ABSTRACT
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.
Subject(s)
Anemia, Sickle Cell/epidemiology , Hearing Loss, Sensorineural/epidemiology , Adolescent , Child , Evoked Potentials, Auditory, Brain Stem/physiology , Female , Humans , Male , Otoacoustic Emissions, Spontaneous/physiology , Retrocochlear Diseases/epidemiologyABSTRACT
OBJECTIVE: The aim of this prospective study was to audiologically evaluate consecutive glaucoma patients with or without exfoliation. STUDY DESIGN: Prospective study. SETTING: Glaucoma Unit and Audiology Department at a university hospital. SUBJECTS AND METHODS: Consecutive subjects with exfoliative glaucoma (XFG) or primary open-angle glaucoma (POAG) aged between 50 and 70 years were enrolled. Auditory thresholds at 0.5, 1.0, 2.0, 4.0, and 8.0 Hz were measured bilaterally. Cochlear activity was assessed by recording distortion product otoacoustic emissions (DPOEs). Functional changes in the retrocochlear auditory pathway were evaluated by auditory brainstem responses (ABRs). RESULTS: One hundred and ten patients with XFG and 85 patients with POAG who presented in a glaucoma clinic were investigated. The mean age of study patients was 66.2 ± 5.6 years; range, 50-70 years). The odds of pathologic ABR central transmission time (interpeak latencies I-III, III-V, and I-V and waves I, III, and V) were 4.34 times higher in patients with XFG than in patients with POAG (95% confidence interval [CI], 2.22-8.49; P < .001). This significant association remained after adjusting for sex and age (odds ratio [OR] 4.12; 95% CI, 2.07-8.22; P < .001). Furthermore, the odds of ABR remained significantly higher in patients with XFG than in patients with POAG (OR 4.36; 95% CI, 2.10-9.06; P < .001) after controlling for systemic diseases (arterial hypertension, coronary heart disease, high cholesterol, and stroke). CONCLUSION: In the first study to compare XFG and POAG monitoring of the peripheral and central auditory pathway, it has been documented that XFG patients show a greater prevalence of retrocochlear pathology.
