Microangiopathy of the inner ear, deafness, and cochlear implantation in a patient with Susac syndrome.

Abstract

CONCLUSION:

The cochlear implant was beneficial as an attempt to restore hearing and improve communication abilities in this patient with profound sensorineural hearing loss secondary to Susac syndrome.

OBJECTIVE:

To report the audiological outcomes of cochlear implantation (CI) in a young woman with Susac syndrome after a 6-month follow-up period. Susac syndrome is a rare disorder. It is clinically characterized by a typical triad of sensorineural deafness, encephalopathy, and visual defect, due to microangiopathy involving the brain, inner ear, and retina.

METHODS:

This was a retrospective review of a case at a tertiary referral center. After diagnosis, the patient was evaluated by a multidisciplinary team and received a cochlear implant in her right ear.

RESULTS:

The patient achieved 100% open-set sentence recognition in noise conditions and 92% monosyllable and 68% medial consonant recognition in quiet conditions after 6 months of implant use. She reported the use of the telephone 3 months after activation.

• Authors: Aline Gomes Bittencourt, Andrea Felice Dos Santos, Maria Valeria Schmidt Goffi-Gomez, Kellen Kutscher, Robinson Koji Tsuji, Rubens De Brito, Ricardo Ferreira Bento
• Journal: Acta oto-laryngologica (Acta Otolaryngol) Vol. 131 Issue 10 Pg. 1123-8 (Oct 2011) ISSN: 1651-2251 [Electronic] England
• PMID: 21736516 (Publication Type: Case Reports, Journal Article)

Topics

• Cochlear Implantation
• Female
• Hearing Loss, Sensorineural (etiology, surgery)
• Hearing Tests
• Humans
• Susac Syndrome (complications)
• Young Adult