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Year : 2017  |  Volume : 23  |  Issue : 3  |  Page : 197-199

Simultaneous labyrinthectomy and cochlear implantation for a case of otosclerosis with intractable vertigo

1 Department of Otology, Neurotology and Implant Otology, Christian Medical College, Vellore, Tamil Nadu, India
2 Department of ENT, Christian Medical College and Hospital, Vellore, Tamil Nadu, India

Date of Web Publication31-Aug-2017

Correspondence Address:
Joyce Pascal Rozario
Mystical Rose, House Number 5-297A6, V. P. Nagar, Kunjibettu Post, Udupi - 576 102, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/indianjotol.INDIANJOTOL_84_16

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Stapedotomy, though a common surgical procedure for otosclerosis, if inadequately performed, can cause dreaded complications of vertigo and profound hearing loss. Labyrinthectomy with cochlear implantation can be considered in cases of intractable vertigo, as numerous reports have shown that the cochlea still remains responsive to electrical stimulation postlabyrinthectomy. This report presents a case of otosclerosis, with severe to profound deafness and intractable vertigo poststapedotomy surgery, which was treated with simultaneous labyrinthectomy and cochlear implantation. This patient had good control of vertigo postoperatively and the Dizziness Handicap Index score pre- and post-operative were 80 and 38, respectively, with significant improvement in speech perception.

Keywords: Cochlear implantation, labyrinthectomy, simultaneous labyrinthectomy and cochlear implantation, stapedotomy

How to cite this article:
Rozario JP, Lepcha A, Mathew J. Simultaneous labyrinthectomy and cochlear implantation for a case of otosclerosis with intractable vertigo. Indian J Otol 2017;23:197-9

How to cite this URL:
Rozario JP, Lepcha A, Mathew J. Simultaneous labyrinthectomy and cochlear implantation for a case of otosclerosis with intractable vertigo. Indian J Otol [serial online] 2017 [cited 2021 Jul 30];23:197-9. Available from: https://www.indianjotol.org/text.asp?2017/23/3/197/213876

  Introduction Top

Vertigo poststapedotomy provides a challenge with respect to treatment options. For patients who develop disabling attacks of vertigo, a range of management options exists including vestibular sedatives, vestibular exercises, intratympanic steroids, intratympanic gentamicin therapy, endolymphatic sac decompression, vestibular nerve section, and labyrinthectomy.[1] Controversy remains over the optimal treatment and timing of intervention, when there is deterioration of hearing and vestibular function. For patient's refractory to conservative measures, without any useful hearing, labyrinthectomy appears to be a good treatment option to eliminate attacks of vertigo, provided the contralateral ear is serviceable.[1]

We present a case of failed stapedotomy who developed severe hearing loss, intractable vertigo, and instability; not responding to adequate conservative management. He was subsequently treated with labyrinthectomy and cochlear implantation with amelioration of symptoms.

  Case Report Top

A middle-aged college teacher presented with complaints of right-sided hearing loss and vertigo, a month after undergoing stapedotomy surgery at a local hospital. Clinical examination revealed a right-sided posterior moderate sized marginal perforation and dislocated stapes piston and bilateral conductive hearing loss. Pure tone audiogram showed a right moderate to severe mixed hearing loss and a left moderate mixed hearing loss. A high-resolution computed tomography (HRCT) of the temporal bone ruled out any other abnormality of the inner ear. A provisional diagnosis of prosthesis dislocation and tympanic membrane perforation of the right ear was made [Figure 1]a.
Figure 1: (a) Preoperative high-resolution computerized tomogram temporal bone showed anteriorly placed sigmoid sinus, well-pneumatized mastoids. (b) Magnetic resonance imaging T2-weighted images showing adequate inner ear fluid density, adequate nerve complexes, and no features suggestive of labyrinthitis ossificans

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Intraoperatively dislocated 4.2 mm teflon stapes piston and necrosed lenticular process of the incus with active perilymph leak at oval window was noted. This was replaced with a 3.5 mm teflon piston, vein graft and fascia seal at stapedotomy. Temporalis fascia placed by underlay technique to close the tympanic membrane perforation. He had an uneventful postoperative period with substantial improvement in hearing and relief of vertigo.

However, 3 weeks postoperative, he presented to our emergency department with high-grade fever, severe vertigo and decreasing hearing in the right ear. The right neotympanum was healed and Grade I nystagmus was noted. The facial nerve was intact bilaterally and neurological examination ruled out evidence of any other cranial nerve dysfunction, meningitis, or cerebellar dysfunction. A clinical diagnosis of right viral labyrinthitis was made and he was hospitalized and treated with intravenous antibiotics, steroids, and vestibular sedatives. Although he symptomatically improved with decreased episodes of vertigo, he developed right profound hearing loss. Auditory amplification provided limited benefit in the left ear with no significant amplification in the right ear. The patient continued to use the hearing aid with adequate auditory verbal therapy. However he presented with severe imbalance and episodes of right tinnitus and vertigo, despite of continued conservative treatment. Electronystagmography revealed right labyrinthine dysfunction and Dix Hallpike positional test was negative. In view of his debilitating vertigo restricting his daily activities including attending work regularly, a course of Intratympanic Gentamicin was administered, which provided no relief. The option of simultaneous labyrinthectomy and implantation was weighed against vestibular nerve section. Preoperative magnetic resonance imaging-HRCT temporal bone ruled out labyrinthitis ossificans [Figure 1]b.

Right simultaneous labyrinthectomy and cochlear implantation was carried out 7 months after the second stapedotomy surgery, using a postauricular approach. A standard cortical mastoidectomy, posterior tympanotomy, and facial recess approach were used. Labyrinthectomy followed by cochlear implantation was done. By extraluminally compressing the anteriorly placed skeletonized sigmoid sinus, the three semicircular canals were delineated and drilled towards their respective ampullary ends into the vestibule, and reactive bone seen in the right vestibule was sent for histopathological examination. Sensory neuroepithelium in the vestibule was excised thereby completing the preganglionic deafferentation labyrinthectomy, and the site was sealed with bone wax and surgiwool.

Standard steps for cochlear implantation then followed; by drilling of well for receiver stimulator. Advanced Bionics HR 90 K advantage cochlear implant HI focus 1 J (CI-1500-01) was placed in implant bed and secured with tie down sutures. A standard cochleostomy was used to insert 16 electrode array atraumatically into scala tympani of basal turn over an insertion time of 45 seconds. Intraoperative impedance and neural response imaging were within normal limits for all electrodes. Layered closure of the wound was done. Postoperative Modified Stenver's view confirmed intracochlear position of electrode. Patient had a smooth postoperative recovery. He was gradually mobilized and discharged with instructions to continue vestibular rehabilitation exercises. Histopathology specimen from vestibule revealed otospongiotic bone [Figure 2]a.
Figure 2: (a) Postoperative: Modified Stenver's view showing intracochlear position of electrode arrays with adequate insertion depth. (b) Postoperative aided audiogram within speech spectrum

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Postoperatively, the patient had symptomatic relief from vertigo and is undergoing regular auditory training for rehabilitation. This patient had good control of vertigo postoperatively, and the Dizziness Handicap Index scores for preoperative and 3 month's postoperative were 80 and 38, respectively. His hearing levels improved with cochlear implant-aided audiogram within speech spectrum, speech discrimination score is 70%, after masking contralateral normal ear, and question and answer score was 100%. He is able to conduct his regular daily and professional activities normally. At 1-year follow-up, the patient is using his implant on the right ear and hearing aid on the left side. He does not have any rotatory vertigo or significant imbalance [Figure 2]b.

  Discussion Top

Dr. Derald Brackmann has the credit of having performed the first cochlear implant in a previously labyrinthectomized patient as early as 1952.[2]

Although there have been initial doubts that otic capsule disruption may preclude successful implantation; evidence exists to show that spiral ganglion cells and the neural element required for successful cochlear implantation survive following surgical labyrinthectomy. Histological analysis on four temporal bones of patients showed preservation of ganglion cells after labyrinthectomy as reported by Chen et al.[3]

Lambert et al. reported similar findings in a study of six postlabyrinthectomy patients, who had successful transtympanic promontory stimulation of the cochlea.[4] These findings are further supported by a study by Ramsden and Timms, who stimulated the cochlea and measured auditory perception threshold, discomfort level, and dynamic ranges for 10 postlabyrinthectomy patients.[5]

Kveton et al. in 1989, have reported performing cochlear implantation 6 weeks following a labyrinthectomy as part of a temporal bone resection for malignancy. The initial postimplantation results were comparable to three other postlingual deafened implant patients, inspite of surgical manipulation of labyrinth.[6]

As early as 1993, Zwolan et al. reported the first simultaneous surgical osseous labyrinthectomy with cochlear implantation on a 47-year-old patient, who was congenitally profoundly deaf with symptoms of Meniere's disease. Although postoperatively she had cessation of vertigo, initial stimulation of the implant caused discomfort. It however resolved after 4 months and she noticed improved sound awareness and speech recognition.[7]

Facer et al. have concluded similar reports of a left cochlear implantation in a patient with profound deafness secondary to ototoxicity, who had previously undergone a left mastoidectomy, labyrinthectomy, and facial nerve decompression with negative electrical auditory brainstem response on the right ear. Unfortunately, facial twitching due to stimulation of several electrodes resulted in incomplete electrode usage.[8]

Good results were obtained by Zanetti et al. when a simultaneous translabyrinthine removal of an acoustic neuroma was performed with cochlear implantation.[9]

Thedinger et al. reported electrical transtympanic promontory stimulation and successful rehabilitation of a patient, 15 years after labyrinthectomy for endolymphatic hydrops, who developed a contralateral large acoustic neuroma.[10] A cochlear implant surgery was performed with a Nucleus 22 channel device followed by successful removal of the contralateral acoustic neuroma, with a good hearing outcome.

Cochlear implantation in advanced otosclerosis is a recent concept. Excellent hearing benefit has been reported by Ruckenstein et al. on patients with advanced otosclerosis who underwent cochlear implantation.[11]

Successful outcomes after cochlear implantation can be explained by the physiopathology of the otosclerosis, which first affects the lateral wall of the cochlea, resulting in degeneration of the spiral ligament and stria vascularis, and secondarily the organ of Corti.[11]

In our patient, the decision to perform cochlear implantation with labyrinthectomy was taken after several stages of more conservative management had failed to provide relief. Vestibular nerve section for vertigo was another option considered for our patient, but in view of increased risks associated with an intracranial procedure, and the risk of damage to the cochlear nerve precluding subsequent cochlear implantation, the option of labyrinthectomy was preferred.[2]

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Conflicts of interest

There are no conflicts of interest.

  References Top

Declau F, van Spaendonck M, Timmermans JP, Michaels L, Liang J, Qiu JP, et al. Prevalence of histologic otosclerosis: An unbiased temporal bone study in Caucasians. Adv Otorhinolaryngol 2007;65:6-16.  Back to cited text no. 1
MacKeith SA, Bottrill LD, Ramsden JD. Simultaneous labyrinthectomy with cochlear implantation in patients with bilateral Ménière's disease. Ann Otol Rhinol Laryngol 2014;123:485-9.  Back to cited text no. 2
Chen DA, Linthicum FH Jr., Rizer FM. Cochlear histopathology in the labyrinthectomized ear: Implications for cochlear implantation. Laryngoscope 1988;98:1170-2.  Back to cited text no. 3
Lambert PR, Ruth RA, Thomas JF. Promontory electrical stimulation in postoperative acoustic tumor patients. Laryngoscope 1992;102:814-9.  Back to cited text no. 4
Ramsden RT, Timms MS. Promontory stimulation following labyrinthectomy. J Laryngol Otol 1991;105:729-31.  Back to cited text no. 5
Kveton JF, Abbott JF, April JF, Drumheller JF, Cohen JF, Poe JF. Cochlear Implantation after trans mastoid labyrinthectomy. The Laryngoscope 1989;99:610-3.  Back to cited text no. 6
Zwolan TA, Shepard NT, Niparko JK. Labyrinthectomy with cochlear implantation. Am J Otol 1993;14:220-3.  Back to cited text no. 7
Facer GW, Facer ML, Fowler CM, Brey RH, Peterson AM. Cochlear implantation after labyrinthectomy. Am J Otol 2000;21:336-40.  Back to cited text no. 8
Zanetti D, Campovecchi CB, Pasini S, Nassif N. Simultaneous translabyrinthine removal of acoustic neuroma and cochlear implantation. Auris Nasus Larynx 2008;35:562-8.  Back to cited text no. 9
Thedinger BA, Cueva RA, Glasscock ME 3rd. Treatment of an acoustic neuroma in an only-hearing ear: Case reports and considerations for the future. Laryngoscope 1993;103:976-80.  Back to cited text no. 10
Ruckenstein MJ, Rafter KO, Montes M, Bigelow DC. Management of far advanced otosclerosis in the era of cochlear implantation. Otol Neurotol 2001;22:471-4.  Back to cited text no. 11


  [Figure 1], [Figure 2]


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