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Fully implantable hearing device as a new treatment of conductive hearing loss in Franceschetti syndrome
International Journal of Pediatric Otorhinolaryngology (2008) 72, 513—517
www.elsevier.com/locate/ijporl
CASE REPORT
Fully implantable hearing device as a new treatment of conductive hearing loss in Franceschetti syndrome ´phane Tringali a,b,*, Nick Pergola c, Chantal Ferber-Viart b,d, Ste Eric Truy b,e, Paul Berger a, Christian Dubreuil a a
Service d’Oto-neurochirurgie, Hospices Civils de Lyon, Centre hospitalier Lyon Sud, ´nite Cedex, France 69495 Pierre-Be b UCB Lyon I UMR 5020, Neurosciences Sensorielles Comportement Cognition, IFR 19, ´de ´ratif des Neurosciences de Lyon, France Institut Fe c Otologics LLC, Boulder, CO, USA d Explorations Audiologiques et Orofaciales, Hospices Civils de Lyon, ´nite, France Centre Hospitalier Lyon-Sud, 69310 Pierre Be e ˆpital Edouart Herriot, Lyon, France Service d’Otologie-Hospices Civils de Lyon, Ho Received 12 September 2007; received in revised form 4 December 2007; accepted 4 December 2007 Available online 7 February 2008
KEYWORDS Conductive hearing loss; Franceschetti; Treacher Collins; Implantable hearing device
Summary The objective is to investigate a new therapeutic option for treatment of conductive hearing loss in children with Franceschetti syndrome. A Carina fully implantable device with the METTM V transducer for conductive applications was implanted in a 14-year-old male suffering from bilateral conductive hearing loss (mean PTA loss: 70 dB on the right ear and 64 dB on the left ear) secondary to Franceschetti (a.k.a Treacher Collins) syndrome with bilateral agenesis of external ear canal and ossicular malformation. Postoperative unaided PTA was unchanged by the surgical procedure. When the implant was activated, mean PTA was 29 dB improved on the implanted ear. The capacity of the Carina METTM ossicular stimulator to provide appropriate gain relative to the degree of hearing loss indicates that the device may be a viable treatment for children with agenesis of external auditory canal and severe malformation of the middle ear. However, these initial results need to be furthered by: (1) additional studies including a greater number of patients to confirm these preliminary results and (2) a long-term follow-up must be carried out to detect possible long-term cochlear adverse effects, in particular on the basilar membrane. # 2008 Published by Elsevier Ireland Ltd.
* Corresponding author at: Service d’Oto-neurochirurgie, Hospices Civils de Lyon, Centre hospitalier Lyon Sud, 69495 Pierre-Be ´nite Cedex, France. Tel.: +33 4 78 86 19 50; fax: +33 4 78 86 33 36. E-mail address: [email protected] (S. Tringali). 0165-5876/$ — see front matter # 2008 Published by Elsevier Ireland Ltd. doi:10.1016/j.ijporl.2007.12.002
514
1. Introduction Conventional hearing aids are subject to many limitations, including occlusion of the ear canal, chronic irritation, acoustic feedback, sound distortion and other limitations [1]. In Franceschetti (a.k.a Treacher Collins) syndrome with bilateral external ear canal agenesis, conventional acoustic hearing aids cannot be used. In this case, a boneanchored hearing aid (BAHA) is one option [2]. However, BAHA implants have the inherent psychological and biological disadvantages of open implants with risks of local inflammation. There is a necessity for constant hygiene in order to avoid inflammation [3,4], and in young active children there is the heightened risk of breaking or extruding the implant. Recently, implantable hearing devices have been developed to overcome some of these problems [1,5—7]. The Symphonix Vibrant Soundbridge was the first device to be used routinely, staring in February 1998 in Europe and August 2000 in United States [6]. The Soundbridge is now being used to treat mixed loss as well [8]. A new fully implantable hearing device has recently been made available by Otologics [7,8]: the Otologics Carina fully implantable system with the Middle Ear Transducer (MET)TM which is an available treatment for moderate to severe sensorineural hearing loss in adults [1,6] and more recently in patients with congenital auricular atresia [7]. This system is CE marked for sale in EU, and is in FDA clinical trials in the US. For the Otologics conductive/mixed loss therapy, the end of the prosthesis can be placed directly on the stapes footplate or directly on the round window if the oval window is completely obliterated by otosclerosis. We report the case of a young male with Franceschetti syndrome including bilateral conductive
S. Tringali et al. hearing loss who underwent a novel middle-ear implantation surgery. The vibratory transducer of the fully implantable system was placed on the stapes footplate, thus bypassing the ossicular chain malformation and external ear canal agenesis.
2. Case report A 14-year-old male with a diagnosis of Franceschetti syndrome presented with a bilateral conductive hearing loss secondary to ossicular malformations and external ear canal agenesis. The patient presented a mean PTA loss of 70 dB on the right ear and 64 dB on the left ear (Fig. 1). On the CT scan, no incus was observed and the stapes was abnormal. The therapy options were proposed to the patient with his parents: unilateral or bilateral BAHA or right middle ear implant. Because the patient was a rowdy boy and risk of BAHA damage was therefore heightened, BAHA was not performed. The Carina fully implantable device (Fig. 2) with the METTM V transducer for conductive applications (Fig. 3) was implanted in the right ear on 15 January 2007. A facial recess approach was used to access the middle ear, incus was absent and there was an abnormal stapes with immobile footplate. The mounting bracket was fixed on the mastoid. A posterior stapedotomy was done. A selected prosthesis attachment, similar to standard titanium prosthesis for stapes footplate placement, was placed into the stapedotomy after attaching it to the transducer (Fig. 4). Postoperative radiograph confirmed the proper location of the various components of the implant (Fig. 5). A hematoma developed in the surgical site postoperatively, requiring minor surgical intervention to clean and evacuate. This follow up included a
Fig. 1 Pre-implantation audiometry : air (AC) and bone (BC) conduction thresholds on the two ears (dB were in hearing level (dB HL)). The right ear was in blue and the left ear in red. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of the article.)
Franceschetti syndrome and implantable hearing device
Fig. 2
515
Carina fully implantable device.
Fig. 3 METTM V transducer with available prostheses which are attached to the tip of the transducer intraoperatively.
10-day course of antibiotics to prevent infection. Following the revision and antibiotics, the postoperative outcome was satisfactory with no further complication. On 13 March 2007, the Carina implant was activated, and the postoperative unaided PTA was found to be unchanged from the preoperative condition (PTA = 73 dB, SRT = 75 dB, SDS = 90%). The internal audio processor was programmed according to the patient’s hearing loss using a fitting algorithm based on implant thresholds and uncom-
Fig. 4 Intraoperative view of the right ear. The transducer was positioned in the posterior part of the oval window (arrow).
Fig. 5 Postoperative radiograph: mounting bracket (1), electronic device (2) and microphone (3).
fortable loudness levels and the desired sensation level targets. The final PTA = 43 dB, the final SRT = 46 dB and the final SDS = 90%. PTA with aid showed a mean PTA improvement of 29 dB (Fig. 6).
3. Discussion In a previous study in 282 patients, the Otologics MET ossicular Stimulator was established to be safe and efficient in moderate to severe sensorineural hearing loss in adults [1]. In these cases, implantation of the electromechanical transducer in contact with the ossicular chain did not result in a change of cochlear status as evidenced by stable postoperative bone conduction thresholds [1,5]. Fully implantable hearing aids need a transcutaneously rechargeable battery combined with a subcutaneous microphone. A new generation of fully implantable hearing aids has been produced by Otologics. It is a progression of their semi-implantable device and is CE marked for sale in the EU and is in US clinical trials. Its principle of sound coupling is the same as in the partially implantable device. The microphone of the fully implantable hearing aid is positioned under the retro auricular skin. So far, both the semi-implantable and fully implantable versions have been constructed and used for patients with normal middle ears and pure sensorineural hearing loss [7]. Recently, this application has been extended to patients with ossicular
516
S. Tringali et al.
Fig. 6 Post-implantation audiometry: free field pure tone thresholds of the right ear. dB were in sound pressure level (SPL). In blue without hearing device and in red with hearing device. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of the article.)
defects [7,8]. This new device must impart its mechanical energy to the cochlea via pathways other than the normal conductive pathway comprising the ossicular chain and oval window, because this pathway has been damaged by the course of middle-ear disease or trauma. Alternative pathways to transmit vibratory mechanical energy to the cochlea include the round window or the stapes footplate after a stapedotomy. This surgical procedure with placement of the tip of transducer into a stapedotomy presents no unusual challenge or difficulty, because most of this procedure is the same as that used for middle-ear implantation of Otologics MET or Symphonix Vibrant Sounbridge [5,6] or for surgical treatment of otosclerosis. As mentioned previously, the active, rambunctious nature of the child prompted his parents to refuse BAHA. Because his hearing loss was bilateral and severe, a fully implantable hearing device was proposed. It was explained to the family that the fully implantable system is a new device which had never been implanted in children and had no longterm follow-up. But as the loss was disabling, they chose this new device.
4. Conclusion Because of the advances in middle ear implant technology, we were able to successfully treat a
conductive hearing loss by directly stimulating the stapes footplate in a child with a Franceschetti syndrome. It may be speculated that children suffering from mild or severe conductive hearing loss could benefit from such a device. However, these results need to be furthered by: (1) additional studies including a greater number of patients to confirm these preliminary results and (2) a long-term follow-up must be carried out to detect possible long-term cochlear adverse effects, in particular on the basilar membrane.
References [1] H.A. Jenkins, J.K. Niparko, W.H. Slattery, J.G. Neely, J.M. Frederickson, Otologics Middle Ear TransducerTM ossicular stimulator: performance results with varying degrees of sensorineural hearing loss, Acta Otolaryngol. 124 (2004) 391—394. [2] W.F. Reuter, C. Marks, S. Jovanovic, M. Gross, Rehabilitation in Franceschetti syndrome: an interdisciplinary approach using bone-anchored hearing aids, Ear Nose Throat J. 76 (402/403) (1997) 411. [3] H. Zeitoun, R. De, S.D. Thomson, D.W. Proops, Difficulties with bone anchored hearing aids in children, J. Laryngol. Otol. 116 (2002) 87—91. [4] M.A. Shirazi, S.J. Marzo, J.P. Leonetti, Perioperative complications with the bone-anchored hearing aid, Otolaryngol. Head Neck Surg. 134 (2006) 236—239. [5] J.F. Kasic, J.M. Frederickson, The Otologics MET ossicular stimulator, Otolaryngol. Clin. North Am. 34 (2001) 501—513. [6] O. Sterkers, D. Boucarra, S. Labassi, J.P. Bebear, C. Dubreuil, B. Frachet, et al., A middle ear implant, the Symphonix Vibrant Soundbridge: retrospective study of
Franceschetti syndrome and implantable hearing device the first 125 patients implanted in France, Otol. Neurotol. 24 (2003) 427—436. [7] R. Siegert, S. Mattheis, J. Kasic, Fully implantable hearing aids in patients with congenital auricular atresia, Laryngoscope 117 (2007) 336—340.
517
[8] F. Venail, J.P. Lavieille, R. Meller, A. Deveze, L. Tardivet, J. Magnan, New perspectives for middle ear implants: first result in otosclerosis with mixed hearing loss, Laryngoscope 117 (2007) 552—555.
Available online at www.sciencedirect.com
www.elsevier.com/locate/ijporl
CASE REPORT
Fully implantable hearing device as a new treatment of conductive hearing loss in Franceschetti syndrome ´phane Tringali a,b,*, Nick Pergola c, Chantal Ferber-Viart b,d, Ste Eric Truy b,e, Paul Berger a, Christian Dubreuil a a
Service d’Oto-neurochirurgie, Hospices Civils de Lyon, Centre hospitalier Lyon Sud, ´nite Cedex, France 69495 Pierre-Be b UCB Lyon I UMR 5020, Neurosciences Sensorielles Comportement Cognition, IFR 19, ´de ´ratif des Neurosciences de Lyon, France Institut Fe c Otologics LLC, Boulder, CO, USA d Explorations Audiologiques et Orofaciales, Hospices Civils de Lyon, ´nite, France Centre Hospitalier Lyon-Sud, 69310 Pierre Be e ˆpital Edouart Herriot, Lyon, France Service d’Otologie-Hospices Civils de Lyon, Ho Received 12 September 2007; received in revised form 4 December 2007; accepted 4 December 2007 Available online 7 February 2008
KEYWORDS Conductive hearing loss; Franceschetti; Treacher Collins; Implantable hearing device
Summary The objective is to investigate a new therapeutic option for treatment of conductive hearing loss in children with Franceschetti syndrome. A Carina fully implantable device with the METTM V transducer for conductive applications was implanted in a 14-year-old male suffering from bilateral conductive hearing loss (mean PTA loss: 70 dB on the right ear and 64 dB on the left ear) secondary to Franceschetti (a.k.a Treacher Collins) syndrome with bilateral agenesis of external ear canal and ossicular malformation. Postoperative unaided PTA was unchanged by the surgical procedure. When the implant was activated, mean PTA was 29 dB improved on the implanted ear. The capacity of the Carina METTM ossicular stimulator to provide appropriate gain relative to the degree of hearing loss indicates that the device may be a viable treatment for children with agenesis of external auditory canal and severe malformation of the middle ear. However, these initial results need to be furthered by: (1) additional studies including a greater number of patients to confirm these preliminary results and (2) a long-term follow-up must be carried out to detect possible long-term cochlear adverse effects, in particular on the basilar membrane. # 2008 Published by Elsevier Ireland Ltd.
* Corresponding author at: Service d’Oto-neurochirurgie, Hospices Civils de Lyon, Centre hospitalier Lyon Sud, 69495 Pierre-Be ´nite Cedex, France. Tel.: +33 4 78 86 19 50; fax: +33 4 78 86 33 36. E-mail address: [email protected] (S. Tringali). 0165-5876/$ — see front matter # 2008 Published by Elsevier Ireland Ltd. doi:10.1016/j.ijporl.2007.12.002
514
1. Introduction Conventional hearing aids are subject to many limitations, including occlusion of the ear canal, chronic irritation, acoustic feedback, sound distortion and other limitations [1]. In Franceschetti (a.k.a Treacher Collins) syndrome with bilateral external ear canal agenesis, conventional acoustic hearing aids cannot be used. In this case, a boneanchored hearing aid (BAHA) is one option [2]. However, BAHA implants have the inherent psychological and biological disadvantages of open implants with risks of local inflammation. There is a necessity for constant hygiene in order to avoid inflammation [3,4], and in young active children there is the heightened risk of breaking or extruding the implant. Recently, implantable hearing devices have been developed to overcome some of these problems [1,5—7]. The Symphonix Vibrant Soundbridge was the first device to be used routinely, staring in February 1998 in Europe and August 2000 in United States [6]. The Soundbridge is now being used to treat mixed loss as well [8]. A new fully implantable hearing device has recently been made available by Otologics [7,8]: the Otologics Carina fully implantable system with the Middle Ear Transducer (MET)TM which is an available treatment for moderate to severe sensorineural hearing loss in adults [1,6] and more recently in patients with congenital auricular atresia [7]. This system is CE marked for sale in EU, and is in FDA clinical trials in the US. For the Otologics conductive/mixed loss therapy, the end of the prosthesis can be placed directly on the stapes footplate or directly on the round window if the oval window is completely obliterated by otosclerosis. We report the case of a young male with Franceschetti syndrome including bilateral conductive
S. Tringali et al. hearing loss who underwent a novel middle-ear implantation surgery. The vibratory transducer of the fully implantable system was placed on the stapes footplate, thus bypassing the ossicular chain malformation and external ear canal agenesis.
2. Case report A 14-year-old male with a diagnosis of Franceschetti syndrome presented with a bilateral conductive hearing loss secondary to ossicular malformations and external ear canal agenesis. The patient presented a mean PTA loss of 70 dB on the right ear and 64 dB on the left ear (Fig. 1). On the CT scan, no incus was observed and the stapes was abnormal. The therapy options were proposed to the patient with his parents: unilateral or bilateral BAHA or right middle ear implant. Because the patient was a rowdy boy and risk of BAHA damage was therefore heightened, BAHA was not performed. The Carina fully implantable device (Fig. 2) with the METTM V transducer for conductive applications (Fig. 3) was implanted in the right ear on 15 January 2007. A facial recess approach was used to access the middle ear, incus was absent and there was an abnormal stapes with immobile footplate. The mounting bracket was fixed on the mastoid. A posterior stapedotomy was done. A selected prosthesis attachment, similar to standard titanium prosthesis for stapes footplate placement, was placed into the stapedotomy after attaching it to the transducer (Fig. 4). Postoperative radiograph confirmed the proper location of the various components of the implant (Fig. 5). A hematoma developed in the surgical site postoperatively, requiring minor surgical intervention to clean and evacuate. This follow up included a
Fig. 1 Pre-implantation audiometry : air (AC) and bone (BC) conduction thresholds on the two ears (dB were in hearing level (dB HL)). The right ear was in blue and the left ear in red. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of the article.)
Franceschetti syndrome and implantable hearing device
Fig. 2
515
Carina fully implantable device.
Fig. 3 METTM V transducer with available prostheses which are attached to the tip of the transducer intraoperatively.
10-day course of antibiotics to prevent infection. Following the revision and antibiotics, the postoperative outcome was satisfactory with no further complication. On 13 March 2007, the Carina implant was activated, and the postoperative unaided PTA was found to be unchanged from the preoperative condition (PTA = 73 dB, SRT = 75 dB, SDS = 90%). The internal audio processor was programmed according to the patient’s hearing loss using a fitting algorithm based on implant thresholds and uncom-
Fig. 4 Intraoperative view of the right ear. The transducer was positioned in the posterior part of the oval window (arrow).
Fig. 5 Postoperative radiograph: mounting bracket (1), electronic device (2) and microphone (3).
fortable loudness levels and the desired sensation level targets. The final PTA = 43 dB, the final SRT = 46 dB and the final SDS = 90%. PTA with aid showed a mean PTA improvement of 29 dB (Fig. 6).
3. Discussion In a previous study in 282 patients, the Otologics MET ossicular Stimulator was established to be safe and efficient in moderate to severe sensorineural hearing loss in adults [1]. In these cases, implantation of the electromechanical transducer in contact with the ossicular chain did not result in a change of cochlear status as evidenced by stable postoperative bone conduction thresholds [1,5]. Fully implantable hearing aids need a transcutaneously rechargeable battery combined with a subcutaneous microphone. A new generation of fully implantable hearing aids has been produced by Otologics. It is a progression of their semi-implantable device and is CE marked for sale in the EU and is in US clinical trials. Its principle of sound coupling is the same as in the partially implantable device. The microphone of the fully implantable hearing aid is positioned under the retro auricular skin. So far, both the semi-implantable and fully implantable versions have been constructed and used for patients with normal middle ears and pure sensorineural hearing loss [7]. Recently, this application has been extended to patients with ossicular
516
S. Tringali et al.
Fig. 6 Post-implantation audiometry: free field pure tone thresholds of the right ear. dB were in sound pressure level (SPL). In blue without hearing device and in red with hearing device. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of the article.)
defects [7,8]. This new device must impart its mechanical energy to the cochlea via pathways other than the normal conductive pathway comprising the ossicular chain and oval window, because this pathway has been damaged by the course of middle-ear disease or trauma. Alternative pathways to transmit vibratory mechanical energy to the cochlea include the round window or the stapes footplate after a stapedotomy. This surgical procedure with placement of the tip of transducer into a stapedotomy presents no unusual challenge or difficulty, because most of this procedure is the same as that used for middle-ear implantation of Otologics MET or Symphonix Vibrant Sounbridge [5,6] or for surgical treatment of otosclerosis. As mentioned previously, the active, rambunctious nature of the child prompted his parents to refuse BAHA. Because his hearing loss was bilateral and severe, a fully implantable hearing device was proposed. It was explained to the family that the fully implantable system is a new device which had never been implanted in children and had no longterm follow-up. But as the loss was disabling, they chose this new device.
4. Conclusion Because of the advances in middle ear implant technology, we were able to successfully treat a
conductive hearing loss by directly stimulating the stapes footplate in a child with a Franceschetti syndrome. It may be speculated that children suffering from mild or severe conductive hearing loss could benefit from such a device. However, these results need to be furthered by: (1) additional studies including a greater number of patients to confirm these preliminary results and (2) a long-term follow-up must be carried out to detect possible long-term cochlear adverse effects, in particular on the basilar membrane.
References [1] H.A. Jenkins, J.K. Niparko, W.H. Slattery, J.G. Neely, J.M. Frederickson, Otologics Middle Ear TransducerTM ossicular stimulator: performance results with varying degrees of sensorineural hearing loss, Acta Otolaryngol. 124 (2004) 391—394. [2] W.F. Reuter, C. Marks, S. Jovanovic, M. Gross, Rehabilitation in Franceschetti syndrome: an interdisciplinary approach using bone-anchored hearing aids, Ear Nose Throat J. 76 (402/403) (1997) 411. [3] H. Zeitoun, R. De, S.D. Thomson, D.W. Proops, Difficulties with bone anchored hearing aids in children, J. Laryngol. Otol. 116 (2002) 87—91. [4] M.A. Shirazi, S.J. Marzo, J.P. Leonetti, Perioperative complications with the bone-anchored hearing aid, Otolaryngol. Head Neck Surg. 134 (2006) 236—239. [5] J.F. Kasic, J.M. Frederickson, The Otologics MET ossicular stimulator, Otolaryngol. Clin. North Am. 34 (2001) 501—513. [6] O. Sterkers, D. Boucarra, S. Labassi, J.P. Bebear, C. Dubreuil, B. Frachet, et al., A middle ear implant, the Symphonix Vibrant Soundbridge: retrospective study of
Franceschetti syndrome and implantable hearing device the first 125 patients implanted in France, Otol. Neurotol. 24 (2003) 427—436. [7] R. Siegert, S. Mattheis, J. Kasic, Fully implantable hearing aids in patients with congenital auricular atresia, Laryngoscope 117 (2007) 336—340.
517
[8] F. Venail, J.P. Lavieille, R. Meller, A. Deveze, L. Tardivet, J. Magnan, New perspectives for middle ear implants: first result in otosclerosis with mixed hearing loss, Laryngoscope 117 (2007) 552—555.
Available online at www.sciencedirect.com