Estimated number and prevalence of patients with delayed endolymphatic hydrops in Japan: a nationwide survey

Abstract

Background

Delayed endolymphatic hydrops (DEH) is a rare disease, and the actual number of patients in Japan remains unknown.

Objective

To investigate the number and prevalence of patients with DEH in Japan.

Methods

In total, 781 departments of otolaryngology in Japan were selected for survey by stratified random sampling according to the total number of hospital beds. We sent questionnaires to the target departments and collected data regarding the number of patients with DEH who visited those departments in 2019.

Results

The overall response rate was 68.0% (531 departments). The estimate number of patients with DEH in Japan was 962, and the prevalence was calculated to be 0.8 per 100,000 population.

Conclusion

Patients with DEH were extremely rare in Japan.

Significance

This may be the first nationwide epidemiological study on the number and prevalence of patients with DEH in Japan or in the world.

Chinese Abstract

背景：迟发性内淋巴积水 (DEH) 是一种罕见病。日本的患者数量仍然未知。

目的：调查日本 DEH 患者的数量和患病率。

方法：选择日本 781 个耳鼻喉科进行调查, 根据病床总数进行分层随机抽样。 我们向这些科发送了调查问卷, 并收集了有关2019年去那里就诊的 DEH 患者人数的数据。

结果：总应答率为68.0%（531个科）。 估计在日本DEH 患者人数为 962 人, 根据计算, 患病率为每 100,000 人中 0.8 人。

结论：DEH患者在日本极为罕见。

意义：这可能是日本或世界首次关于DEH 患者数量和患病率的全国性流行病学研究。

Keywords:

• Delayed endolymphatic hydrops
• nationwide survey
• prevalence

Acknowledgements

The authors express our sincere appreciation for the assistance and cooperation of all members of the Department of Otolaryngology at the University of Toyama.

Disclosure statement

No potential conflict of interest was reported by the authors.

Additional information

Funding

This work was supported by a grant from the Ministry of Health and Welfare of Japan [Grant Number 20FC1048].