evaluate the outcome of cochlear implantation in cases with enlarged vestibular aqueduct (EVA) and Mondini deformity (incomplete partition type II) following cochlear implantation and comparing the results with cochlear implant cases without inner ear anomalies. Patients and methods 69 children all less than 6 years of age, CI surgeries underwent from April 2014 to December 2015 at the National Hearing & Speech institute (HSI) Giza-Egypt. Eight patients were congenital prelingual severe to profound hearing loss with enlarged vestibular aqueduct (EVA) and Mondini deformity (incomplete partition type II) Isolated EVA was detected in four cases, two cases with bilateral incomplete partition type II (Mondini deformity) and two cases with bilateral incomplete partition II (Mondini deformity) associated with dilated vestibule & vestibular aqueducts bilaterally with short lateral, SCC (semicircular canal), Beside those 8 cases, a random sample (16 control cases) . The ECAP thresholds determined with neural response telemetry (NRT) or Auditory response threshold (ART) software provide a good starting point for locating the behavioral T and C levels. Auditory skills were evaluated before start of rehabilitation and after 6 months of rehabilitation using LittlEARS Questionnaire and Auditory Checklists and compared to the auditory skills of other cochlear implantees with normal inner ear. Results: This study showed that the auditory skills and audiological performance of children with congenital ear anomalies developed over a period of 6 months after cochlear implantation, in a similar manner to those of young children with radiologically normal inner ears. Conclusion Cochlear implantation can be successfully performed in children with enlarged vestibular aqueduct (EVA) and Mondini dysplasia (incomplete partition type II) .These children and their parents can expect significant auditory benefits from this intervention.