Susac syndrome - A report of cochlear implantation and review of otologic manifestations in twenty-three patients

Michelle M. Roeser, Colin L W Driscoll, Jon K. Shallop, Rene H. Gifford, Jan Kasperbauer, Michael B. Gluth

Research output: Contribution to journalArticle

29 Citations (Scopus)

Abstract

OBJECTIVE: Susac syndrome is a disease condition of unknown cause consisting of vestibulocochlear dysfunction, retinopathy, and multifocal encephalopathy derived from microangiopathy of the ear, retina, and brain, respectively. We present a unique case of bilateral cochlear implantation in a Susac syndrome patient and seek to describe in detail the specific nature of the otologic manifestations of this disease. STUDY DESIGN: Clinical records of 23 patients diagnosed with Susac syndrome were reviewed. Analysis included demographics, clinical course, and audiometric data. An additional review of relevant vestibulocochlear data is undertaken among the approximately 100 previously reported cases. RESULTS: Of the 23 patients with Susac syndrome, 19 (83%) were women. Mean age was 36 years, ranging from 19 to 69 years. Ten patients (43.5%) reported a fluctuating hearing loss, 14 (61%) reported tinnitus, and 13 (56.5%) noted vertigo. Eleven patients (48%) presented with bilateral symptoms, and 12 (52%) were unilateral. In the 34 affected ears, the pure-tone average was 41.5 dB, and the mean percent hearing loss was 26.4%. Forty-seven percent of the affected ears had American Academy of Otolaryngology-Head and Neck Surgery hearing classification type A. Only 26.5% of the affected ears had 100% word recognition. Statistical analysis supported an overall "upsloping" pattern of hearing loss. Bilateral simultaneous cochlear implantation was successful in restoring significant hearing in our patient. CONCLUSION: Susac syndrome is a rare and potentially devastating disease. Hearing loss is quite variable. Low- and mid-range frequencies seem to be most commonly affected. Patients whose hearing loss meets criteria should be considered for cochlear implantation.

Original languageEnglish (US)
Pages (from-to)34-40
Number of pages7
JournalOtology and Neurotology
Volume30
Issue number1
DOIs
StatePublished - Jan 1 2009

Fingerprint

Susac Syndrome
Cochlear Implantation
Hearing Loss
Ear
Hearing
Ear Diseases
Tinnitus
Vertigo
Otolaryngology
Brain Diseases
Retina
Neck
Head
Demography

Keywords

  • Cochlear implant
  • Sensorineural hearing loss
  • Susac syndrome

ASJC Scopus subject areas

  • Otorhinolaryngology
  • Sensory Systems
  • Clinical Neurology

Cite this

Susac syndrome - A report of cochlear implantation and review of otologic manifestations in twenty-three patients. / Roeser, Michelle M.; Driscoll, Colin L W; Shallop, Jon K.; Gifford, Rene H.; Kasperbauer, Jan; Gluth, Michael B.

In: Otology and Neurotology, Vol. 30, No. 1, 01.01.2009, p. 34-40.

Research output: Contribution to journalArticle

Roeser, Michelle M. ; Driscoll, Colin L W ; Shallop, Jon K. ; Gifford, Rene H. ; Kasperbauer, Jan ; Gluth, Michael B. / Susac syndrome - A report of cochlear implantation and review of otologic manifestations in twenty-three patients. In: Otology and Neurotology. 2009 ; Vol. 30, No. 1. pp. 34-40.
@article{9c823c0e6ed64350b7f8b6182bd0a586,
title = "Susac syndrome - A report of cochlear implantation and review of otologic manifestations in twenty-three patients",
abstract = "OBJECTIVE: Susac syndrome is a disease condition of unknown cause consisting of vestibulocochlear dysfunction, retinopathy, and multifocal encephalopathy derived from microangiopathy of the ear, retina, and brain, respectively. We present a unique case of bilateral cochlear implantation in a Susac syndrome patient and seek to describe in detail the specific nature of the otologic manifestations of this disease. STUDY DESIGN: Clinical records of 23 patients diagnosed with Susac syndrome were reviewed. Analysis included demographics, clinical course, and audiometric data. An additional review of relevant vestibulocochlear data is undertaken among the approximately 100 previously reported cases. RESULTS: Of the 23 patients with Susac syndrome, 19 (83{\%}) were women. Mean age was 36 years, ranging from 19 to 69 years. Ten patients (43.5{\%}) reported a fluctuating hearing loss, 14 (61{\%}) reported tinnitus, and 13 (56.5{\%}) noted vertigo. Eleven patients (48{\%}) presented with bilateral symptoms, and 12 (52{\%}) were unilateral. In the 34 affected ears, the pure-tone average was 41.5 dB, and the mean percent hearing loss was 26.4{\%}. Forty-seven percent of the affected ears had American Academy of Otolaryngology-Head and Neck Surgery hearing classification type A. Only 26.5{\%} of the affected ears had 100{\%} word recognition. Statistical analysis supported an overall {"}upsloping{"} pattern of hearing loss. Bilateral simultaneous cochlear implantation was successful in restoring significant hearing in our patient. CONCLUSION: Susac syndrome is a rare and potentially devastating disease. Hearing loss is quite variable. Low- and mid-range frequencies seem to be most commonly affected. Patients whose hearing loss meets criteria should be considered for cochlear implantation.",
keywords = "Cochlear implant, Sensorineural hearing loss, Susac syndrome",
author = "Roeser, {Michelle M.} and Driscoll, {Colin L W} and Shallop, {Jon K.} and Gifford, {Rene H.} and Jan Kasperbauer and Gluth, {Michael B.}",
year = "2009",
month = "1",
day = "1",
doi = "10.1097/MAO.0b013e31818b6ac2",
language = "English (US)",
volume = "30",
pages = "34--40",
journal = "Otology and Neurotology",
issn = "1531-7129",
publisher = "Lippincott Williams and Wilkins",
number = "1",

}

TY - JOUR

T1 - Susac syndrome - A report of cochlear implantation and review of otologic manifestations in twenty-three patients

AU - Roeser, Michelle M.

AU - Driscoll, Colin L W

AU - Shallop, Jon K.

AU - Gifford, Rene H.

AU - Kasperbauer, Jan

AU - Gluth, Michael B.

PY - 2009/1/1

Y1 - 2009/1/1

N2 - OBJECTIVE: Susac syndrome is a disease condition of unknown cause consisting of vestibulocochlear dysfunction, retinopathy, and multifocal encephalopathy derived from microangiopathy of the ear, retina, and brain, respectively. We present a unique case of bilateral cochlear implantation in a Susac syndrome patient and seek to describe in detail the specific nature of the otologic manifestations of this disease. STUDY DESIGN: Clinical records of 23 patients diagnosed with Susac syndrome were reviewed. Analysis included demographics, clinical course, and audiometric data. An additional review of relevant vestibulocochlear data is undertaken among the approximately 100 previously reported cases. RESULTS: Of the 23 patients with Susac syndrome, 19 (83%) were women. Mean age was 36 years, ranging from 19 to 69 years. Ten patients (43.5%) reported a fluctuating hearing loss, 14 (61%) reported tinnitus, and 13 (56.5%) noted vertigo. Eleven patients (48%) presented with bilateral symptoms, and 12 (52%) were unilateral. In the 34 affected ears, the pure-tone average was 41.5 dB, and the mean percent hearing loss was 26.4%. Forty-seven percent of the affected ears had American Academy of Otolaryngology-Head and Neck Surgery hearing classification type A. Only 26.5% of the affected ears had 100% word recognition. Statistical analysis supported an overall "upsloping" pattern of hearing loss. Bilateral simultaneous cochlear implantation was successful in restoring significant hearing in our patient. CONCLUSION: Susac syndrome is a rare and potentially devastating disease. Hearing loss is quite variable. Low- and mid-range frequencies seem to be most commonly affected. Patients whose hearing loss meets criteria should be considered for cochlear implantation.

AB - OBJECTIVE: Susac syndrome is a disease condition of unknown cause consisting of vestibulocochlear dysfunction, retinopathy, and multifocal encephalopathy derived from microangiopathy of the ear, retina, and brain, respectively. We present a unique case of bilateral cochlear implantation in a Susac syndrome patient and seek to describe in detail the specific nature of the otologic manifestations of this disease. STUDY DESIGN: Clinical records of 23 patients diagnosed with Susac syndrome were reviewed. Analysis included demographics, clinical course, and audiometric data. An additional review of relevant vestibulocochlear data is undertaken among the approximately 100 previously reported cases. RESULTS: Of the 23 patients with Susac syndrome, 19 (83%) were women. Mean age was 36 years, ranging from 19 to 69 years. Ten patients (43.5%) reported a fluctuating hearing loss, 14 (61%) reported tinnitus, and 13 (56.5%) noted vertigo. Eleven patients (48%) presented with bilateral symptoms, and 12 (52%) were unilateral. In the 34 affected ears, the pure-tone average was 41.5 dB, and the mean percent hearing loss was 26.4%. Forty-seven percent of the affected ears had American Academy of Otolaryngology-Head and Neck Surgery hearing classification type A. Only 26.5% of the affected ears had 100% word recognition. Statistical analysis supported an overall "upsloping" pattern of hearing loss. Bilateral simultaneous cochlear implantation was successful in restoring significant hearing in our patient. CONCLUSION: Susac syndrome is a rare and potentially devastating disease. Hearing loss is quite variable. Low- and mid-range frequencies seem to be most commonly affected. Patients whose hearing loss meets criteria should be considered for cochlear implantation.

KW - Cochlear implant

KW - Sensorineural hearing loss

KW - Susac syndrome

UR - http://www.scopus.com/inward/record.url?scp=59849094810&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=59849094810&partnerID=8YFLogxK

U2 - 10.1097/MAO.0b013e31818b6ac2

DO - 10.1097/MAO.0b013e31818b6ac2

M3 - Article

C2 - 19108037

AN - SCOPUS:59849094810

VL - 30

SP - 34

EP - 40

JO - Otology and Neurotology

JF - Otology and Neurotology

SN - 1531-7129

IS - 1

ER -