Otolaryngology Case Reports 3 (2017) 12e14
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Facial nerve stimulation outcomes after cochlear implantation with cochlear-facial dehiscence Christina H. Fang, MD a, Sei Y. Chung, BS b, Leila J. Mady, MD, PhD, MPH c, Nicole Raia, ScD d, Huey-Jen Lee, MD e, Yu-Lan Mary Ying, MD b, f, Robert W. Jyung, MD Associate Professor, Director, Otology and Neurotology b, f, * a
Department of Otolaryngology e Head and Neck Surgery, Albert Einstein College of Medicine, Bronx, NY, United States Department of Otolaryngology e Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, NJ, United States Department of Otolaryngology e Head and Neck Surgery, University of Pittsburgh School of Medicine, Pittsburgh, PA, United States d University Hospital, Department of Otolaryngology e Head and Neck Surgery, Division of Audiology, Newark, NJ, United States e Department of Radiology, Rutgers New Jersey Medical School, Newark, NJ, United States f Center for Skull Base and Pituitary Surgery, Neurological Institute of New Jersey, Rutgers New Jersey Medical School, Newark, NJ, United States b c
a r t i c l e i n f o
a b s t r a c t
Article history: Received 12 April 2017 Accepted 13 April 2017 Available online 20 April 2017
Objectives: To examine the occurrence of facial nerve stimulation (FNS) and outcomes in patients with cochlear-facial dehiscence (CFD) who underwent cochlear implantation (CI). Methods: The medical charts and imaging of three patients with bilateral sensorineural hearing loss (SNHL) who presented for CI evaluation were reviewed. Results: Patient 1 (P1) had a history of Meniere's disease. Patient 2 (P2) had a history of medulloblastoma treated with surgery and chemoradiation. Patient 3 (P3) had a history of progressive SNHL. Audiometry showed moderate-to-severe SNHL in P1, severe-to-profound SNHL in P2, and profound SNHL in P3. All had poor speech discrimination ability. Temporal bone computed tomography (CT) coronal cuts were suspicious for bilateral dehiscence between the superior basal turn of the cochlea and labyrinthine segment of the facial nerve in P2 and P3, with maximum dehiscence lengths of 2.0 mm on the left in P2, and 1.8 mm on the right in P3. A thin bony partition was visualized bilaterally in P1. The left ear of P1, left ear of P2, and right ear of P3 were implanted. Facial nerve stimulation occurred immediately upon activation in P1 and P2, which resolved by decreasing the dynamic range of the offending electrodes. No FNS was observed in P3. Conclusion: Cochlear-facial dehiscence can predispose patients to post-implant FNS. Prior temporal bone irradiation may carry a higher risk of FNS. We recommend scrutiny for CFD in CTs of CI candidates and appropriate risk counseling for FNS if CFD is discovered and more frequent monitoring for FNS by audiology. © 2017 Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/ licenses/by/4.0/).
Keywords: Cochlear implantation Cochlear-facial dehiscence Cochlear dehiscence Otic capsule dehiscence Facial nerve stimulation
Introduction Cochlear-facial dehiscence (CFD) is a recently described otic capsule dehiscence between the basal turn of the cochlea (BTC) and the labyrinthine segment of the facial nerve (LSFN). A well-known complication of cochlear implantation (CI), occurring in 1e14% of cases, is facial nerve stimulation (FNS) [1e5]. FNS is thought to
* Corresponding author. Department of Otolaryngology-Head and Neck Surgery, Rutgers New Jersey Medical School, 90 Bergen St., Suite 8100, Newark, NJ 07103, United States. E-mail address:
[email protected] (R.W. Jyung).
occur due to electrical current shunting through the cochlear bony wall separating the BTC from the LSFN. Bigelow et al. described the first report of FNS following CI in a patient with suspected CFD [6]. We present three CFD patients who underwent CI and discuss their outcomes. Case presentations Case 1 re's disease sustained An 81-year old male with left-sided Menie right-sided sensorineural hearing loss (SNHL) from the sound of an
http://dx.doi.org/10.1016/j.xocr.2017.04.003 2468-5488/© 2017 Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
C.H. Fang et al. / Otolaryngology Case Reports 3 (2017) 12e14
airbag deployment. He had used an in-the-ear hearing aid with limited benefit due to recruitment. He denied other otologic complaints. Pure tone audiometry revealed moderate sloping to moderately-severe right-sided SNHL and severe left-sided SNHL. Monaurally-aided speech discrimination ability was poor (0% and 2% in the right and left, respectively), when assessed using the Hearing In Noise Test in Quiet at 47dBHL. All patients in this series had temporal bone CTs performed on a GE Lightspeed Pro 16 (GE Healthcare, Milwaukee, WI) with 1.25-mm cuts with a 0.625-mm retrograde formats. His CT showed a very thin bony partition between the BTC and the LSFN bilaterally (Fig. 1A and B). A Cochlear Nucleus Freedom device was implanted on the left, without complications. One month later at initial stimulation of the Nucleus 5 speech processor, the implant test revealed all electrodes were in compliance. He was seen for mapping two months postactivation, at which time he reported FNS (eye twitching) on the fourth progressive program (dynamic range of 36 current units), which was determined to be caused by electrodes 9e13. These were disabled and electrodes 9, 11e13 were later re-introduced without FNS by globally decreasing dynamic range and changing the pulse width. Three years post-implantation, he is able to hear within normal limits from 250 to 6000 Hz with excellent open set speech discrimination ability.
Case 2 A 34-year old male with a history of medulloblastoma treated with surgical resection, cisplatin chemotherapy, and radiation, with bilateral progressive SNHL was referred for CI. He had used a right in-the-ear hearing aid. He denied other otologic complaints. Pure tone audiometry revealed a severe sloping to profound right-sided SNHL and a profound left-sided SNHL. Monaurallyaided speech discrimination ability was poor (52% and 0% in the right and left ears, respectively). His CT showed a maximum leftsided dehiscence length of 2.0 mm (Fig. 1C).
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A Cochlear Nucleus Freedom device was implanted on the left, without complications. Intraoperatively, focal dehiscences of the mastoid cortex were noted, and his temporal bone demonstrated diffuse, brittle post-radiation changes. One month later at initial stimulation, the implant test revealed all electrodes were in compliance. FNS occurred immediately during Auto Neural Response Telemetry (Auto-NRT) on electrodes: 1, 3, 6, 8, 10, 13 and 19. The patient was given progressive maps created based on psychophysics threshold levels. Six months post-initial stimulation, the patient experienced FNS (left eye, nostril, upper and lower lip twitching). Offending electrodes 2 and 8e11 were identified and deactivated. Seven months post-activation, his speech discrimination ability was slightly worse (92%e86%), and he also reported increased hearing difficulty. All offending electrodes were successfully re-introduced with smaller dynamic ranges determined by establishing comfort levels based on observed FNS. The patient currently receives excellent benefit from his CI. He can hear within normal limits and has excellent speech discrimination ability.
Case 3 A 59-year old male with severe obstructive sleep apnea was referred for CI evaluation for a long-standing bilateral SNHL. He had been using a left-sided hearing aid with perceived benefit until three months prior to his presentation, he experienced a “popping” sensation and sudden left-sided hearing loss after blowing his nose. He denied other otologic complaints. Audiometry revealed bilateral profound SNHL with no speech discrimination ability in both aided and unaided conditions. His CT showed a maximum right-sided dehiscence length of 1.8 mm (Fig. 1D). A Cochlear Nucleus Freedom device was implanted on the right, without complications. At initial stimulation, the implant test revealed all electrodes were in compliance, and impedances were high, yet flat. To date, he has not experienced FNS. He can hear within normal limits from 250 to 6000Hz, and has excellent open-
Fig. 1. High-resolution temporal bone CTs were performed on a GE Lightspeed Pro 16 (GE Healthcare, Milwaukee, WI) in three cases. (A) In Case 1, review of the CT showed very thin bony partitions between the basal turn of the cochlea and the labyrinthine segment of the facial nerve on the right. (B) Similar findings were seen on the left. (C) In Case 2, coronal views were suspicious for dehiscence between the cochlea and facial nerve and had a maximum length of 2.0 mm on the left side. (D) In Case 3, there was a maximum dehiscence length of 1.8 mm on the right side in the coronal view.
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set sentence discrimination ability in quiet and competing background noise. Discussion The current authors initially described CFD as an entity where patients typically present with autophony, pulsatile tinnitus, and vertigo in addition to SNHL [7]. We subsequently conducted an analysis of over 1000 coronally-sectioned temporal bones and demonstrated the prevalence of CFD to be 0.59% [8]. Our current case series addresses the increased concern for FNS following CI in CFD patients. Studies have shown that electrodes in the upper BTC result in more FNS than those placed more distally [6,9]. Dissection of 8 temporal bones showed that electrodes 8e11 on a Nucleus-22 electrode array were closest to the LSFN [6]. Similarly, Electrodes 9e13 and electrodes 2, 8e11 were responsible for FNS in Case 1 and 2, respectively. FNS is managed by reprogramming the offending electrodes or by disabling the offending electrodes altogether. The current of the offending electrodes can be set below the stimulus level for FNS but above those of auditory stimulatory levels [3,10]. Acceptable resolution of FNS was achieved in Case 1 and 2, who are presently able to hear within normal limits. Similar to CI patients with SSCD, we demonstrated that CFD patients can also undergo CI with excellent outcomes [11]. Conclusion CFD may theoretically increase the risk of FNS following CI. One case in our series did not experience FNS at all. Two cases did experience FNS; however, symptoms were easily controlled by inactivating certain electrodes, providing reassurance that CFD should not deter anyone from performing CI surgery. We recommend careful review of all pre-operative CT scans for the presence of CFD, and identified patients should be counseled about the possible risk of FNS.
Financial disclosures This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. Conflicts of interest None. References [1] Niparko JK, Oviatt DL, Coker NJ, Sutton L, Waltzman SB, Cohen NL. Facial nerve stimulation with cochlear implantation. VA cooperative study group on cochlear implantation. Otolaryngol-Head neck Surg Off J Am Acad Otolaryngol-Head Neck Surg 1991;104:826e30. [2] Kelsall DC, Shallop JK, Brammeier TG, Prenger EC. Facial nerve stimulation after Nucleus 22-channel cochlear implantation. Am J Otol 1997;18:336e41. [3] Cohen NL. Medical or surgical complications related to the Nucleus multichannel cochlear implant. Ann Otol, Rhinol, Laryngol 1989;98:754. [4] Berrettini S, Vito de A, Bruschini L, Passetti S, Forli F. Facial nerve stimulation after cochlear implantation: our experience. Acta otorhinolaryngol Ital Organo uff della Soc ital otorinolaringol chir cerv.-facc 2011;31:11e6. [5] Smullen JL, et al. Facial nerve stimulation after cochlear implantation. Laryngoscope 2005;115:977e82. http://dx.doi.org/10.1097/ 01.MLG.0000163100.37713.C6. [6] Bigelow DC, Kay DJ, Rafter KD, Montes M, Knox GW, Yousem DM. Facial nerve stimulation from cochlear implants. Am J Otol 1998;19:163e9. [7] Blake DM, Tomovic S, Vazquez A, Lee HJ, Jyung RW. Cochlear-facial dehiscenceea newly described entity. Laryngoscope 2014;124:283e9. http:// dx.doi.org/10.1002/lary.24223. [8] Fang CH, et al. Prevalence of cochlear-facial dehiscence in a study of 1,020 temporal bone specimens. Otol Neurotol 2016;37:967e72. http://dx.doi.org/ 10.1097/MAO.0000000000001057. [9] Rotteveel LJ, Proops DW, Ramsden RT, Saeed SR, van Olphen AF, Mylanus EA. Cochlear implantation in 53 patients with otosclerosis: demographics, computed tomographic scanning, surgery, and complications. Otol Neurotol Off Publ Am Otol Soc Am Neurotol Soc Eur Acad Otol Neurotol 2004;25: 943e52. [10] Shea 3rd JJ, Domico EH. Facial nerve stimulation after successful multichannel cochlear implantation. Am J Otol 1994;15:752e6. [11] Puram SV, Roberts DS, Niesten ME, Dilger AE, Lee DJ. Cochlear implant outcomes in patients with superior canal dehiscence. Cochlear Implant Int 2013. http://dx.doi.org/10.1179/1754762813Y.0000000044.