Cochlear implantation in a patient with Cogan’s syndrome and bilateral deafness

Introduction

Cogan’s Syndrome is a rare and typically relapsing disease of the audiovestibular and ocular system. Affected patients often suffer from progressive bilateral hearing loss. An autoimmune vasculitis is assumed to be the etiology of the disease.

Case Report

A 44-year-old patient with known Cogan’s syndrome and progressive bilateral hearing loss presented herself in our clinic inquiring about a cochlear implantation. MRI and CT imaging showed a normal anatomy. In particular both, cochlear and vestibular system, were filled with fluid. In addition to the hearing loss the patient suffered from bilateral vestibulopathy. During the surgery pronounced sclerosis of the round window membrane was detected. A test electrode was placed intracochlearly due to complicated opening of the cochlea via the round window. Afterwards, a CI612-electrode from Cochlear Ltd. could be completely inserted. Intraoperative measurements revealed low resistance of the electrodes with partially low NRT. Intraoperative DVT imaging confirmed a correct intracochlear placement of the electrode. Only seven weeks after surgery the patient reported a significant hearing improvement. The Freiburg monosyllabic test showed a speech intelligibility of 75% at 65dB.

Conclusion

Even if imaging does not provide definitive evidence, intracochlear sclerosis must be expected in patients with Cogan’s syndrome. In cases with progressive hearing loss early cochlear implantation should therefore be considered. In most cases cochlear implantation provides good results.

Publikationsverlauf

Artikel online veröffentlicht:
24. Mai 2022