1 INTRODUCTION
Cochlear implantation (CI) has become a life-transforming device for
children with severe and profound hearing loss (HL). However, in the
beginning, CI was a contraindication for inner ear malformations (IEMs),
which are, constitute approximately 20%–35% congenital sensorineural
hearing loss cases.1-4 Preoperative audiologic and
radiologic evaluations are very important for cochlear implant
candidates. With advanced imaging techniques such as CT and MRI,
cochlear structures could be appropriately evaluated. With these
advanced techniques, cochleovestibular malformations were classified,
and it was reported that the majority of children with IEMs anomalies
have bilateral severe to profound HL and are candidates for either CI or
Auditory Brainstem Implant (ABI).5-7 It was known that
CI is an effective treatment modality for individuals with IEMs.
Sennaroglu and Bajin5 were classified IEMs eight
groups: Complete Labyrinthine Aplasia (Michel Deformity), Rudimentary
Otocyst, Cochlear Aplasia, Common Cavity, Cochlear Hypoplasia,
Incomplete Partition of the Cochlea I-II-III, Enlarged Vestibular
Aqueduct, Cochlear Aperture Abnormalities. The incidence of IEMs was
reported at 11.5% in our clinic.3
Cochlear implant outcomes depend on different parameters such as onset
and duration of HL, the age at implantation, presence of
neurodevelopmental disorders, the anatomy of the inner ear and
physiology of the auditory nerve, family support, socioeconomic level,
access to rehabilitation services, and regular CI programming. Important
information about cochleovestibular anomalies has allowed in the
preoperative period with advanced technology in imaging
methods.6,7 Because of comorbidity of IEMs with
different types, especially children, could be suffering from auditory
perception. Many studies have compared the speech perception and
language skills of children with and without IEMs. A study by Farhood et
al,8 showed the benefits of CI in the speech
perception of patients with IEMs. Eisenman et al,9reported that the progression was slower in children with IEMs for
auditory perception.
In the current study, the principal objective was to compare the
preoperative and postoperative auditory performance of children with and
without IEMs. The specific objective of this study was to elaborate
auditory perception outcomes of IEMs types.