ABSTRACT
PURPOSE OF REVIEW: To highlight the recent advances in the understanding of the diagnosis and management of viral inner ear disorders. Congenital sensorineural hearing loss (cSNHL), sudden sensorineural hearing loss (SSNHL), Ménière's disease, and vestibular neuritis/viral labyrinthitis are discussed. RECENT FINDINGS: Cytomegalovirus infection during pregnancy is an under-recognized cause of hearing loss and central nervous system disease amongst the general population. Prevention of maternal infection and treatment of affected newborns with ganciclovir are promising interventions. Recent evidence in SSNHL patients has resulted in recommendations against viral serology or the use of antivirals. There appears to be an increased risk of SSNHL in patients with comorbid hypertension and diabetes. The viral hypothesis of Ménière's disease remains unproven. In patients with an acute episode of vestibular neuritis, there is presently not sufficient evidence to support the routine use of corticosteroids or antiviral medications. SUMMARY: cSNHL remains the most clearly defined of the viral inner ear disorders. The evidence for viral involvement in SSNHL, Ménière's disease, and vestibular neuritis is indirect and equivocal. This review highlights the recent advancements in the diagnosis and management of these disorders.
Subject(s)
Cytomegalovirus Infections/drug therapy , Hearing Loss, Sudden/virology , Labyrinthitis/drug therapy , Meniere Disease/drug therapy , Meniere Disease/virology , Adrenal Cortex Hormones/therapeutic use , Adult , Animals , Antiviral Agents/therapeutic use , Child , Cytomegalovirus Infections/diagnosis , Female , Hearing Loss, Sensorineural/diagnosis , Hearing Loss, Sensorineural/drug therapy , Hearing Loss, Sudden/drug therapy , Hearing Loss, Sudden/physiopathology , Humans , Infant, Newborn , Labyrinthitis/virology , Male , Meniere Disease/congenital , Mice , Needs Assessment , Pregnancy , Prognosis , Risk Factors , Severity of Illness IndexABSTRACT
Four patients (7 ears) with a large vestibular aqueduct (VA) were examined for history of vertigo and vestibular function. Vertigo was observed in all the 4 patients. The caloric responses were significantly poorer in ears with a large VA than in the controls. On a patient with a large VA who had several attacks of sudden hearing loss and vertigo following minor head trauma, long-time exposure to sunshine, common cold, and exercise, vestibular function tests were performed several times; the caloric responses were found to fluctuate and direction changing apogeotropic positional nystagmus was observed. These findings suggest that in patients with a large VA, not only hearing but also the vestibular function are generally impaired. We believe than direct transmission of intracranial pressure changes to the inner ear or subsequent inner ear fluid movement through the large endolymphatic sac and duct have an influence on the cochlea and vestibule.
Subject(s)
Tomography, X-Ray Computed , Vestibular Aqueduct/abnormalities , Vestibular Diseases/congenital , Vestibular Function Tests , Adolescent , Adult , Auditory Threshold/physiology , Caloric Tests , Child , Female , Functional Laterality/physiology , Humans , Male , Meniere Disease/congenital , Meniere Disease/diagnostic imaging , Nystagmus, Physiologic/physiology , Recurrence , Reference Values , Vestibular Aqueduct/diagnostic imaging , Vestibular Diseases/diagnostic imagingABSTRACT
Mondini's deformity is the second most common malformation seen in congenital genetic deafness. It is characterized by bony and membranous abnormalities of the inner ear, with a wide range of morphological and functional abnormalities. The importance of the Mondini malformation is that the patient is at an increased risk of developing meningitis or bilateral total hearing loss (or both) at an early age. Six cases are presented which illustrate the various sequelae of the Mondini malformation including meningitis, vertigo, and auditory deterioration. Decompression of the endolymphatic sac was undertaken in three patients. Emphasis is placed on the danger of head trauma, even minor, in these patients. The necessity of early diagnosis with adequate patient counseling is stressed.