Cochlear Implantation in Children with Anomalous Cochleovestibular Anatomy: Our Experience

Area of Science:

• Otolaryngology
• Neurosurgery
• Audiology

Background:

• Congenital cochlear malformations present unique challenges for hearing rehabilitation.
• Cochlear implantation is a potential treatment for hearing loss associated with these malformations.

Purpose of the Study:

• To evaluate the surgical outcomes and postimplantation performance of cochlear implantation in patients with various cochlear malformations.
• To identify factors influencing outcomes in this patient group.

Main Methods:

• Seventeen patients with cochlear malformations (enlarged vestibular aqueduct, Mondini's dysplasia, common cavity, incomplete partition type 2) underwent cochlear implantation.
• Operative findings, including facial nerve anatomy and cerebrospinal fluid (CSF) leaks, were documented.
• Speech perception performance was assessed using standard tests postimplantation.

Main Results:

• CSF leaks occurred in thirteen patients and were successfully repaired.
• No abnormal facial nerve anatomy or surgical complications were observed.
• All patients, except two with common cavity deformity, achieved complete electrode insertion.
• Improved speech perception was demonstrated by all patients postimplantation.
• Patients with enlarged vestibular aqueduct showed better outcomes compared to other malformations.

Conclusions:

• Cochlear implantation is a viable and effective rehabilitation strategy for congenital deafness in the presence of cochlear malformations.
• Enlarged vestibular aqueduct malformations appear to be associated with more favorable outcomes following cochlear implantation.