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

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Abstract

Importance

Vogt-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.

Objective

To describe audiological and vestibular findings of VKH in Chile, and determine correlations with disease activity.

Design

Cross sectional study of Chilean patients with VKH.

Setting

Patients assessed between 2018 and 2021 with demographics, ophthalmologic, audiological and otolithic evaluations.

Participants

Subjects accomplishing VKH diagnostic criteria of the International Nomenclature Committee in Uveitis recruited in a referral academic center in Santiago, Chile.

Main Outcome and Measures

Primary 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.

Results

54 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.

Conclusions

Otolaryngology 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 Points

Question

Are 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?

Findings

In 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.

Meaning

VKH has neurotologic features that can aid diagnosis and staging of the disease. Thus, physicians must be aware of these manifestations.

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