Pediatric cochlear implantation in residual hearing candidates.

OBJECTIVES: To propose categories for the various types of residual hearing in children and to review the outcomes of cochlear implantation (CI) in children with these different hearing conditions. METHODS: We identified 53 children with residual hearing who had received a cochlear implant. Five groups were arbitrarily defined based on auditory features: G1, characterized by low-frequency residual hearing (n=5); G2, characterized by severe sensorineural hearing loss (SNHL) and low speech discrimination (n=12); G3, characterized by asymmetric SNHL (n=9); G4, characterized by progressive SNHL (n=15); and G5, characterized by fluctuating SNHL (n=12). The main audiometric features and outcomes of the groups were analyzed. RESULTS: The mean age at implantation was 10.15 years (range, 2.5-21 years). The mean preoperative score for the discrimination of open-set words was 48%; this score increased to 74% at 12 months and 81% at 24 months after the CI procedure (G1 to G5, respectively: 79/62/77%, 50/81/88%, 59/75/86%, 35/74/67%, and 39/69/80%). Children who were implanted after 10 years of age did not improve as much as those who were implanted at a younger age (open-set word list speech perception [OSW] score at 12 months: 62% vs 83%; P=.0009). Shorter delays before surgery were predictive of better performance (P=.003). Inner ear malformation and SLC26A4 mutations were not predictive of the outcome. CONCLUSIONS: CIs provide better results compared with hearing aids in children with residual hearing. Factors that may impact the benefits of CIs in patients with residual hearing are age, delay in performing the CI procedure, which ear is implanted, and initial underestimation of the patient's hearing difficulties.

An uncommon cause of allergic fungal sinusitis: Rhizopus oryzae.

We report what we believe is the first case of allergic fungal rhinosinusitis (AFRS) caused by the fungus Rhizopus oryzae. Our patient was a 32-year-old woman who presented with unilateral nasal polyps and chronic nasal dysfunction. Computed tomography of the sinuses detected left-sided pansinusitis and bone erosion. T2-weighted magnetic resonance imaging demonstrated a signal void that suggested the presence of a fungal infection. The patient underwent unilateral ethmoidectomy. Histologic examination of the diseased tissue identified allergic mucin with 70% eosinophils and no fungal hyphae. Mycologic culture detected R oryzae. After a short period of improvement, the patient experienced a recurrence, which was confirmed by radiology. A second surgery was performed, and the same fungal hyphae were found in the mucus and on culture, which led us to suspect AFRS. Since no IgE test for R oryzae was available, we developed a specific immunologic assay that confirmed the presence of specific IgG, which identified a high degree of immunologic reaction against our homemade R oryzae antigens. With a long course of systemic antifungal treatment, the patient's symptoms resolved and no recurrence was noted at 5 years of follow-up.

Embolization in the management of recurrent secondary post-tonsillectomy haemorrhage in children.

OBJECTIVES: The purpose of this study was to evaluate the clinical efficacy and safety of endovascular treatment (EVT) compared with iterative surgery (IS) in paediatric patients with secondary post-tonsillectomy haemorrhage (PTH) refractory to surgical haemostasis (SH). METHODS: We retrospectively identified 424 consecutive children with secondary PTH. PTH ceased spontaneously in 215 patients, but SH was required in the remaining patients, failing in 15 cases. In these 15 children, we analyzed the benefit of EVT by comparing the outcomes of the nine patients who underwent IS with the six children who underwent an EVT. RESULTS: After a first attempt at surgical haemostasis failed, the success rate of additional surgical procedures was 50% for the second procedures (6/12) and 67% (2/3) for the third. Conversely, EVT was always successful, even though no vascular source of bleeding was found in any patient. EVT did not elicit any complications. Moreover, it tended to reduce the hospitalization duration and the number of red blood cell transfusions. CONCLUSIONS: In the event of failure of the first attempt at surgical haemostasis in the presence of secondary PTH, our study suggests that in most instances, endovascular treatment is preferred to iterative surgical haemostasis, even if no vascular source of bleeding is found. KEY POINTS: • In recurrent secondary post-tonsillectomy haemorrhage, the rate of success declined with additional surgery. • Endovascular treatment was always successful and did not elicit any complications. • Embolization was useful even if no source of bleeding was found. • Embolization tended to reduce hospitalization duration and red blood cell transfusions. • Embolization may be considered as an alternative option to iterative surgery.

Restricted neural plasticity in vestibulospinal pathways after unilateral labyrinthectomy as the origin for scoliotic deformations.

Adolescent idiopathic scoliosis in humans is often associated with vestibulomotor deficits. Compatible with a vestibular origin, scoliotic deformations were provoked in adult Xenopus frogs by unilateral labyrinthectomy (UL) at larval stages. The aquatic ecophysiology and absence of body-weight-supporting limb proprioceptive signals in amphibian tadpoles as a potential sensory substitute after UL might be the cause for a persistent asymmetric descending vestibulospinal activity. Therefore, peripheral vestibular lesions in larval Xenopus were used to reveal the morphophysiological alterations at the cellular and network levels. As a result, spinal motor nerves that were modulated by the previously intact side before UL remained permanently silent during natural vestibular stimulation after the lesion. In addition, retrograde tracing of descending pathways revealed a loss of vestibular neurons on the ipsilesional side with crossed vestibulospinal projections. This loss facilitated a general mass imbalance in descending premotor activity and a permanent asymmetric motor drive to the axial musculature. Therefore, we propose that the persistent asymmetric contraction of trunk muscles exerts a constant, uncompensated differential mechanical pull on bilateral skeletal elements that enforces a distortion of the soft cartilaginous skeletal elements and bone shapes. This ultimately provokes severe scoliotic deformations during ontogenetic development similar to the human syndrome.