J Neurol Surg B Skull Base 2019; 80(S 01): S1-S244
DOI: 10.1055/s-0039-1679713
Poster Presentations
Georg Thieme Verlag KG Stuttgart · New York

Cochlear Implant Failure after Stereotactic Radiosurgery for Vestibular Schwannoma in a Patient with Crest Syndrome

Matthew M. May
1   Department of Otorhinolaryngology, Mayo Clinic, Rochester, Minnesota, United States
,
Michael J. Link
2   Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota, United States
,
Matthew L. Carlson
1   Department of Otorhinolaryngology, Mayo Clinic, Rochester, Minnesota, United States
› Author Affiliations
Further Information

Publication History

Publication Date:
06 February 2019 (online)

 
 

    Introduction: Cochlear implantation can be successfully performed in select patients with vestibular schwannoma (VS) for hearing rehabilitation. CREST syndrome and scleroderma is a connective tissue disorder characterized with inflammatory, fibrotic, and degenerative changes including morphologic changes in small arteries and arterioles. We present an unusual case of delayed facial nerve paralysis and loss of cochlear implant function after stereotactic radiosurgery (SRS) for a vestibular schwannoma in a patient with advanced CREST syndrome.

    Clinical Presentation: A 68-year-old male with a history of CREST syndrome and scleroderma presented for cochlear implant evaluation. The patient had asymmetric bilateral SNHL, left worse than right, and an MRI revealed a left 1.8-cm vestibular schwannoma of the IAC. The tumor volume was 3240 mm3 and treated with marginal dose and maximal dose of 12.5 and 25 Gy, respectively. The patient did well, and he was subsequently implanted one month later on the left side with MED-EL Synchrony Flex 28. Preoperative left-sided CNC was 0% in the best aided condition and 3 months postoperative CNC 60%. Approximately 6 months after SRS, the patient had an abrupt loss of cochlear implant function and partial facial paralysis, House–Brackmann grade 4 function. The patient was treated with oral steroids and device integrity testing suggested no internal device failure. Follow-up MRI revealed good response to SRS with central tumor necrosis. One year later after SRS, the patient has recovered House–Brackmann grade 2 function but continues to not have any auditory percept from the cochlear implant.

    Conclusion: We suspect that seventh and eighth cranial neuropathy in our patient may be due to increased sensitivity to radiotherapy as a result of his underlying microangiopathic disease. Patients with CREST syndrome may have a higher risk of cranial nerve dysfunction after SRS.


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    No conflict of interest has been declared by the author(s).