OBJECTIVE:The aim of this study is to describe the rehabilitative outcomes of pediatric auditory brainstem implant (ABI) users in the Department of Otolaryngology in the Hacettepe University. It was a retrospective study, and all patients' files were reviewed. MATERIALS and METHODS:The data was collected from 41 children who were fitted with ABI between 2005 and 2013. Inclusion criteria for children in our study are profound, congenital bilateral sensory-neural hearing loss with anomalies (such as cochlear, labyrinthine, and cochlear nerve aplasia) and more than one year of auditory experience with ABI. Post-meningitis patients and neurofibromatosis type 2 (NF2) patients were excluded. Auditory perception was evaluated using the Meaningful Auditory Integration Scale (MAIS), Functioning after Pediatric Cochlear Implantation (FAPCI) instrument, Categories of Auditory Performance (CAP), and Children's Auditory Perception Skills Test in Turkish (CIAT). Speech intelligibility was categorized with speech intelligibility rating (SIR), and language development was assessed using the Test of Early Language Development-Third Edition (TELD-3) and Manchester Spoken Language Development Scale (MSLD). RESULTS:All patients gained basic audiological functions and were able to recognize and discriminate sounds by the third month of ABI surgery. According to the duration of ABI use and learning skills, patients revealed development from word identification to sentence recognition level in a wide spectrum. CONCLUSION:Preliminary results indicate that all children have gained basic auditory perception skills. On the other hand, language and speech development data were varying among children. Additional handicaps seemed to slow down progression. Secondary improvement was seen at psychosocial areas with respect to behavioral and social adjustment as well as eagerness to start communication.

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Objective: To study the effect of age at auditory brainstem implant (ABI) surgery on auditory perception, language, and speech intelligibility. Study Design: Retrospective single cohort design. Setting: Tertiary referral center. Patients: In this study, 30 pediatric ABI users with no significant developmental issues were included. Participants were divided into two groups, according to age at surgery (Early Group: < 3 yr old [n ¼ 15], Late Group: ! 3 yr old [n ¼ 15]). Groups were matched by duration of ABI use and participants were evaluated after 5 years (AE1 yr) experience with their device. The mean age at ABI surgery was 22.27 (ranged AE 6.5) months in the early group, 45.53 (ranged AE 7.9) months in the late group. Intervention(s): Retrosigmoid craniotomy and ABI placement. Main Outcome Measure(s): Auditory perception skills were evaluated using the Meaningful Auditory Integration Scale and Categories of Auditory Performance from the Children's Auditory Perception Test Battery. We used a closed-set pattern perception subtest, a closed-set word identification subtest, and an open-set sentence recognition subtest. Language performance was assessed with the Test of Early Language Development and Speech Intelligibility Rating, which was administered in a quiet room. Results: In this study, the results demonstrated that the Early Group's auditory perception performance was better than the Late Group after 5 years of ABI use, when children had no additional needs (U ¼ 12, p < 0.001). Speech intelligibility was the most challenging skill to develop, in both groups. Due to multiple regression analysis, we found that auditory perception categories can be estimated with speech intelligibility scores, pattern perception scores, receptive language scores, and age at ABI surgery variables in ABI users with no additional handicaps. Conclusions: ABI is a viable option to provide auditory sensations for children with cochlear anomalies. ABI surgery under age 3 is associated with improved auditory perception and language development compared with older users.

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.

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Cochlear implants (CIs) became life-transforming devices for children with severe hearing loss. Initially, CIs were contraindicated for children with inner ear malformations (IEMs), who constitute approximately 20%-35% of the congenital sensorineural hearing loss cases. 1-3 Cochlear structures can be evaluated by computed tomography (CT) and magnetic resonance imaging (MRI). Most children with IEMs have bilateral severe to profound hearing loss and are candidates for a CI or an auditory brainstem implant (ABI). 4-6 The IEMs' classification system includes eight groups 4 : Complete Labyrinthine Aplasia (Michel Deformity), Rudimentary Otocyst, Cochlear Aplasia (CA), Common Cavity (CC), Cochlear Hypoplasia (CH), Incomplete Partition of the Cochlea I-II-III (IP-I, IP-II, IP-III), Enlarged Vestibular Aqueduct (EVA), and Cochlear Aperture Abnormalities (CAA). The IEMs' incidence at our clinic is 11.5%, 2 but at present, they comprise 21% of children undergoing CI surgery.

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Osteofibrous dysplasia is a rare and benign disease that originates from the tibia or fibula. The symptoms of osteofibrous dysplasia include painless enlargement and bowing of the tibia and pain occurring in the presence of pathological fracture. Herein a male infant who was admitted with redness and swelling on the right leg and diagnosed as pathological tibia fracture due to left tibia osteofibrous dysplasia on the third day of life was presented. To our knowledge, this is the earliest presentation of osteofibrous dysplasia with a pathological fracture in a neonate. Therefore, it must be suspected in neonatal bone fractures.