Scoping Review of Cochlear Implantation in Susac's Syndrome

Overview

Journal World J Otorhinolaryngol Head Neck Surg

Date 2021 May 17

PMID 33997722

Citations 3

Authors

Philip L Perez

Andrew A McCall

Barry E Hirsch

Affiliations

Soon will be listed here.

Abstract

Objective: Scoping review of published literature to establish clinical characteristics and audiologic outcomes in patients diagnosed with Susac's Syndrome(SS) who have undergone cochlear implantation (CI).

Data Sources: All published studies of CI in SS and contribution of two of our own patients who have not been reported previously.

Methods: A comprehensive search of MEDLINE (via PubMed) was carried out in March 2020 using the following keywords and related entry terms: Susac's Syndrome, Cochlear Implantation.

Results: Our search identified a total of five case reports of CI in SS. With the addition of our two patients reported here, we analyzed characteristics and outcomes in seven patients. Mean age at implantation was 30 years old (range 19-46), with six women and one man implanted. Mean time from onset of hearing loss to implantation was 17 months (range three months to four years). Best reported postoperative speech understanding was reported via different metrics, with six of seven patients achieving open set speech scores of 90% or better, and one subject performing at 68%. Vestibular symptoms were present preoperatively in four of seven patients (57%), with vestibular testing reported in two patients, and showing vestibulopathy in one patient. No complications were reported following cochlear implantation.

Conclusion: Cochlear implantation is a viable option for hearing rehabilitation in patients with SS, with levels of attainment of open set speech comparable to other populations of CI candidates.

Citing Articles

Impact of dexmedetomidine on agitation and inflammatory response during recovery from anesthesia in young children following cochlear implantation surgery.

Cheng Q, Chen C, Li X, Wu L, Zhao Z BMC Anesthesiol. 2025; 25(1):102.

PMID: 40000934 PMC: 11852807. DOI: 10.1186/s12871-025-02970-x.

Phenotyping vestibulocochlear manifestations in Susac syndrome: a cohort study.

Roelens A, Vandekerckhove M, Maes L, Dekeyser C, Hemelsoet D, Van Driessche V Eur Arch Otorhinolaryngol. 2024; 282(3):1189-1200.

PMID: 39379650 DOI: 10.1007/s00405-024-09011-2.

Susac syndrome: neurological update (clinical features, long-term observational follow-up and management of sixteen patients).

Bose S, Papathanasiou A, Karkhanis S, Appleton J, King D, Batra R J Neurol. 2023; 270(12):6193-6206.

PMID: 37608221 PMC: 10632257. DOI: 10.1007/s00415-023-11891-z.

Susac Syndrome: A Rare cause of Bilateral Sensorineural hearing loss from North India-A Case Report with Review of literature.

Bhatia S, Sharma D, Bahl V, Sharma M, Gupta V, Nanda M Indian J Otolaryngol Head Neck Surg. 2022; 74(2):196-204.

PMID: 35813770 PMC: 9256882. DOI: 10.1007/s12070-021-02853-w.

References

Aftab S, Semaan M, Murray G, Megerian C . Cochlear implantation outcomes in patients with autoimmune and immune-mediated inner ear disease. Otol Neurotol. 2010; 31(8):1337-42. DOI: 10.1097/MAO.0b013e3181f0c699. View

Garcia-Carrasco M, Mendoza-Pinto C, Cervera R . Diagnosis and classification of Susac syndrome. Autoimmun Rev. 2014; 13(4-5):347-50. DOI: 10.1016/j.autrev.2014.01.038. View

Low W, See J, Ng W, Fong A, Chew S . An unusual case of post-cochlear implant performance degradation in a patient with suspected Cogan's syndrome. Cochlear Implants Int. 2018; 20(2):94-99. DOI: 10.1080/14670100.2018.1548077. View

Magro C, Poe J, Lubow M, Susac J . Susac syndrome: an organ-specific autoimmune endotheliopathy syndrome associated with anti-endothelial cell antibodies. Am J Clin Pathol. 2011; 136(6):903-12. DOI: 10.1309/AJCPERI7LC4VNFYK. View

Crawley B, Close A, Canto C, Harris J . Susac's syndrome: intratympanic therapy for hearing loss and a review of the literature. Laryngoscope. 2009; 119(1):141-4. DOI: 10.1002/lary.20040. View

Francis H, Makary C, Halpin C, Crane B, Merchant S . Temporal bone findings in a case of Susac's syndrome. Otol Neurotol. 2011; 32(8):1198-204. PMC: 3177404. DOI: 10.1097/MAO.0b013e31822e9665. View

Capiluppi E, Romano L, Luccarelli S, Macerollo A, Cislaghi G . Aggressive immunosuppression in Susac's syndrome: 10 years of follow-up. Neurol Sci. 2018; 39(10):1807-1809. DOI: 10.1007/s10072-018-3481-4. View

Bittencourt A, Santos A, Goffi-Gomez M, Kutscher K, Tsuji R, de Brito R . Microangiopathy of the inner ear, deafness, and cochlear implantation in a patient with Susac syndrome. Acta Otolaryngol. 2011; 131(10):1123-8. DOI: 10.3109/00016489.2011.593549. View

Lavinsky L, Scarton F, Lavinsky-Wolff M, Lavinsky J, Motta L . Successful cochlear implantation in a Susac syndrome patient. Braz J Otorhinolaryngol. 2013; 78(6):123. PMC: 9446366. DOI: 10.5935/1808-8694.20120045. View

10.

Bacciu A, Pasanisi E, Di Lella F, Guida M, Bacciu S, Vincenti V . Cochlear implantation in patients with Cogan syndrome: long-term results. Eur Arch Otorhinolaryngol. 2014; 272(11):3201-7. DOI: 10.1007/s00405-014-3376-9. View

11.

Dorr J, Krautwald S, Wildemann B, Jarius S, Ringelstein M, Duning T . Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol. 2013; 9(6):307-16. DOI: 10.1038/nrneurol.2013.82. View

12.

Roeser M, Driscoll C, Shallop J, Gifford R, Kasperbauer J, Gluth M . Susac syndrome--a report of cochlear implantation and review of otologic manifestations in twenty-three patients. Otol Neurotol. 2008; 30(1):34-40. DOI: 10.1097/mao.0b013e31818b6ac2. View

13.

Greco A, De Virgilio A, Gallo A, Fusconi M, Turchetta R, Tombolini M . Susac's syndrome--pathogenesis, clinical variants and treatment approaches. Autoimmun Rev. 2014; 13(8):814-21. DOI: 10.1016/j.autrev.2014.04.004. View

14.

Ammar M, Kolomeyer A, Bhatt N, Tamhankar M, Mullen M, Brucker A . RECURRENT BRANCH RETINAL ARTERY OCCLUSION FROM SUSAC SYNDROME: CASE REPORT AND REVIEW OF LITERATURE. Retin Cases Brief Rep. 2018; 14(4):315-320. DOI: 10.1097/ICB.0000000000000751. View

15.

Heng L, Bailey C, Lee R, Dick A, Ross A . A review and update on the ophthalmic implications of Susac syndrome. Surv Ophthalmol. 2019; 64(4):477-485. DOI: 10.1016/j.survophthal.2019.01.007. View

16.

Kleffner I, Duning T, Lohmann H, Deppe M, Basel T, Promesberger J . A brief review of Susac syndrome. J Neurol Sci. 2012; 322(1-2):35-40. DOI: 10.1016/j.jns.2012.05.021. View

17.

Rennebohm R, Asdaghi N, Srivastava S, Gertner E . Guidelines for treatment of Susac syndrome - An update. Int J Stroke. 2018; 15(5):484-494. DOI: 10.1177/1747493017751737. View

18.

Grover N, Whiteside O, Ramsden J . Susac syndrome: outcome of unilateral cochlear implantation. J Laryngol Otol. 2011; 125(8):856-8. DOI: 10.1017/S0022215111000600. View

19.

Bateman N, Johnson I, Gibbin K . Susac's syndrome: a rare cause of fluctuating sensorineural hearing loss. J Laryngol Otol. 1998; 111(11):1072-4. DOI: 10.1017/s0022215100139374. View

20.

Patel V, Dunklebarger M, Zacharia T, Isildak H . Otologic manifestations of Susac syndrome. Acta Otorhinolaryngol Ital. 2019; 38(6):544-553. PMC: 6325654. DOI: 10.14639/0392-100X-2166. View