Article

What is the optimal workup for a child with bilateral sensorineural hearing loss?

Division of Pediatric Otolaryngology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. U.S.A.. .
The Laryngoscope (Impact Factor: 2.14). 04/2013; 123(4):809-10. DOI: 10.1002/lary.23425
Source: PubMed

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    ABSTRACT: Purpose of review: Cytomegalovirus (CMV) is an important cause of congenital sensorineural hearing loss (SNHL) that may represent up to 20% of nonsyndromic cases. Congenital CMV is an unrecognized cause of SNHL that all otolaryngologists should be aware of as it represents the only treatable cause of congenital SNHL. Recent findings: The current review highlights the recent advances on congenital CMV, including methods of diagnosis and prevention of progression of hearing loss with valganciclovir treatment. A recent study has shown that 6 months of oral valganciclovir treatment of infants with congenital CMV disease improves audiologic and neurodevelopmental outcomes to at least 2 years of age. Early treatment could, thus, have an important impact on these children. Summary: Increased awareness of congenital CMV SNHL by an otolaryngologist should lead to increased testing, and thus diagnosis, of this condition in newborns diagnosed with congenital SNHL. Prompt treatment with valganciclovir in these patients may consequently lead to prevention of progressive SNHL. Further research toward a CMV vaccine is hoped to eventually lead to prevention of congenital CMV.
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    ABSTRACT: Objectives/HypothesisTo determine the feasibility and cost effectiveness of incorporating cytomegalovirus (CMV) testing to determine the etiology of pediatric hearing loss. Study DesignRetrospective study of children presenting with sensorineural hearing loss (SNHL) at one institution from 2008 to 2013. Methods Children aged 3 years or younger who presented to the senior author (A.P.) between May 2008 and September 2013 with confirmed SNHL were evaluated. These children underwent a sequential diagnostic paradigm that incorporated CMV testing if no obvious etiology could be determined from the history or physical examination. ResultsOne hundred eleven children with SNHL were evaluated between 2008 and 2013. Eighty-three children underwent CMV testing, imaging, and a genetic evaluation. Those with confirmed or probable CMV-induced SNHL made up 30% of all children tested (n=25), the largest group identified. CMV screening had the lowest cost compared to genetic testing or imaging for all types of hearing loss, except for those with auditory neuropathy spectrum disorder. Conclusion We present the first sequential diagnostic paradigm utilizing CMV testing for children presenting with SNHL. The relatively high incidence of CMV-induced SNHL, the low cost for this assay, and the indirect benefits from early diagnosis support the role of early CMV testing for these patients. Level of Evidence4. Laryngoscope, 124:2624-2629, 2014
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    ABSTRACT: Enlarged vestibular aqueduct is the most common radiographically identified cause of congenital sensorineural hearing loss and is frequently progressive. Imaging is often ordered during the workup of children with congenital sensorineural hearing loss in part to identify enlarged vestibular aqueduct given concern for progression with head trauma. However, this association has not been systematically evaluated. We aimed to determine the rate of progression and association with head trauma in individuals with enlarged vestibular aqueduct. Systematic review of primary studies identified through PubMed, Embase, Cochrane, and Web of Science. Meta-analysis was performed on patient-level data describing enlarged vestibular aqueduct, progressive sensorineural hearing loss, and head trauma extracted from articles identified on systematic review according to PRISMA guidelines. Twenty-three studies (1115 ears with enlarged vestibular aqueduct) met inclusion criteria. Progressive sensorineural hearing loss was found in 39.6% of ears, with trauma-associated progression in 12%. Limited case-control data show no difference in the incidence of progression between patients with and without head trauma. Long-term progressive sensorineural hearing loss is common in enlarged vestibular aqueduct, but its association with head trauma is not strongly supported. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2015.
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