Characteristics of sensorineural hearing loss in children with inner ear anomalies.

PURPOSE: To determine whether hearing loss in children with inner ear anomalies has some distinctive characteristics when compared to children with hearing loss but without inner ear anomalies.
METHODS: Temporal bone computed tomography scans of 69 patients with sensorineural hearing loss were examined for inner ear abnormalities of which 17 were identified. The medical histories of these patients were reviewed for the characteristics of their hearing loss, including initial presentation, natural history, and nature of loss, as well as the family history of hearing loss and risk factors for hearing loss. These were compared to age-matched controls with hearing loss but without inner ear anomalies.
RESULTS: Seventeen patients had inner ear anomalies. Records of 14 of these patients were compared to patients without inner ear anomalies. Regarding age of onset, 71.4% of patients with anomalies had onset of their hearing loss at less than 2 years old vs 78.6% without anomalies. Regarding unilateral vs bilateral, 42.9% of patients with anomalies were unilateral vs 28.6% of patients without anomalies. For patients with anomalies, 85.7% were stable and 14.3% were progressive; without anomalies, 71.4% were stable, 21.4% were progressive, and 7.1% were fluctuating. Regarding family history, only 14.3% of patients without anomalies had a positive family history vs 56% of patients with anomalies.
CONCLUSIONS: Children with inner ear anomalies and sensorineural hearing loss have an increased incidence of unilateral hearing loss and stable hearing loss as compared to controls with sensorineural hearing loss without inner ear anomalies. In addition, children with inner ear anomalies and sensorineural hearing loss are less likely to have a family history of hearing loss.