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Published on: February 29, 2020
Long-term Results of ABI in Children With Severe Inner Ear Malformations
Levent Sennaroğlu1, Gonca Sennaroğlu, Esra Yücel
1*Department of Otolaryngology, Faculty of Medicine, Hacettepe University, Ankara, Turkey †Department of Audiology, Faculty of Health Sciences, Hacettepe University, Ankara, Turkey ‡Department of Language and Speech Pathology, Faculty of Health Sciences, Hacettepe University, Ankara, Turkey §Department of Neurosurgery, Faculty of Medicine, Hacettepe University, Ankara, Turkey ||Department of Radiology, Faculty of Medicine, Hacettepe University, Ankara, Turkey ¶Department of Biostatistics, Faculty of Medicine, Hacettepe University, Ankara, Turkey #Department of Audiology, Faculty of Health Sciences, İstanbul University, İstanbul, Turkey **Department of Otolaryngology, Liv Hospital, İstanbul, Turkey ††Department of Neurosurgery, Faculty of Medicine, Marmara University, İstanbul, Turkey.
Insights
Auditory brainstem implants (ABI) offer effective long-term outcomes for children with severe inner ear malformations, enabling understanding of common phrases. Bilateral stimulation with cochlear implants and ABI is recommended for suitable pediatric cases.
Area of Science:
- Otolaryngology
- Pediatric audiology
- Neurosurgery
Background:
- Severe inner ear malformations pose significant challenges to auditory development in children.
- Auditory brainstem implantation (ABI) is an alternative to cochlear implantation (CI) when the auditory nerve is not suitable for CI.
Purpose of the Study:
- To evaluate the long-term audiological and language outcomes in children with severe inner ear malformations who received an auditory brainstem implant (ABI).
Main Methods:
- A retrospective chart review of 60 children who underwent ABI surgery between 2006 and 2014.
- Preoperative assessments included audiological, radiological, and developmental evaluations.
- Outcomes were measured using Categories of Auditory Performance (CAP), speech intelligibility rate (SIR), functional auditory performance of cochlear implant (FAPCI), and language development scales.
Main Results:
- Of 35 children with at least one year follow-up, most achieved CAP 5, indicating understanding of common phrases without lip-reading.
- Speech intelligibility improved with better hearing thresholds.
- While ABI is effective, performance was generally lower than typical CI users, with faster progress in the first two years.
Conclusions:
- Auditory brainstem implantation (ABI) is an acceptable and effective treatment for pediatric patients with severe inner ear malformations.
- Bilateral stimulation, combining cochlear implantation (CI) with contralateral ABI, should be considered for eligible children.
Objective:
To report the long-term outcomes of children who received auditory brainstem implant (ABI) because of severe inner ear malformations.
Study Design:
Retrospective chart review.
Setting:
Tertiary referral otolaryngology clinic.
Subjects And Methods:
Between July 2006 and October 2014, 60 children received ABI at Hacettepe University. Preoperative work up included otolaryngologic examination, audiological assessment, radiological evaluation together with assessment of language development and psychological status. The surgeries were performed via retrosigmoid approach with a pediatric neurosurgeon. Intraoperatively, electrical auditory brainstem response was utilized. Initial stimulation was done 4 to 5 weeks postoperatively. Outcomes were evaluated with Categories of Auditory Performance (CAP), speech intelligibility rate (SIR), functional auditory performance of cochlear implant (FAPCI) and Manchester Spoken Language Development Scale scores; receptive and expressive language ages were determined.
Results:
Sixty children who received ABI were between ages of 12 and 64 months. Thirty-five patients with follow up period of at least 1 year, were reported in means of long-term audiological and language results. The most prevelant inner ear malformation was cochlear hypoplasia (n = 19). No major complication was encountered. Majority of the patients were in CAP 5 category, which implies that they can understand common phrases without lip reading. SIR was found out to be better with improving hearing thresholds. Children with ABI were performing worse than average cochlear implantation (CI) users when FAPCI scores were compared. Patients with the best hearing thresholds have expressive vocabulary of 50 to 200 words when evaluated with Manchester Spoken Language Development Scale. There was no relationship between the number of active electrodes and hearing thresholds. The type of inner ear anomaly with the best and the worst hearing thresholds were common cavity and cochlear aperture aplasia, respectively. Patients with additional handicaps had worse outcomes. Among 35 children, 29 had closed set discrimination and 12 developed open set discrimination above 50%. It was determined that, progress of the patients is faster in the initial 2 years when compared with further use of ABI.
Conclusion:
ABI is an acceptable and effective treatment modality for pediatric population with severe inner ear malformations. Bilateral stimulation together with CI and contralateral ABI should be utilized in suitable cases.

