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Clinical Challenge
November 2016

A Rare Cause of Bilateral Conductive Hearing Loss in a Young Boy

Author Affiliations
  • 1Department of Otolaryngology, University of Connecticut Health Center, Farmington
  • 2Department of Clinical Otolaryngology, University of Connecticut School of Medicine, Farmington
  • 3Jefferson Radiology, Connecticut Children's Medical Center, Hartford
JAMA Otolaryngol Head Neck Surg. 2016;142(11):1123-1124. doi:10.1001/jamaoto.2015.3311

A young boy presented to the pediatric otolaryngology clinic with bilateral conductive hearing loss (CHL) and delayed speech development. Examination revealed normal otoscopic findings, and an audiogram demonstrated moderate low-frequency CHL of 45 dB in the right ear and mild 30-dB CHL in the left ear with a normal tympanogram (Figure, A). Two sets of tympanostomy tubes were placed the year before his initial visit for presumed otitis media–related CHL, but his hearing did not improve. An axial computed tomographic (CT) scan performed at that time was interpreted as normal. Owing to concerns of visual changes and possible associated genetic cause for hearing loss (HL), magnetic resonance imaging (MRI) was ordered 1 year after initial presentation and showed no central or peripheral otologic abnormalities. A genetic evaluation revealed no abnormalities. Follow-up audiograms at 3 and 6 months showed similar persistent CHL despite normal otoscopy results. The patient and family decided to treat the CHL with bilateral hearing aid amplification and a frequency modulation (FM) system at school. After 30 months of persistent CHL with an unknown etiology, a repeated CT scan of the temporal bones was performed (Figure, B and C). Operative intervention vs amplification was discussed, and the patient and family decided to continue with hearing aids because he had improved performance in school. Surgical treatment will be considered in the future if there is a decline in hearing.

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