A rare association between neurofibromatosis type I and Cogan's syndrome: Case report

Abstract

Sensorineural hearing loss (SNHL) has been rarely reported in neurofibromatosis type 1 (NF type 1) and literature data concerning its pathogenesis are lacking. We report a patient affected by NF type 1, who several years later developed Cogan's syndrome, with a quickly progressive bilateral SNHL, leading to total deafness. Cochlear implantation was carried out in a partially ossified cochlea, with a good outcome. On the basis of our findings, we underscore the importance of comprehensive evaluation of patients with NF type 1 who develop SNHL, to accurately evaluate the pathogenesis of the hearing loss and explore other possible causes of hearing impairment especially with the emergence of new symptoms. If a retrocochlear lesion is excluded, a cochlear implantation is a viable option for those NF type 1 patients who develop a severe to profound SNHL. We also highlight the importance of a prompt and accurate radiological study of the petrous bone in patients with Cogan's syndrome considered for cochlear implantation, in order to plan a proper surgical approach and the importance of a thorough post-implant medical follow-up.

Key words::

• neurofibromatosis type 1
• Cogan's syndrome
• deafness
• ossified cochlea

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.