OBJECTIVES/HYPOTHESIS The role of antiendothelial cell antibodies in systemic vasculitis has been reported. The aim of the study was to define the clinical associations of serum antiendothelial cell antibodies in patients with sudden sensorineural hearing loss. STUDY DESIGN A prospective study in patients with sudden sensorineural hearing loss. METHODS Serum samples were taken from 59 conse...

Five adult siblings presented with autosomal recessive sensorineural hearing loss: two had high-frequency loss, whereas the other three had severe-to-profound loss affecting all frequencies. Genetic evaluation revealed that a homozygous mutation in CDH23 (which encodes cadherin 23) caused the hearing loss in all five siblings and that a heterozygous, hypofunctional variant (V586M) in plasma-mem...

Background Harboyan syndrome, defined as congenital corneal dystrophy associated with progressive sensorineural hearing loss, was first described by Harboyan in 1971. It is a hereditary disease manifested by eye lesions consistent with corneal endothelial dystrophy and progressive sensorineural hearing loss. There is bilateral symmetric progressive hearing loss, which may be either dominant or ...

The aim of this review is to provide an updated overview of hereditary hearing loss, with special attention to the etiological diagnosis of sensorineural hearing loss, the genes most frequently mutated in our environment, the techniques available for their analysis and the clinical implications of genetic diagnosis. More than 60% of childhood sensorineural hearing loss is genetic. In adults, th...

We reported the recovery of sensorineural hearing loss and tinnitus in a 22-year-old man after complete removal of intracranial portion of jugular foramen schwannoma via the retrosigmoid approach. The aim of this case report was to present the excision of a large jugular foramen schwannoma via the retrosigmoid approach and to describe the improvement of sensorineural hearing loss related to ara...

Deployment of the air bag in a passenger vehicle accident rarely causes otologic injuries. However, sensorineural hearing loss induced by air bag deployment is extremely rare, with only a few cases reported in the English literature. A 38-year-old man involved in a traffic accident while driving his car at 40 km/hour presented with right sensorineural hearing loss and tinnitus, without associat...

Minimal Hearing Loss (MHL) is a commonly overlooked clinical problem; it affects about 5% of children. Children with MHL may be at risk academically due to their poorer speech perception in the noisy classrooms [1]. MHL has generally been defined as a group of hearing disorders that include mild hearing loss, unilateral hearing loss, and high-frequency hearing loss. MHL may result from conducti...

Conclusion: Acute bilateral sensorineural hearing loss is a well described phenomenon in the setting of leukemia but can also be seen in myelodysplastic syndrome. Given the well-recognized association of MDS with autoimmune disease, an autoimmune cause of our patient's hearing loss must be considered. Unfortunately our patient did not response to corticosteroids; more research is needed to dete...

A case study is presented of a 17-year-old male who sustained an anoxic brain injury and sensorineural hearing loss secondary to carbon monoxide poisoning. Audiological data is presented showing a slightly asymmetrical hearing loss of sensorineural origin and mild-to-severe degree for both ears. Word recognition performance was fair to poor bilaterally for speech presented at normal conversatio...

Multiple sclerosis (MS) is the most common demyelinating disease of the central nervous system. MS involves different regions of the central nervous system in different periods, and causes demyelination. MS is a neuromotor disorder which progresses with remissions and relapses. Symptoms of MS may regress completely or heal after the relapses leaving sequelae. Sudden sensorinerural hearing loss ...