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Objective To study gain and cutaneous tolerance of the Sophono Alpha1 implant, used for unilateral hearing rehabilitation in children with ear atresia, and to demonstrate non-inferiority compared to the referral closed skin device, BAHA® on a test-band. Methods and materials Fifteen children included in a prospective clinical trial from September 2010 to November 2012. Tertiary care center. Main outcomes: Pure tone and speech audiometry before surgery and at M6 and 12; Speech-in-noise tests with and without Alpha1 at M6. Quality of life questionnaire completed before surgery and at M6; children and parent's satisfaction assessed at M6. Cutaneous appearance assessed at M6, M12 and during the last clinical assessment. Results Patients’ ages ranged from 61 to 129 months. They had a pure conductive deafness with a mean air conduction pure-tone average (ACPTA) of 69.02 ± 9.31 dB, and a mean SRT of 71.73 ± 9.20 dB. Follow-up was 12–32 months. At M6, the mean aided ACPTA was 33.49 ± 4.89 dB, the mean aided SRT 38.27 ± 4.54 dB and the mean aided SRT in noise was statistically improved (−7.80 dB). Sophono Alpha1 demonstrated non-inferiority compared to BAHA® on a test-band. At M12, mean aided ACPTA was 2.94 dB higher ( p < 0.001) but the mean SRT variation (+0.73 dB) was not significantly different ( p = 0.35). At M12, all children used the implant 5 to 12 h daily (mean: 10) without cutaneous complications. Both children and parents reported being satisfied or very satisfied. The score for 7/10 questions in silence or noisy environment was statistically improved when wearing the device. Conclusions This prospective study demonstrate non inferiority, good cutaneous tolerance, satisfaction of children and parents and improvement of the quality of life with the Sophono Alpha1 compared to BAHA® on a test-band. In the light of this study and of the previously published series, the aided threshold is between that obtained with BAHA on a softband and percutaneous BAHA. The Sophono Alpha1 device needs to be part of the hearing solutions proposed for the hearing rehabilitation in children with ear atresia. [ABSTRACT FROM AUTHOR]

Objectives To analyze vestibular function Q2 (VF) after cochlear implantation (CI) in children. Methods Retrospective cohort study at a tertiary pediatric referral center. 43 children that had vestibular testing (VT) after unilateral cochlear implantation, from 2001 to 2010, were included. CT scan of the temporal bone was done systematically before surgery. VT included three tests: head-impulse test, caloric tests, vestibular evoked myogenic potentials. VF was graded in: normal (type 1), partial dysfunction (type 2), severe dysfunction and areflexia (type 3). In 12 cases, VT was done before and after CI. Vestibular function was analyzed looking to side, etiology and preoperative status. Results Mean age at CI was 2.9 years. Bilateral inner ear malformation were retrieved in 16%. Before surgery, 50% of children had normal vestibular responses, 4/12 had bilateral type 2, two had asymmetrical VF. In this group, after surgery, 2 children had VF worsening, none on the CI side only. Considering all 43 patients, post operative VT showed normal response in 48.8% and type 2&3 in 16.2%. Children had asymmetrical poorer vestibular function on the side of CI in 19%. Among them, 75% had normal contralateral VF. Sensorineural hearing loss etiologies known to be associated with vestibulopathy (Usher/Meningitis/Inner Ear Malformations/CMV) were associated to abnormal vestibular function more frequently than in other causes ( p = 0.01). Conclusion Half of the children had initial vestibular dysfunction. In our study, 20% of cochlear implantation could have worsened vestibular function. As vestibular function should be part of the choice in cochlear implantation, side of implantation and survey; and vestibular tests are uneasy to achieve in pediatric population, we propose a three-steps evaluation and gradation which allow easier comparison. [ABSTRACT FROM AUTHOR]

Sudden sensorineural hearing loss (SSNHL) is relatively rare and its physiopathology remains unclear, particularly in children. Our goal was to evaluate clinical characteristics, etiologies, management, treatment outcomes and prognostic factors in the pediatric population. We performed a retrospective chart review of all children registered for SSNHL between August 2004 and September 2017 in a tertiary care pediatric hospital. We analysed data regarding clinical symptoms, audiological characteristics, diagnostic investigations and treatment outcomes. Thirty-five patients were included. Mean age was 12 years (range 4–18 years). Male:female ratio was 15:20. Hearing loss was left-sided for 18 patients, right-sided for 12 patients and bilateral for 5 patients. Degree of hearing loss varied from mild to profound across frequencies in the 40 ears studied. Thirty-four patients had associated otologic symptoms: the most frequent was tinnitus (28 ears), followed by vertigo (23 ears), otalgia (5 ears) and sensation of blocked ear (5 ears). Twenty-nine patients received systemic steroids and 3 intra-tympanic steroids. In the treated group, 69% had improvement on the audiograms (14% total, 55% partial). Vestibular tests were performed in 16 patients and were abnormal in 10 patients. Radiological examination included computed tomography scan (n = 16) and/or magnetic resonance imaging (n = 33). They revealed 2 bilateral enlarged vestibular aqueducts, 1 labyrinthitis, 1 intra-cochlear haemorrhage. SSNHL can affect speech and language development in children. There are differences among the pediatric population, including inner ear malformation and immune disease. Specific work up is proposed. Appropriate diagnosis and therapeutic management are discussed. • Pediatric acute deafness is important to recognize as it affects speech development. • Specific workup should be proposed as etiologies are different from adults. • Associated otologic symptoms do not seem to be prognostic factors. • Intra-tympanic injections should be considered as salvage therapy in children. • We encourage practitioners to publish their experience to beef up limited data. [ABSTRACT FROM AUTHOR]