Hyperacusis correlates with active and chronic-recurrent Vogt-Koyanagi-Harada disease

ImportanceVogt-Koyanagi-Harada disease (VKH) is a severe ocular disorder with multi-organ affection including the ear. Few studies have evaluated otolaryngologic manifestations. Current classification criteria of uveitis only includes tinnitus and disacusis. ObjectiveTo describe audiological and vestibular findings of VKH in Chile, and determine correlations with disease activity. DesignCross sectional study of Chilean patients with VKH. SettingPatients assessed between 2018 and 2021 with demographics, ophthalmologic, audiological and otolithic evaluations. ParticipantsSubjects accomplishing VKH diagnostic criteria of the International Nomenclature Committee in Uveitis recruited in a referral academic center in Santiago, Chile. Main Outcome and MeasuresPrimary outcome was the correlation between VKH activity and neurotologic symptoms. Demographics, symptoms and signs were assessed by structured survey and physical examination. Disability was assessed by DHI and HIIE-S. Neuro-otologic diagnostics tests were cVEMP, audiogram, suprathreshold click ABR, MLAEP, and DPOAE. Complete case correlation analysis was performed. Results54 patients were evaluated. 65% reported ear symptoms at disease presentation. 32% of ear symptoms started synchronically and 30% started before eye symptoms. Ear and vestibular symptoms produced predominantly mild disability. The history of nausea, falls or hyperacusis during presentation correlated with disease activity at study evaluation. Hyperacusis at evaluation correlated with active or chronic-recurrent VKH. Neurotologic tests revealed mild ear impairment and few correlations with eye disease. ConclusionsOtolaryngology features are frequent in VKH and may precede eye disease. Hyperacusis at evaluation may indicate current or persistent ocular disease. A broader spectrum of neurotologic symptoms must be considered in current classification of uveitis. Key PointsO_ST_ABSQuestionC_ST_ABSAre there characteristic findings in the hearing and otolithic function of patients with Vogt-Koyanagi-Harada disease (VKH), and do they correlate to the eye disease activity? FindingsIn a cross sectional study of 54 patients with VKH, a high frequency of neuro-otologic manifestations, and some associations to activity, chronicity and recurrence of VKH were found. Importantly, some of the ear symptoms may precede VKH diagnosis. MeaningVKH has neurotologic features that can aid diagnosis and staging of the disease. Thus, physicians must be aware of these manifestations.