A Pediatric Case of Cogan’s Syndrome With Internal Otitis

TL;DR

A 10-year-old boy with Cogan’s syndrome showed inner ear inflammation, not Ménière’s disease, highlighting the need for careful timing of cochlear implant surgery.

Contribution

This case highlights internal otitis as a cause of auditory dysfunction in Cogan’s syndrome and emphasizes surgical timing challenges.

Findings

MRI showed no endolymphatic hydrops but enhanced contrast effects in the cochlea and vestibular apparatus.

The patient’s hearing did not recover with drugs, necessitating cochlear implants.

Timing of surgery was complicated by inner ear fibrosis and drug treatment status.

Abstract

Cogan’s syndrome is characterized by ocular symptoms and auditory vestibular dysfunction. Auditory vestibular dysfunction in Cogan’s syndrome is believed to be similar to Ménière’s disease, but the cause is not known in detail. We present the case of a 10-year-old boy with Cogan’s syndrome. The patient had panuveitis, bilateral hearing loss, and bilateral vestibular dysfunction. MRI revealed no evidence of endolymphatic hydrops, which is a cause of Ménière’s disease, and enhanced contrast effects on the bilateral cochlear and vestibular apparatus. The caloric test, the video-head impulse test, and the vestibular evoked muscle potential test also showed severe vestibular dysfunction. Based on the above, the auditory vestibular dysfunction in this patient was considered to be caused by internal otitis. The patient’s vision recovered after treatment with steroids and immunosuppressive drugs, but his hearing did not recover. He underwent bilateral cochlear implantation and had a good postoperative course but we encountered difficulty in deciding when to perform cochlear implantation. This case demonstrates the importance of determining the timing of surgery in consideration of the ossification and fibrosis of the inner ear and the drug administration status.

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