Dysfunction of the auditory system in sickle cell anaemia: a systematic review with meta-analysis.

OBJECTIVES: To describe the occurrence of both peripheral and central auditory system dysfunction in sickle cell anaemia (SCA) patients and discuss the different mechanisms hypothesised to be responsible for these alterations. METHODS: An electronic search was conducted using PubMed Central® (MEDLINE), LILACS® and Scopus® databases. This systematic review was performed in accordance with the PRISMA statement. Original observational studies that utilised audiological tests for auditory system evaluations in SCA were included. RESULTS: A total of 183 records were found in the databases searched. Twenty-one of these studies met the inclusion criteria. Pooled prevalence of sensorineural hearing loss (SNHL) was 20.5% (CI: 10.3-33%). Retrocochlear involvement was detected with the auditory brainstem response, which assesses integrity of the cochlea, 8th cranial nerve and brainstem structures. In addition, the increase in otoacoustic emission amplitudes in SCA patients indicates changes in cochlear micromechanics and precedes the expression of a detectable cochlear pathology. CONCLUSION: The prevalence of the SNHL is higher than in the general population. Dysfunction of the auditory system may be present in SCA patients, with the most probable mechanism being the presence of circulatory disturbances resulting from the chronic inflammatory state of the disease.

DYSFONCTIONNEMENT DU SYSTÈME AUDITIF DANS L'ANÉMIE FALCIFORME: REVUE SYSTÉMATIQUE ET MÉTA-ANALYSE: OBJECTIFS: Décrire l'apparition de dysfonctionnements du système auditif périphérique et central chez les patients atteints d'anémie falciforme (AF) et discuter des différents mécanismes supposés responsables de ces altérations. MÉTHODES: Une recherche électronique a été effectuée dans les bases de données PubMed Central® (MEDLINE), LILACS® et Scopus® . Cette revue systématique a été réalisée conformément au PRISMA Statement. Des études observationnelles originales utilisant des tests audiologiques pour évaluer le système auditif dans l'AF ont été incluses. RÉSULTATS: Un total de 183 enregistrements ont été trouvés dans les bases de données consultées. Vingt et une de ces études répondaient aux critères d'inclusion. La prévalence poolée de la perte auditive neurosensorielle (SNHL) était de 20,5% (IC: 10,3% à 33%). L'atteinte rétrocochléaire a été détectée avec la réponse du tronc cérébral auditif, qui évalue l'intégrité de la cochlée, du huitième nerf crânien et de la structure du tronc cérébral. De plus, l'augmentation des amplitudes des émissions otoacoustiques chez les patients atteints de l'AF indique des modifications de la micromécanique cochléaire et précède l'expression d'une pathologie cochléaire détectable. CONCLUSION: La prévalence de la SNHL est plus élevée que dans la population générale. Un dysfonctionnement du système auditif peut être présent chez les patients AF, le mécanisme le plus probable étant la présence de troubles circulatoires résultant de l'état inflammatoire chronique de la maladie.

Is It Safe for Patients With Cardiac Channelopathies to Undergo Routine Dental Care? Experience From a Single-Center Study.

Background Brugada syndrome and long-QT syndrome may account for at least one third of unexplained sudden cardiac deaths. Dental care in patients with cardiac channelopathies is challenging because of the potential risk of life-threatening events. We hypothesized that the use of local dental anesthesia with lidocaine with and without epinephrine is safe and does not result in life-threatening arrhythmias in patients with channelopathies. Methods and Results We performed a randomized, double-blind pilot trial comparing the use of 2% lidocaine without a vasoconstrictor and with 1:100 000 epinephrine in 2 sessions of restorative dental treatment with a washout period of 7 days (crossover trial). Twenty-eight-hour Holter monitoring was performed, and 12-lead electrocardiography, digital sphygmomanometry, and anxiety scale assessments were also conducted at 3 time points. Fifty-six dental procedures were performed in 28 patients (18 women, 10 men) with cardiac channelopathies: 16 (57.1%) had long-QT syndrome, and 12 (42.9%) had Brugada syndrome; 11 (39.3%) of patients had an implantable defibrillator. The mean age was 45.9±15.9 years. The maximum heart rate increased after the use of epinephrine during the anesthesia period from 82.1 to 85.8 beats per minute (P=0.008). In patients with long-QT syndrome, the median corrected QT was higher, from 450.1 to 465.4 ms (P=0.009) at the end of anesthesia in patients in whom epinephrine was used. The other measurements showed no statistically significant differences. No life-threatening arrhythmias occurred during dental treatment. Conclusions The use of local dental anesthesia with lidocaine, regardless of the use of a vasoconstrictor, did not result in life-threatening arrhythmias and appears to be safe in stable patients with cardiac channelopathies. Clinical Trial Registration URL: http://www.clinicaltrials.gov. Unique identifier: NCT03182777.

Favorable Safety Experience of Local Dental Anesthesia in ICD Recipients with Cardiac Channelopathies

Abstract Background Dental anesthetic management in implantable cardioverter defibrillator (ICD) recipients with cardiac channelopathies (CCh) can be challenging due to the potential risk of life-threatening arrhythmias and appropriate ICD therapies during procedural time. Objectives The present study assessed the hypothesis that the use of local dental anesthesia with 2% lidocaine with 1:100,000 epinephrine or without a vasoconstrictor can be safe in selected ICD and CCh patients, not resulting in life-threatening events (LTE). Methods Restorative dental treatment under local dental anesthesia was made in two sessions, with a wash-out period of 7 days (cross-over trial), conducting with a 28h - Holter monitoring, and 12-lead electrocardiography, digital sphygmomanometry, and anxiety scale assessments in 3 time periods. Statistical analysis carried out the paired Student's t test and the Wilcoxon signed-rank test. In all cases, a significance level of 5% was adopted. All patients were in stable condition with no recent events before dental care. Results Twenty-four consecutive procedures were performed in 12 patients (9 women, 3 men) with CCh and ICD: 7 (58.3%) had long QT syndrome (LQTS), 4 (33.3%) Brugada syndrome (BrS), and 1 (8.3%) Catecholaminergic polymorphic ventricular tachycardia (CPVT). Holter analysis showed no increased heart rate (HR) or sustained arrhythmias. Blood pressure (BP), electrocardiographic changes and anxiety measurement showed no statistically significant differences. No LTE occurred during dental treatment, regardless of the type of anesthesia. Conclusion Lidocaine administration, with or without epinephrine, can be safely used in selected CCh-ICD patients without LTE. These preliminary findings need to be confirmed in a larger population with ICD and CCh.