Cochlear implantation in a bilateral sensorineural hearing loss patient ...

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Cochlear implantation in a bilateral sensorineural hearing loss patient with relapsing polychondritis. Authors; Authors and affiliations. Young Joon Seo; Jae ...

Rheumatol Int (2012) 32:479–482 DOI 10.1007/s00296-009-1259-y


Cochlear implantation in a bilateral sensorineural hearing loss patient with relapsing polychondritis Young Joon Seo • Jae Young Choi Si Hong Kim • Tae-Jung Kim

Received: 29 July 2009 / Accepted: 28 November 2009 / Published online: 5 January 2010 Ó Springer-Verlag 2009

Abstract Relapsing polychondritis (RP) is a rare disease which presents chondritis in multiple organs. Characteristic features include auricular chondritis, arthritis, nasal chondritis, ocular inflammation, respiratory tract involvement and audiovestibular damage. Fifty percent of cases of RP are associated with inner ear symptoms such as dizziness and hearing loss. We have recently encountered a case of RP in a 34-year-old man who had recurrent chondritis of both auricles and progressive bilateral profound sensorineural hearing loss; he had been treated many times with steroids, immunosuppressants, plasmaphresis treatments. We perfomed a successful cochlear implant surgery on the left ear of this patient. This raises the possibility of using cochlear implants in treating patients with immune-mediated inner ear disease as well as such RP patients. Keywords Relapsing polychondritis  Cochlear implantation  Sensorineural hearing loss  Immune-mediated inner ear disease (IMIED)

Introduction Since Jaksch-Wartenhorst’s original description in 1923, relapsing polychondritis (RP) has been classified as an autoimmune disease that primarily manifests as cartilaginous tissue destruction [1]. Inner ear disorders appear in 40–50% of patients with RP during the course of the disease [2]. Hearing loss has also been associated with RP, though the pathogenesis of the hearing loss has remained obscure until now. Profound hearing loss is rare in RP [3]. Y. J. Seo  J. Y. Choi (&)  S. H. Kim  T.-J. Kim Yonsei University College of Medicine, Seoul, Korea e-mail: [email protected]

We encountered a patient with RP who presented with relapsing, profound, bilateral, sensorineural hearing loss despite steroid therapy, immunosuppressants, and plasmaphresis. We performed a successful cochlear implantation on the patient. To our knowledge, this is the first report of cochlear implantation in a patient with RP-associated sensorineural hearing loss.

Case report A 34-year-old man referred to our hospital at the end of June 2007 suffered from RP with severe sensorineural hearing loss of both ears. He received the diagnosis of RP based on the presence of auricular chondritis, a saddle nose deformity (Fig. 1), polyarthritis, conjunctivitis, respiratory tract chondritis, perichodral lymphohistiocytic infiltration and fibrosis (Fig. 2), and cochlear-vestibular dysfunction. He had had a deafness in his right ear since birth of unknown etiology with normal hearing in the left ear. He began experiencing relapsing auricular chondritis initially in September 2002 and after that he suffered from polyarthritis in his fingers, knees, and shoulders. In October 2003, he developed true vertigo accompanied with tinnitus followed by sudden onset sensorineural hearing loss in the left ear (Fig. 3a). Because he had a previous deafness of right ear, a hearing loss of left ear made him a big trouble in his life. The patient was unable to regain his hearing despite receiving steroid and immunosuppressant (methotrexate) therapy. Finally, he underwent optional plasmapheresis treatment. After his first plasmapheresis treatment, he recovered 30–40 dB of low frequency hearing on the left side. But it did not seem to work on his right ear (Fig. 3b). He continued steroid and immunosuppressant therapy after completing plasmapheresis. Although his



Fig. 1 a Cauliflower-like auricular chondritis of the right ear; b depressed and deformed dorsum of the nose

general condition improved initially, his hearing loss began to worsen again. His hearing had worsened to 80 dB hearing levels on the left and he finally got complete deafness on the left, too. He was referred to our hospital

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after a year and a half with deafness in both ears (Fig. 5c). Auditory brainstem response (ABR) and distortion product otoacoustic emissions (DPOE) tests showed no response, and he had vestibular dysfunction on vestibular function tests like caloric testing and posturography, among others. Over the course of 3 years, hearing aids in the left ear did not improve his hearing performance. Temporal bone CT showed slight cochlear narrowing, but the endolymphatic space was well preserved on temporal MRI (Fig. 4). Additionally on MRI, there were no vestibulocochlear nerve or ganglion abnormalities. Pre-operative speech evaluation revealed that he had difficulties in recognizing environmental sounds with categories of auditory performance (CAP) score of zero and a monosyllabic word discrimination test score of 40% with lip reading and 0% without it. We decided to do the cochlear implantation on his left side of ear because he had been used to listen with the left ear for so a long time and there was a possibility that the nerve function of his left side could be preserved. A cochlear implantation was performed using a Clarion Hires90k. The device was implanted into the scala tympani

Fig. 2 a Biopsy of ear showing degenerated cartilage with perichondral lymphohistiocytic infiltration and fibrosis (9200); b higher magnification (9400), the cartilage matrix loses basophilic staining, inflammatory cells infiltrate the cartilage, chondrocytes become vacuolated and necrotic, and cartilage is replaced by fibrous tissue

Fig. 3 Pure tone audiogram. a Before treatment for sudden sensorineural hearing loss in 10 October 2003. b After first plasmapheresis in 23 October 2003, he gained 30–40 dB hearing for low frequency in


only left ear. c Progressive hearing loss despite of common treatments such as steroids, immunosuppressants, plasmapheresis in 20 June 2007

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Fig. 4 a, b Slight second turn cochlear(white arrows) narrowing in axial view of temporal bone CT. c, d Preserved endolymphatic space (white arrows) in coronal and axial view of temporal MRI

Fig. 5 a Transorbital ocular view after cochlear implantation shows cochlear implant leads were well inserted (arrow). b Pure tone threshold 3 months after cochlear implantation on 21 January 2008

well. Ten of a total 12 leads of the cochlear implant were well inserted and confirmed by transorbital ocular view (Fig. 5a) and neural response imaging (NRI). There was no post-operative wound dehiscence, flap necrosis, or infection. He had an excellent response at switch-on and discriminated the sounds heard as though they were coming through a microphone kept at a distance. An aided audiogram showed a 40 dB threshold through all frequencies (Fig. 5b). Speech evaluation performed 4 months after cochlear implantation showed a CAP score of 5 and monosyllabic word discrimination test score of 90% with lip reading, 40% without it. He had improvement in the sentence discrimination test with a score of 92% compared to a pre-operative test score of 17%. He has had no problem communicating with anyone and has continued to be trained by language therapists.

Discussion RP is a multisystem disorder and associated with autoimmune diseases such as systemic lupus erythematosus, rheumatoid arthritis, and Sjo¨gren’s syndrome. JakschWartenhorst initially reported RP as a polychondropathy in 1923 [1]. The clinical features include auricular chondritis, arthritis, laryngotracheal symptoms, nasal chondritis, ocular inflammation, audiovestibular symptoms, and cardiovascular symptoms [4]. Audiovestibular symptoms also exist [6]. Hearing loss can be conductive or sensorineural. Conductive hearing loss is due to edema or the collapse of the external auditory canal, or secondary to auricular collapse. There may also be Eustachian tube chondritis, which may cause an effusion. The pathogenesis of sensorineural hearing loss in RP patients is unclear. Miyazawa et al. [7]



performed immunostaining of type II collagen in pathological preparations of the temporal bone in three patients with RP accompanied with sensorineural hearing loss and found specific degeneration of the type II collagen-enriched tectorial membrane, suggesting that this degeneration is involved in the development of hearing loss. Issing et al. [10] reported that anti-labyrinthine antibodies were detected in the serum of an RP patient with audiovestibular dysfunction. These autoantibodies or autoreactive T cells may induce inflammation or apoptotic cell death in the inner ear. Schuknecht [11] noted the absence of cartilage within the inner ear and implicated anobliterative vasculitis of the labyrinthine artery or its branches. This hypothesis is consistent with the known occurrence of systemic necrotizing vasculitis in RP [12]. This case met all of the criteria for RP described by McAdam [5]. This patient also had histologic findings (the cartilage matrix loses basophilic staining, the inflammatory cells infiltrate the cartilage, chondrocytes become vacuolated and necrotic, the cartilage is replaced by fibrous tissue) consistent with an RP diagnosis. Sensorineural hearing loss appears in 40–50% of patients with RP during the course of the disease, but most do not progress to profound hearing loss [3]; therefore, treatments commonly applied to RP patients such as NSAIDs, steroids, or immunosuppressive agents (AZT, cyclophosphamide, etc.) can improve or preserve the hearing of patients with RP [13]. Occasionally, profound hearing loss is caused by progressive RP, which does not to respond to common treatments. In immune-mediated inner ear diseases (IMIED) such as SLE, Cogan’s syndrome, and Buerger’s disease, among others, cochlear implantation has been performed with good results [15]. Several reports have suggested that the pathogenesis of RP could be similar to that of IMIED and that the tectorial membrane could be an important site affected by autoantibodies in RP. Cochlear implantation directly stimulates the spiral ganglion located over the cochlea and can be a very effective treatment for cochlear dysfunction including RP. We report an RP patient with profound hearing loss despite treatment with steroids, immunosuppressive agents, and plasmapheresis who underwent a cochlear implantation with good


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results. Cochlear implantation will remain a good optional treatment for IMIED, including RP with profound hearing loss.

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