Audiological and Speech Performance After Cochlear Implantation in Cochlear Aplasia Deformity

Abstract

Inner ear malformation is a congenital anomaly associated with prelingual sensory neural hearing loss in the pediatric population. This is a case report of bilateral radiologically diagnosed cochlear aplasia in a child who underwent unilateral left cochlear implantation with audiological results at one-year follow-up after surgery. Sixteen months after the cochlear implantation surgery, the child could produce 200 words and say a sentence with two words. In certain cases of inner ear malformation, the subgrouping of cystic cavity can be difficult and should not delay the trial of cochlear implant provided an acceptable anatomical appearance of the inner ear with cochleovestibular nerve existence and a proper electrode used for implantation.

Long‐term audiologic outcomes and potential outcome predictors of cochlear implantation in cochlear aplasia with dilated vestibule: A case series

“…In the literature, the audiologic outcomes of CI in patients with CA with either normal or dilated vestibules were shown by CAP5 and CAP4 at 4 and 3 years after implantation, respectively, without longer-term follow-up. 3 Another CADV child who underwent unilateral CI at the age of 2.7 years showed age-appropriate language development at around 4 years of age, 5 suggesting that CADV patients are not necessarily poor CI candidates, contrary to Sennaroglu et al…”

“…Bong Jik Kim 1 Hyoungwon Jeon 2 Yehree Kim 2 Sang-Yeon Lee 3 Jin Hee Han 1,2 Nayoung Yi 1 Jong Woo Chung 4 Seung-Ha Oh 3,5 Byung Yoon Choi…”

“…In more detail, CA has been largely viewed as a contraindication to CI. However, satisfactory short‐term outcomes have also been anecdotally reported in CA at the case report level 3,5 . These contradictory data in the literature have made it more difficult to predict CI outcomes in CADV patients.…”

“…However, satisfactory short-term outcomes have also been anecdotally reported in CA at the case report level. 3,5 These contradictory data in the literature have made it more difficult to predict CI outcomes in CADV patients. Moreover, even in these case reports describing successful outcomes, the follow-up period was insufficient; thus, there is no information on whether children with CADV can show maximum auditory capability, defined as categories of auditory performance (CAP) score of 7 (CAP7).Since long-term audiologic results have yet to be reported, the prognostic factors of this disease entity have not been well investigated.…”

How to Maximize the Outcomes of Cochlear Implantation in Common Cavity and Cochlear Aplasia With Dilated Vestibule, the Most Severe Inner Ear Anomalies?

“…Nonetheless, CI has been generally considered a valid option for CC despite inconsistent outcomes [ 3 ]. Satisfactory short-term outcomes of CI for cochlear aplasia have also been anecdotally reported at the case report level [ 4 , 5 ], although CADV has been traditionally viewed as a contraindication to CI. We are thus faced with the question: what accounts for these contradictory results of CI for CC/CADV?…”