Otolaryngology Manifestations of Primary Ciliary Dyskinesia: A Multicenter Study.

OBJECTIVE: This project aims to prospectively and objectively assess otolaryngological manifestations and quality of life of children with primary ciliary dyskinesia (PCD) and compare these findings with healthy pediatric controls. STUDY DESIGN: Cross-sectional. SETTING: Two high-volume pediatric PCD specialty centers. METHODS: Standardized clinical assessment; Sino-Nasal Outcome Test 22 (SNOT-22); Hearing Environment and Reflection Quality of Life (HEAR-QL); Reflux Symptom Index (RSI); standardized physical examination of the sinonasal, laryngeal, and otological systems; and investigations including pure-tone audiograms (PTAs) and sinonasal cultures were collected. RESULTS: Forty-seven children with PCD and 25 control participants were recruited. Children with PCD had more upper airway symptoms than healthy children. They had significantly higher scores in both SNOT-22 and RSI, indicating worse sinonasal and reflux symptoms, with worse quality of life on the HEAR-QL index compared to healthy children (P < .05). Fifty-two percent of children with PCD-related hearing loss were not aware of their hearing deficit that was present on audiological assessment, and only 23% of children who had ventilation tubes had chronic otorrhea, most of which was easily controlled with ototopic drops. Furthermore, although all children with PCD had chronic rhinosinusitis, only 36% of them were using topical nasal treatment. The most common bacteria cultured from the middle meatus were Staphylococcus aureus in 11 of 47 (23%), followed by Streptococcus pneumoniae in 10 of 47 (21%). CONCLUSION: This multisite cohort highlights the importance of otolaryngology involvement in the management of children with PCD. More rigorous otolaryngological management may lead to reductions in overall morbidity and improve quality of life for children with PCD.

Importancia clínica de la prueba calórica mínima / Clinical importance of the minimal caloric test

Introducción: En la evaluación de la patología vestibular habitualmente se utiliza la prueba calórica clásica de Fitzgerald y Hallpike (PCC). Sin embargo, esta sólo evalúa la función del nervio vestibular superior a través de la estimulación del canal semicircular lateral. La prueba calórica mínima (PCM) permite evaluar tanto la función del nervio vestibular superior como del nervio vestibular inferior a través de la estimulación del canal semicircular lateral y posterior respectivamente. Se desconoce el real impacto de la PCM en la evaluación clínica de pacientes con sintomatología vestibular. Objetivo: Cuantificar la utilidad diagnóstica de la PCM en pacientes con sintomatología vestibular. Material y método: Estudio diagnóstico transversal. Se obtuvo la sensibilidad y especificidad de la PCM al comparar la respuesta del canal semicircular lateral en la PCM con la prueba calórica convencional. Se evaluó en cuántos casos la información sobre la función del nervio vestibular inferior aportada por la PCM fue relevante en el diagnóstico vestibular. Resultados: Se estudió a 44 pacientes. Tanto la sensibilidad como la especificidad de la PCM para detectar patología al estimular el canal semicircular lateral fue de 93 por ciento (IC95 por ciento: 84 por ciento-100 por ciento y 80 por ciento-100 por ciento respectivamente). En 16 por ciento (N =7) de los casos la PCM detectó patología del nervio vestibular inferior con PCC normal. Conclusiones: La PCM es un examen complementario útil en la evaluación de pacientes con sintomatología vestibular, puesto que tiene una alta sensibilidad y especificidad para detectar patología del nervio vestibular superior a través de la estimulación del canal semicircular lateral y además, aporta información sobre el estado del nervio vestibular inferior a través de la estimulación del canal semicircular posterior, que no aporta la PCC.

Introduction: The classic Fitzgerald-Hallpike caloric test (CCT) is usually utilized for evaluation of vestibular pathology. With this test, however, superior vestibular nerve function is assessed through stimulation of the lateral semicircular canal only. The minimal caloric test (MCT) allows for evaluation of both superior and inferior vestibular nerve function, through horizontal and posterior semicircular canals stimulation, respectively. The actual impact of MCT on the clinical evaluation of patients presenting with vestibular symptoms is unknown. Purpose: To quantify the diagnostic usefulness of MCT in patients with vestibular symptoms. Material and Method: Transversal diagnostic study. Sensitivity and specificity of MCT were obtained by comparing the lateral semicircular canal response in both MCT and the conventional caloric test. We assessed the number of cases in which the vestibular nerve function information obtained with MCT was relevant for vestibular diagnostic. Results: 44 patients were studied. The sensitivity and specificity of MCT to detect pathologic conditions via lateral semicircular canal stimulation were 93 percent (Cl 95 percent: 84 percent-100 percent and 80 percent-100 percent, respectively). In 16 percent (n =7) of the cases, MCT detected inferior vestibular nerve involvement with normal CCT Conclusions: MCT is a complementary test, which can be useful for evaluation of patients with vestibular symptoms, given its high sensitivity and specificity to detect superior vestibular nerve pathology through lateral semicircular canal stimulation. In addition, it provides information on the inferior vestibular nerve status, through posterior semicircular canal stimulation. The latter is not provided by conventional CCT.