Children with unilateral cochlear nerve canal stenosis have bilateral cochleovestibular anomalies.

OBJECTIVES/HYPOTHESIS: To investigate the cochleovestibular apparatus bilaterally in children with isolated unilateral bony cochlear nerve canal (bCNC) stenosis. STUDY DESIGN: Retrospective review. METHODS: Imaging studies of children with unilateral bCNC stenosis (<1.0 mm) on computed tomography imaging (N = 36) were compared with controls imaged due to trauma without temporal bone injury (N = 32). Twenty-six measurements were obtained in each ear, assessing the bony internal auditory canal (IAC), cochlea, and vestibular end-organs, and were analyzed using one-way analysis of variance for intersubject comparisons and paired t tests for intrasubject comparisons with a Bonferroni adjustment for multiple comparisons (P = .0006). RESULTS: Patients with bCNC stenosis had a smaller IAC (P < .000) and cochlea (P < .000) on the stenotic side as compared with controls. Although the vestibular end-organ was also smaller in bCNC ears, this difference was not significant. The contralateral ear also had a smaller bCNC (P < .000) and cochlea (P < .000) as compared with controls, although to a lesser degree than the stenotic side. CONCLUSIONS: Children with unilateral bCNC stenosis have abnormal biometry of both the cochlea and the vestibular end-organ in the affected and the normal contralateral ear as compared with controls. LEVEL OF EVIDENCE: 3b Laryngoscope, 129:2403-2408, 2019.

Development of an International Odor Identification Test for Children: The Universal Sniff Test.

OBJECTIVE: To assess olfactory function in children and to create and validate an odor identification test to diagnose olfactory dysfunction in children, which we called the Universal Sniff (U-Sniff) test. STUDY DESIGN: This is a multicenter study involving 19 countries. The U-Sniff test was developed in 3 phases including 1760 children age 5-7 years. Phase 1: identification of potentially recognizable odors; phase 2: selection of odorants for the odor identification test; and phase 3: evaluation of the test and acquisition of normative data. Test-retest reliability was evaluated in a subgroup of children (n = 27), and the test was validated using children with congenital anosmia (n = 14). RESULTS: Twelve odors were familiar to children and, therefore, included in the U-Sniff test. Children scored a mean ± SD of 9.88 ± 1.80 points out of 12. Normative data was obtained and reported for each country. The U-Sniff test demonstrated a high test-retest reliability (r27 = 0.83, P < .001) and enabled discrimination between normosmia and children with congenital anosmia with a sensitivity of 100% and specificity of 86%. CONCLUSIONS: The U-Sniff is a valid and reliable method of testing olfaction in children and can be used internationally.

Novel variants in GNAI3 associated with auriculocondylar syndrome strengthen a common dominant negative effect.

Auriculocondylar syndrome is a rare craniofacial disorder comprising core features of micrognathia, condyle dysplasia and question mark ear. Causative variants have been identified in PLCB4, GNAI3 and EDN1, which are predicted to function within the EDN1-EDNRA pathway during early pharyngeal arch patterning. To date, two GNAI3 variants in three families have been reported. Here we report three novel GNAI3 variants, one segregating with affected members in a family previously linked to 1p21.1-q23.3 and two de novo variants in simplex cases. Two variants occur in known functional motifs, the G1 and G4 boxes, and the third variant is one amino acid outside of the G1 box. Structural modeling shows that all five altered GNAI3 residues identified to date cluster in a region involved in GDP/GTP binding. We hypothesize that all GNAI3 variants lead to dominant negative effects.

The effect of dexamethasone on post-tonsillectomy nausea, vomiting and bleeding.

UNLABELLED: It has been stated, that the administration of Dexamethasone has an impact on the morbidity following tonsillectomy. OBJECTIVE: To re-calculate the blood values for Dexamethasone when given as fixed doses and to evaluate the effect of Dexamethasone on post-operative nausea, vomiting and bleeding rates following tonsillectomy. MATERIALS AND METHODS: The charts of 272 children (2-15 years) who had undergone tonsillectomy were analyzed. The rates of post-operative nausea, vomiting and bleeding in relation to Dexamethasone were calculated-in general and different doses (0 mg/kg, <0.15 mg/kg, >0.15 mg/kg). STUDY DESIGN: Retrospective cohort study. RESULTS: Dexamethasone was administered in 121 children (43.7%) according to the preference of the anesthesist (mean dose: 0.2 +/- 0.12 mg/kg; range: 0.04 - 0.62 mg/kg). There was no significant difference in nausea and vomiting (p=0.953) or bleeding (p=0.827) across groups receiving or not receiving Dexamethasone. Stratification into three different groups of Dexamethasone concentration also did not identify a dose-related risk of postoperative nausea or vomiting (p=0.98) or bleeding (p=0.71). CONCLUSION: At least under common non-controlled conditions in the clinic, Dexamethasone does not appear to have an effect on nausea or vomiting or bleeding following tonsillectomy.

The effect of dexamethasone on post-tonsillectomy nausea, vomiting and bleeding / Efeito da dexametasona sobre sangramento, vômito e náusea pós-amigdalectomia

It has been stated, that the administration of Dexamethasone has an impact on the morbidity following tonsillectomy. OBJECTIVE: To re-calculate the blood values for Dexamethasone when given as fixed doses and to evaluate the effect of Dexamethasone on post-operative nausea, vomiting and bleeding rates following tonsillectomy. MATERIALS AND METHODS: The charts of 272 children (2-15 years) who had undergone tonsillectomy were analyzed. The rates of post-operative nausea, vomiting and bleeding in relation to Dexamethasone were calculated-in general and different doses (0 mg/kg, <0.15 mg/kg, >0.15 mg/kg). STUDY DESIGN: Retrospective cohort study. RESULTS: Dexamethasone was administered in 121 children (43.7 percent) according to the preference of the anesthesist (mean dose: 0.2 +/- 0.12 mg/kg; range: 0.04 - 0.62 mg/kg). There was no significant difference in nausea and vomiting (p=0.953) or bleeding (p=0.827) across groups receiving or not receiving Dexamethasone. Stratification into three different groups of Dexamethasone concentration also did not identify a dose-related risk of postoperative nausea or vomiting (p=0.98) or bleeding (p=0.71). CONCLUSION: At least under common non-controlled conditions in the clinic, Dexamethasone does not appear to have an effect on nausea or vomiting or bleeding following tonsillectomy.

É conhecido o impacto da administração de dexametasona sobre a morbidade no pós-operatório de amigdalectomia. OBJETIVO: Recalcular os valores séricos para dexametasona quando administrada em doses fixas e avaliar seus efeitos sobre as taxas de náusea, vômito e sangramento no pós-operatório de amigdalectomia. MATERIAIS E MÉTODOS: Analisamos os prontuários de 272 crianças (idades entre 2-15 anos) submetidas a amigdalectomias. As taxas de náusea, vômitos e sangramentos foram calculadas para a dexametasona em geral e em diferentes doses (0 mg/kg; <0,15 mg/kg; >0,15 mg/ kg). TIPO DE ESTUDO: Coorte retrospectivo. RESULTADOS: A dexametasona foi administrada em 121 crianças (43,7 por cento), baseado na preferência do anestesista (dose média: 0,2 +/- 0,12 mg/kg; variação: 0,04 - 0,62 mg/kg). Não houve diferença significativa em termos de náuseas e vômitos (p=0,953) ou sangramento (p=0,827) entre os grupos de pacientes que receberam e não receberam dexametasona. Mesmo a estratificação em três grupos de diferentes concentrações de dexametasona não identificou risco dose-dependente de náusea ou vômito pós-operatório (p=0,98) ou sangramento (p=0,71). CONCLUSÃO: Pelo menos sob condições não-controladas normais da clínica, a dexametasona parece não ter efeito sobre a incidência de náuseas, vômito ou sangramento após amigdalectomia.