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Analysis of Hearing Loss in Patients with Enlarged Vestibular Aqueduct Syndrome
Author(s) -
AjosePopoola Bunmi,
Wilkin Abigail,
Huang Lin,
Kenna Margaret A.,
Gopen Quinton
Publication year - 2011
Publication title -
the laryngoscope
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.181
H-Index - 148
eISSN - 1531-4995
pISSN - 0023-852X
DOI - 10.1002/lary.22268
Subject(s) - laryngology , otorhinolaryngology , medicine , otology , medical school , gerontology , general surgery , ophthalmology , surgery , medical education
describing frequency, type, and clinical course of hearing loss (HL) in patients with EVA. Methods: The medical records and imaging of 94 patients with unilateral or bilateral EVA between 1996 and 2010 were identified from the Children's Hospital Boston database. Eligible patients included those with temporal bone computed tomography (CT) or magnetic resonance (MRI), documented patient follow-up, and evaluation for a mutation in SLC26A4 (PDS) gene. Results: 147 ears were evaluated. All ears had sufficient baseline data to classify the degree of HL using 4 frequency pure tone average (PTA): (range, 5-130 dBHL; mean 44 dBHL; SD 29 dBHL). HL was mild in 33 (22%) ears, moderate in 51 (35%) ears, severe in 30 (20%) ears, and profound in 9 (6%) ears. Normal PTA was observed in 24 (16%) of patients. The type of HL could be defined in 77 ears. Nine (12%) had a conductive HL, 17 (22%) had sensorineural HL, and 47 (61%) had a mixed HL. The majority of Air-bone gaps (ABGs) were detected at the lower frequencies, though there was no significant correlation between the frequency of ABGs and the presence of a mutation in SLC26A4, or laterality of EVA. Individuals with SLC26A4 mutations had a similar degree of HL when compared with individuals without the mutation. In addition, in the absence of the SLC26A4 mutation, individuals with bilateral EVA did not have a significantly higher degree of HL than individuals with unilateral EVA. Conclusions: The presence of mutant SLC26A4 alleles did not have a significant association with severity of HL. Bilateral EVA was not associated with a significant degree of HL when compared to unilateral EVA. This information will be used to counsel patients and their families on the clinical course of EVA. We performed a retrospective medical chart review of the radiology records for patients seen at Children's Hospital Boston with a radiographic diagnosis of EVA. We obtained institutional review board approval from Children's Hospital Boston before beginning this study. Inclusion criteria for the study were a diagnosis of EVA in at least one ear based on CT criteria, an otologic and audiologic assessment, and available images and records for review. Ninety-four patients were found to have sufficient data for inclusion in this study. Extracted data included demographic information, known medical diagnoses, audiologic data, and any pertinent laboratory and imaging studies. The median age of enrollment was 8.3 years-old (range 1.9-26.3 years). Audiologic assessments …