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Hearing loss in pediatric patients with isolated nonsyndromic sagittal synostosis.

OBJECTIVE: Recent studies have shown increased rates of speech, language, cognitive and behavioral abnormalities in patients with isolated nonsyndromic sagittal synostosis. Little is known regarding the prevalence and type of hearing abnormalities in children with craniosynotosis. This study characterizes hearing loss in pediatric patients with isolated nonsyndromic sagittal synostosis.

METHODS: Retrospective chart review of isolated nonsyndromic sagittal synostosis patients in a pediatric craniofacial clinic to determine the prevalence, severity, and type of hearing loss.

RESULTS: Fifteen of 57 patients with isolated nonsyndromic sagittal synostosis had hearing loss (26% with a 95% confidence interval of 14.9 to 37.7%). Twelve patients demonstrated a conductive loss and three an unspecified type of loss (no bone conduction thresholds were recorded for these patients). The prevalence of conductive hearing loss in our population was 21% with a 95% confidence interval of 10.4 to 31.6%. All cases of hearing loss were mild or moderate in severity.

CONCLUSIONS: The most common hearing impairment in patients with isolated nonsyndromic sagittal synostosis is conductive hearing loss likely secondary to middle ear effusion. These patients do not appear to have a higher frequency of middle ear effusion and conductive hearing loss than the normal population of comparable age. We conclude that there is no evidence of increased risk of hearing loss in our study population. We infer that hearing loss does not play a causative role in the elevation of risk for speech, language, behavioral and cognitive impairments which these patients experience. Health care professionals are obligated to ensure that conductive loss from middle ear effusion does not exacerbate these impairments. This obligation can safely be performed by managing hearing loss in the INSS population similar to hearing loss in comparably aged normal children.

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