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Cochlear nerve deficiency is one of the known causes of congenital sensorineural hearing loss. Management of hearing loss in children with cochlear nerve deficiency poses a multidimensional challenge. The absent or hypoplastic cochlear nerve may prevent electrical stimulation from reaching the brainstem and the auditory cortex. A deficient cochlear nerve can be associated with other inner ear malformations, which may diminish the success of cochlear implantation in those children. Promising results in adults after auditory brainstem implantation led to the expansion of candidacy to include the pediatric populations who were contraindicated for CIs. To review the outcomes of cochlear implantation versus that of auditory brainstem implantation in children with various conditions of the auditory nerve. This retrospective chart review study comprised two pediatric groups. The first group consisted of seven ABI recipients with cochlear nerve aplasia and the second group consisted of another seven children with cochlear nerve deficiencies who underwent CI surgery. The participants’ auditory skills and speech outcomes were assessed using different tests selected from the Evaluation of Auditory Responses to Speech (EARS) test battery. There were some individual variations in outcomes depending on the status of the auditory nerve. The mean CAP score of the ABI group was 2.87, while the mean SIR score was 0.62. On the other hand, the mean CAP score of the CI group was 1.29, while the mean SIR score was 0.42. Our results are in good agreement with the reported auditory perception and speech and language development outcomes of pediatric auditory brainstem implantation. We added to the growing body of literature on the importance of verifying and identifying the status of the cochlear nerve in the decision-making process of the surgical management of those pediatric groups.

Identification of the inner ear malformation types from radiographs is a complex process. We hypothesize that each inner ear anatomical type has a uniqueness in its appearance in radiographs. The outer contour of the inner ear was captured from the mid-modiolar section, perpendicular to the oblique-coronal plane, from which the A-value was determined from CT scans with different inner ear anatomical types. The mean A-value of normal anatomy (NA) and enlarged vestibular aqueduct syndrome (EVAS) anatomical types was greater than for Incomplete Partition (IP) type I, II, III and cochlear hypoplasia. The outer contour of the cochlear portion within the mid-modiolar section of NA and EVAS resembles the side view of Aladdin’s lamp; IP type I resembles the side-view of the Sphinx pyramid and type II a Pomeranian dog’s face. The steep spiraling cochlear turns of IP type III resemble an Auger screw tip. Drawing a line parallel to the posterior margin of internal auditory canal (IAC) in axial-view, bisecting the cavity into cochlear and vestibular portions, identifies common-cavity; whereas a cavity that falls under the straight-line leaving no cochlear portion identifies cochlear aplasia. An atlas of the outer contour of seventy-eight inner ears was created for the identification of the inner malformation types precisely.

One of the concerns during the cochlear implant candidacy process is the presence of chronic otitis media which could delay the implantation process. The aim of this study was to evaluate the surgical difficulties and the long-term complications in children with otitis media and to examine whether it is necessary to delay the implantation until the infection is resolved. The study used a comparative retrospective design based on chart review of all patients who received their implant(s) from January to December of 2012. A total of 200 patients were identified and were followed for 4 years post surgery. Patients were divided into three groups based on their history of otitis media (non-otitis media, chronic otitis media with effusion, and acute otitis media). Data included long-term complications, operative time and duration from first clinical visit to the time until implantation was received. None of study participants had long-term complications during the study period. The operative time was longer in the acute otitis media group with a difference of 45 min. The average delay in cochlear implantation due to the presence of otitis media in chronic group was more than 5 months. Pediatric patients with otitis media could be implanted in one stage safely and effectively.