Congenital cytomegalovirus (CMV) infection as a cause of permanent bilateral hearing loss: A quantitative assessment

Grosse, Scott D.; Ross, Danielle S.; Dollard, Sheila C.

doi:10.1016/j.jcv.2007.09.004

Cited by 316 publications

(253 citation statements)

References 30 publications

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“…A recent review of multiple studies found that of infected infants, 13% had symptoms at birth, and in 0.5%, the infection proved fatal (63). Approximately 20% of the totalprimarily, but not exclusively, those symptomatic at birth - suffer from permanent sequelae, commonly sensorineural hearing loss (64). Seronegative mothers who become infected during pregnancy have a much higher risk of transmitting the virus and of bearing an affected infant compared with women who are seropositive at conception, but infants of the latter group can also suffer sequelae (65).…”

Section: Infection and Diseasementioning

confidence: 99%

Cytomegalovirus: pathogen, paradigm, and puzzle

Boeckh

Geballe²

2011

J. Clin. Invest.

315

258

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Section: Infection and Diseasementioning

confidence: 99%

Cytomegalovirus: pathogen, paradigm, and puzzle

Boeckh

Geballe²

2011

J. Clin. Invest.

315

258

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“…9,10,[12][13][14]20 Certain environmental (nongenetic) factors play a major etiologic role in hearing loss. 59 In the United States, congenital CMV infection is the most common nongenetic cause of hearing loss among children. Of the 20,000-40,000 infants born with congenital CMV infection each year, 90% have no detectable clinical abnormalities at birth, yet 10-15% of these asymptomatic infants will develop sensorineural hearing loss which can present in early childhood, can be unilateral or bilateral, and is often progressive.…”

Section: Genetic and Nongenetic Etiologies Of Hearing Lossmentioning

confidence: 99%

American College of Medical Genetics and Genomics guideline for the clinical evaluation and etiologic diagnosis of hearing loss

Alford

Arnos

Fox

et al. 2014

Genetics in Medicine

214

134

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DEFINITIONSDeaf: a community with a distinct culture and language shaped by the experience of being deaf or hard of hearing, which may include deaf, hard-of-hearing, and hearing individuals deaf: an auditory phenotype characterized by a total or neartotal loss of the ability to hear hard of hearing: an auditory phenotype characterized by a partial loss of the ability to hear hearing loss: an auditory phenotype characterized by any degree of loss of the ability to hear; depending on cause, hearing loss can be temporary or permanent-this guideline focuses on permanent hearing loss Hearing loss is a common and complex condition that can occur at any age, can be inherited or acquired, and is associated with a remarkably wide array of etiologies. The diverse causes of hearing loss, combined with the highly variable and often overlapping presentations of different forms of hearing loss, challenge the ability of traditional clinical evaluations to arrive at an etiologic diagnosis for many deaf and hard-of-hearing individuals. However, identifying the etiology of a hearing loss may affect clinical management, improve prognostic accuracy, and refine genetic counseling and assessment of the likelihood of recurrence for relatives of deaf and hard-of-hearing individuals. Linguistic and cultural identities associated with being deaf or hard of hearing can complicate access to and the effectiveness of clinical care. These concerns can be minimized when genetic and other health-care services are provided in a linguistically and culturally sensitive manner. This guideline offers information about the frequency, causes, and presentations of hearing loss and suggests approaches to the clinical evaluation of deaf and hard-of-hearing individuals aimed at identifying an etiologic diagnosis and providing informative and effective patient education and genetic counseling.

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“…Congenital cytomegalovirus (CMV) is the one of the most common causes of sensorineural hearing loss in children and an important cause of neurodevelopmental delay (e.g., Demmler 1991; Grosse et al 2008;Foulon et al 2008Foulon et al , 2012Manicklal et al 2013;Delany et al 2014). The incidence of CMV infection within various geographic regions is estimated to be about 0.5 %.…”

Section: Introductionmentioning

confidence: 99%

Cytomegalovirus (CMV) Infection Causes Degeneration of Cochlear Vasculature and Hearing Loss in a Mouse Model

Carraro

Almishaal

Hillas

et al. 2016

JARO

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Cytomegalovirus (CMV) infection is one of the most common causes of congenital hearing loss in children. We have used a murine model of CMV infection to reveal functional and structural cochlear pathogenesis. The cerebral cortex of Balb/c mice (Mus musculus) was inoculated with 2000 pfu (plaque forming units) of murine CMV on postnatal day 3. At 6 weeks of age, cochlear function was monitored using auditory brainstem response (ABR) and distortion product otoacoustic emission (DPOAE) measures. Histological assessment of cochlear vasculature using a corrosion cast technique was made at 8 weeks. Vascular casts of mCMV-damaged cochleas, and those of untreated control animals, were examined using scanning electron microscopy. We find very large variations in the degree of vascular damage in animals given identical viral injections (2000 pfu). The primary lesion caused by CMV infection is to the stria vascularis and to the adjacent spiral limbus capillary network. Capillary beds of the spiral ligament are generally less affected. The initial vascular damage is found in the mid-apical turn and appears to progress to more basal cochlear regions. After viral migration to the inner ear, the stria vascularis is the primary affected structure. We suggest that initial auditory threshold losses may relate to the poor development or maintenance of the endocochlear potential caused by strial dysfunction. Our increased understanding of the pathogenesis of CMV-related hearing loss is important for defining methods for early detection and treatment.

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Congenital cytomegalovirus (CMV) infection as a cause of permanent bilateral hearing loss: A quantitative assessment

Cited by 316 publications

References 30 publications

Cytomegalovirus: pathogen, paradigm, and puzzle

Cytomegalovirus: pathogen, paradigm, and puzzle

American College of Medical Genetics and Genomics guideline for the clinical evaluation and etiologic diagnosis of hearing loss

Cytomegalovirus (CMV) Infection Causes Degeneration of Cochlear Vasculature and Hearing Loss in a Mouse Model

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