Cogan syndrome as a rare cause of deafness

Cogan syndrome is a rare systemic disease characterized by the occurrence of audiovestibular and ocular symptoms. We present the case of a 62-year-old female patient who received a cochlear implant after unilateral deafness. Despite successful implantation, the disease progressed with chronic headache, recurrent otitis, fluctuating intracochlear impedances, and slowly progressing hearing loss in the contralateral...

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Cochlear implantation appears to be a viable rehabilitation option for Cogan syndrome-related deafness, but this single case report is insufficient to change standard practice; clinicians should maintain awareness of Cogan syndrome as a rare autoimmune cause of sudden or progressive sensorineural hearing loss.

Cogan syndrome is frequently misdiagnosed or diagnosed late; this case highlights the importance of recognizing rare autoimmune etiologies of hearing loss and the potential role of cochlear implantation in end-stage disease.

1. 01Cogan syndrome is a rare autoimmune condition causing both eye inflammation and inner-ear damage, including deafness.
2. 02A 62-year-old female patient with Cogan syndrome-related deafness underwent cochlear implantation.
3. 03Case published in the peer-reviewed journal HNO (German Society of Oto-Rhino-Laryngology).
4. 04This is a single case report (N=1); findings cannot be generalized without larger studies.
5. 05Cochlear implantation may be considered when medical management fails in autoimmune hearing loss.

PMID

42530644

DOI

10.1007/s00106-026-01791-9.

Journal

HNO

Publication type

case_report

Evidence level

4

Sample size

1

Population

62-year-old female with Cogan syndrome presenting with deafness and audiovestibular symptoms

Intervention

Cochlear implantation for Cogan syndrome-related deafness