Long-term Results of ABI in Children With Severe Inner Ear Malformations.

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.

Comparison of various bone cement ossiculoplasty techniques and functional results.

OBJECTIVE: In this study, functional results of different bone cement ossiculoplasty techniques are compared. METHODS: Retrospective case review at a tertiary referral center. Patients who underwent middle ear surgery and bone cement ossiculoplasty between 2006-2012 were included. A total of 52 patients, including 30 patients with 'Incus to stapes' (Group 1) and 13 patients with 'malleus to stapes' (Group 2), five patients with 'incudoplasty + stapedotomy' (Group 3), and four patients with 'malleus to incus' (Group 4) ossiculoplasty were enrolled in the study. Pre-operative and post-operative audiological findings of each group were evaluated. RESULTS: The mean hearing gain (the difference between pre-operative and post-operative air bone gap (ABG)) was 13 dB for Group 1, 30 dB for Group 2, 24 dB for Group 3, and 9 dB for Group 4. The pre-operative air pure tone averages (PTA) of groups 1, 2, and 3 improved significantly in the post-operative period (p < 0.05). Closure of post-operative ABG of patients to less than 20 dB and 10 dB were as follows: ∼70% and 43% in group 1; 86% and 76% in group 2; 100% and 60% in group 3; and 75% and 50% in group 4, respectively. CONCLUSIONS: The results showed that glass ionomer cement is a simple and effective method for reconstruction of ossicular discontinuity in various ossicular chain pathologies and can be an alternative to conventional rebridging techniques such as sculpted incus interposition or partial ossicular replacement prosthesis (PORP).

Traumatic multiple lower cranial nerve palsy: a case report and review of the literature.

Lower cranial nerve palsy, also known as Collet-Sicard syndrome, is a fairly rare pathology characterized by unilateral palsy of the IX-X-XI-XII cranial nerves. We report a multiple cranial nerve palsy developing after a head trauma that might have been considered negligible. A 16-year-old boy was admitted with swallowing and articulation problems and difficulty lifting one shoulder after a head trauma suffered during a football match. No pathology was revealed in the subsequent imaging. Cranial nerve palsies due to head trauma are very rare. Awareness of the possibility of such a condition, leading to early recognition and treatment, may result in significant functional recovery.

Fibrodysplasia ossificans progressiva: a case report.

Fibrodysplasia ossificans progressiva (FOP) is a rare, severely disabling, autosomal dominant disease characterized by recurrent painful episodes of soft tissue swelling and the development of heterotopic ossification. The main target is the axial musculature, but eventually ectopic bone formation occurs in the ligaments, the fascia, the tendons and the joint capsules. Small soft tissue traumas and intramuscular injections exacerbate this extraskeletal bone formation. We present a 16-year-old male patient who has osseous lesions beginning from the left ramus mandible and extending along the sternocleidomastoid muscle, vertebral region and deltoid, with visible restriction in temporomandibuler joint movement. Surgery was not performed due to parental concerns. Unfortunately, no effective medical therapy for FOP is known. These patients may require extra care during some oral surgery and anesthetic procedures. In this report, the importance of the decision to perform surgery has been stressed.