The first description of large vestibular aqueduct (LVA) was by Carlo Mondini in 1791 from his dissection of the temporal bones of a boy born deaf (Mondini, 1791) Mondini not only described and drew the LVA which he measured at seven lines or about 15 mm wide, but he opened the voluminous endo-lymphatic sac which he found to be 'full of a rather viscous humour, clear and yellowish which obvious...

We read with interest the paper by Propst et al.1 concerning temporal bone imaging by computed tomography (CT) in patients with GJB2-related deafness. The GJB2 gene, encoding the connexin 26 protein, which is involved in gap junction formation, has been found to be the most common cause of autosomal recessive nonsyndromic sensorineural hearing loss (SNHL) in numerous world populations.2 Several...

OBJECTIVES To determine the prevalence of inner ear anomalies and the frequency of different anomaly types among cochlear implant recipients. METHODS This study included a retrospective chart review of all patients who received cochlear implants between January 2009 and January 2013 in King Abdulaziz University Hospital cochlear implant program in Riyadh, Saudi Arabia. All subjects underwent ...

Turner syndrome (TS) is the human being's most frequent sex chromosome abnormality. Progressive sensorineural hearing loss is documented in more than 50% of the women affected by this syndrome. Although Mondini defect is the cochlear congenital malformation most frequently identified in other polymalformative syndromes, it has rarely been reported in TS. We describe the case of a 32-year-old wo...