Approximately 2% of congenital profound deafness cases are due to cochlear nerve (CN) deficiency. MRI is essential to confirm if the nerve is deficient, but because of limitations with resolution, especially when the internal auditory canal is narrowed, it is often unable to distinguish between hypoplasia and aplasia. A full audiometric test battery should always be performed, even if the MRI suggests CN aplasia, as there will sometimes be evidence of audition. Electrically evoked auditory brainstem response testing can be carried out transtympanically via the round window or using an intracochlear test electrode to help determine the status of the CN. If any test suggests the presence of a CN, then cochlear implantation (CI) should be considered. Children should be followed up closely with audiometric, electrophysiological and language assessments to determine the benefits. They may initially show benefit but fail to progress. CI results are variable and often result in poor outcomes with Categories of Auditory Perception scores of <5. Auditory brainstem implantation (ABI) can be considered when CI is contraindicated or fails to provide adequate benefit. This may provide better outcomes, but this form of surgery has greater risks and future device replacement (in case of device failure) may be complicated. Careful patient selection is required when considering ABI as significant learning difficulties make programming extremely challenging. Patients should be given the option of CI first and then ABI. A small minority of patients presenting late (around 2-3 years of age) may be candidates for simultaneous CI and ABI.

Management of Cochlear Nerve Hypoplasia and Aplasia.

Objectives To evaluate long-term language development in children with prelingual deafness who received auditory brainstem implants (ABIs) compared with children who received cochlear implants (CIs) at the same hospital Additional non-auditory disabilities were taken into account Study design Retrospective cohort study Setting Tertiary referral center Patients Ten children with bilateral malformations of the cochlea and/or cochlear nerve who received ABIs, including seven with additional disabilities, and 147 children with CIs as a reference group, including 22 children with additional disabilities Intervention ABIs were implanted at 13 to 62 years of age Follow-up ranged from 11 to 77 years Main outcome measures Receptive and expressive language abilities were assessed using the Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS), the Categories of Auditory Performance (CAP), the Meaningful Use of Speech Scale (MUSS), and the Speech Intelligibility Rate (SIR) Results Of the 10 children with ABIs, seven had long-term follow-up data Within 1 year, six of the seven children with ABIs could identify sounds, respond to speech, and use their voice to attract attention Language skills developed at a slower rate than in children with CIs and reached the same competence level when additional disabilities were absent These language skills matched, on average, those of children with CIs with additional disabilities Conclusion For deaf children with bilateral inner ear malformations, ABIs provide satisfactory auditory input Children with ABIs are able to develop receptive and expressive language skills comparable to those of children with CIs with additional disabilities Using this knowledge, preoperative parent counselling can be refined

Pediatric Auditory Brainstem Implant Users Compared With Cochlear Implant Users With Additional Disabilities.

OBJECTIVE To determine audiological outcomes of children who use a cochlear implant (CI) in one ear and an auditory brainstem implant (ABI) in the contralateral ear. DESIGN Retrospective case review. SETTING Tertiary referral hospital. PARTICIPANTS Twelve children followed with CI and contralateral auditory brainstem implant (ABI) by Hacettepe University Department of Otorhinolaryngology and Audiology in Turkey. All children were diagnosed with different inner ear malformations with cochlear nerve aplasia/hypoplasia. CI was planned in the ear with better sound detection during behavioural testing with inserted ear phones and with better CN as seen on MRI. Due to the limited auditory and speech progress with the cochlear implant, ABI was performed on the contralateral ear in all subjects. MAIN OUTCOME MEASURES Audiological performance and auditory perception skills of children with cochlear nerve deficiency (CND) who use bimodal electrical stimulation with CI and contralateral ABI. RESULTS Mean age of the subjects was 84.00 ± 33.94 months. Age at CI surgery and ABI surgery was 25.00 ± 10.98 months and 41.50 ± 16.14 months, respectively. However, hearing thresholds only with CI and only with ABI did not reveal significant difference, and auditory perception scores improved with bimodal stimulation. The MAIS scores were significantly improved from unilateral CI to bimodal stimulation (P = .002). Pattern perception and word recognition scores were significantly higher with the bimodal condition when compared to CI only and ABI only conditions. CONCLUSION Children with CND showed better performance with CI and contralateral ABI combined. Depending on the audiological and radiological results, bimodal stimulation should be advised for children with CND.

Bimodal stimulation in children with inner ear malformation: One side cochlear implant and contralateral auditory brainstem implant.

Auditory brainstem implantation in children with hearing loss: Effect on speech production.

Importance Common cavity deformity is a rare congenital inner ear malformation associated with profound hearing loss and attributed to an early developmental arrest of the cochlear-vestibular structures. This narrative review highlights the need to identify reliable indicators of hearing rehabilitation outcome. Observations Despite its relatively simple definition, common cavity deformity varies widely in morphologic features, presence of cochlear nerve fibers and remnants of Corti organ, and outcome after cochlear or brainstem implant. Conclusions and Relevance Cochlear implant has been shown to be a valid option for common cavity deformity, but its outcome remains variable and poor. Identification of specific neuroradiologic, audiologic, and neurophysiologic prognostic features; tailoring of the surgical approach; and standardization of outcome measures are needed to optimize the management of common cavity deformity and hearing rehabilitation after implant.

Epidemiologic, Imaging, Audiologic, Clinical, Surgical, and Prognostic Issues in Common Cavity Deformity: A Narrative Review

‘Second Consensus Meeting on Management of Complex Inner Ear Malformations: Long Term Results of ABI in Children and Decision Making Between CI and ABI’ took place on 5–6 April 2013 in Kyrenia, Nor...

Consensus statement: Long-term results of ABI in children with complex inner ear malformations and decision making between CI and ABI

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Consensus statement: Long-term results of ABI in children with complex inner ear malformations and decision making between CI and ABI