Hearing changes in the contralateral ear with juvenile unilateral profound hearing loss of unknown origin

SummaryObjectives: Investigate contralateral hearing changes in patients with juvenile unilateral profound hearing loss (JUPHL) of unknown origin because patients with JUPHL have potentially bilateral hearing loss. Study design: A retrospective study. Setting: A tertiary referral center. Patients: Fifty patients with JUPHL of unknown origin included 42 unilaterally deaf patients and 8 patients with hearing loss over 70 dB HL. The age of the patients was less than 15 years old at the first visit to our outpatient clinic. Patients with inner ear anomalies were excluded using CT images. Main outcome measures: The results of pure tone audiometry (PTA) were investigated. To obtain reliable PTA results, patients were over 6 years old. The following items were examined: level, fluctuation and worsening of hearing. Results: A hearing level over 30 dB was observed at 20%, 4%, and 10% of patients in the low, middle and high frequencies, respectively. Hearing fluctuations were observed in 72%, 42% and 64% at the low, middle and high frequencies, respectively. Hearing worsening was observed in 8%, 4% and 4% at the low, middle and high frequencies, respectively. Conclusion: Seventy-two percent of patients with JUPHL had fluctuating hearing at low frequencies, and 8% of them had worsening hearing. These results imply that over 70% of patients with JUPHL may develop contralateral hearing loss and that we have to follow-up patients with unilateral juvenile profound hearing loss for a long time.