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Eur Arch OtorhinolaryngolDOI 10.1007/s00405-014-2907-8

CAsE REpORt

Hearing rehabilitation with single‑stage bilateral vibroplasty in a child with Franceschetti syndrome

Sona Sargsyan · Torsten Rahne · Sabrina Kösling · Gerburg Eichler · Stefan K. Plontke

Received: 1 October 2013 / Accepted: 21 January 2014 © springer-Verlag Berlin Heidelberg 2014

with Franceschetti syndrome (mandibulofacial dysostosis). Ct revealed bilateral middle-ear malformations as well as an atretic right and stenotic left external auditory canal. Due to craniofacial dysmorphia airway and (post)operative, management is significantly more difficult in patients with a Franceschetti syndrome which in this case favoured a sin-gle-stage bilateral procedure. No intra- or postoperative sur-gical complications were reported. the middle-ear implants were activated 4 weeks after surgery. In the audiological examination 6 months after surgery, the child showed 100 % speech intelligibility with activated implants on each side.

Keywords Child · Vibrant soundbridge · Vibroplasty · Bilateral · Mixed hearing loss · Franceschetti syndrome · Mandibulofacial dysostosis · treacher Collins syndrome

AbbreviationsAC Air conductionAMEI Active middle-ear implantsBaha Bone-anchored hearing aidsBC Bone conductiondB DecibelFMt Floating mass transducerkHz KilohertzMRI Magnetic resonance imagingVsB Vibrant soundbridgepORp partial ossicular replacement prosthesis

Introduction

treacher Collins syndrome, also known as treacher Col-lins Franceschetti syndrome or mandibulofacial dysostosis, is an autosomal dominant disorder of craniofacial morpho-genesis which affects 1 in 25,000–50,000 newborns. At

Abstract Hearing is of utmost importance for normal speech and social development. Even children who have mild or unilateral permanent hearing loss may experience difficulties with understanding speech, as well as prob-lems with educational and psycho-social development. the increasing advantages of middle-ear implant technologies are opening new perspectives for restoring hearing. Active middle-ear implants can be used in children and adoles-cents with hearing loss. In addition to the well-documented results for improving speech intelligibility and quality of hearing in sensorineural hearing loss active middle-ear implants are now successfully used in patients with con-ductive and mixed hearing loss. In this article we present a case of successful, single-stage vibroplasty, on the right side with the fixation of the FMt on the stapes and pORp CLip vibroplasty on the left side in a 6-year-old girl with bilateral mixed hearing loss and multiple dyslalia associated

s. sargsyan Department of Otorhinolaryngology, Yerevan state Medical University, Yerevan, Armenia

s. sargsyan · t. Rahne · s. K. plontke (*) Department of Otorhinolaryngology, Head and Neck surgery, University of Halle-Wittenberg, Ernst-Grube-str. 40, 06120 Halle (saale), Germanye-mail: stefan.plontke@uk-halle.de

t. Rahnee-mail: torsten.rahne@uk-halle.de

s. Kösling Department of Diagnostic Radiology, University of Halle-Wittenberg, Halle (saale), Germany

G. Eichler Department of Anesthesiology and Intensive Care Medicine, University of Halle-Wittenberg, Halle (saale), Germany

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least 30–50 % of children with this syndrome have bilat-eral, severe, conductive hearing loss due to bilateral exter-nal auditory meatus stenosis or atresia and middle-ear mal-formation [1, 2].

Active middle-ear implants (AMEI) are a new devel-opment in aural atresia surgery. the Vibrant soundbridge (VsB, Vibrant Med-El, Innsbruck, Austria) is a partially implantable hearing system, consisting of an external micro-phone, a sound processor, amplifier, and a floating mass transducer (FMt) as the inner part [3]. the VsB is based on receiving sound with the microphone located behind the ear, processing and amplifying sound by a processor, and transmitting energy and the information inductively to the implant. the combination of tympanoplasty procedures with the implantation of a VsB has been termed “vibroplasty”.

the VsB was originally approved for adults above 18 years of age with moderate to severe sensorineural hearing loss and the FMt was to be clipped onto the long process of the incus. In 2006, Colletti et al. [4] placed the FMt directly onto the round window for seven patients with mixed hearing loss and obtained good audiometric results. the indications were extended to mixed or con-ductive deafness in the adult and to children [5]. In cases of congenital middle and outer ear deformities, a reason-able size middle cavity and a reasonably developed stapes suprastructure, an oval window with mobile footplate or round window is usually required for coupling the FMt to the inner ear via deformed ossicles, the stapes footplate, or the round window membrane [6].

Active middle-ear implants are a relatively new technol-ogy and due to information from the manufacturer only few children worldwide have been fitted with them.

We report for the first time the results and assess the short time safety and efficiency of a bilateral, simultane-ous (one-stage) vibroplasty in a 6-year-old child with con-genital mixed hearing loss associated with Franceschetti syndrome.

Case report

A 6-year-old girl was admitted to the hospital for surgical treatment with the diagnosis of Franceschetti syndrome (mandibulofacial dysostosis, treacher Collins syndrome) associated with bilateral ear malformation, meatal steno-sis, moderate to severe hearing loss, and multiple dyslalia. Electrophysiological thresholds measured with ABR were 70 dB nHL on both sides. pure-tone audiometry in free field done at the age of 1 year showed 55–75 dB HL thresh-olds on the right side and 40–50 dB HL on the left side at 0.5–4 kHz. the audiogram at the age of 3 years showed BC thresholds ≤10 dB HL and AC thresholds of 45–65 dB HL on both sides.

At the age of 4 years, AC thresholds on both sides were 50–70 dB at 0.5–2 kHz and 90–100 dB at 4 kHz. the child had been fitted with bilateral softband Baha® bone conduction devices (Cochlear Corp., Australia) for sev-eral years. speech audiometry (Mainzer audiometric test for children) at the age of 4 years showed 0 % word rec-ognition score without hearing aids, 80 % word recogni-tion score on each side with monaural fitted Baha® and 90 % with bilateral use of BAHA™. the patient and the parents repeatedly asked for an alternative solution to the softband Baha®.

Clinical examination revealed hypoplasia of the facial bones with bilaterally symmetric convex facial profile along with a characteristic downward slant of the eyes, lower eye-lid abnormalities, including sparse, partially absent lashes, and external ear abnormalities including severe stenosis on the left and atresia on the right external auditory canal.

High-resolution computed tomography (Ct) of the tem-poral bones (Figs. 1, 2) showed a bilateral reduced pneu-matisation including hypoplastic tympanic cavities and

Fig. 1 temporal bone Ct (a, b axial, c coronal image) shows bilater-ally a reduced pneumatisation, small tympanic cavity, and dysplasia of the malleus and incus. On the left the tympanic cavity is opacified and the external auditory meatus is stenotic (arrows). Bony atresia (stars) of the right external auditory meatus

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almost completely absent mastoid air cells. the right tym-panic cavity was well ventilated and the left one opacified. Bilaterally, the round and the oval window niches could be identified; the malleus and incus appeared dysplastic. the left stapes was difficult to distinguish within the opacifica-tion; the right one seemed to be hypoplastic. No variants of the facial nerves’ courses were found. there was a stenosis of the left external auditory canal filled with fibrotic tissue and a bony atresia on the right side. the inner ear was nor-mal. the changes correspond to a combined middle–exter-nal ear malformation.

MRI of the temporal bone showed a normal finding of the inner ear, internal auditory canal, and cerebellopontine angle including the 7th and 8th cranial nerves.

Surgery

Because of the increased risk of multiple intubations due to facial hypoplasia, ear surgery was preceded by the

necessary dental treatment for caries in the same session. After opening the right middle-ear space the dysplas-tic malleus appeared fixed and the mobility of the incus appeared reduced. the stapes showed a hypoplastic ante-rior crus, but its mobility was normal. the round window niche could not be sufficiently overseen. the middle-ear space did not appear large enough to connect the FMt to the stapes via a Bell or CLip coupler. therefore, the FMt was crimped directly to the stapes head, once the dysplastic malleus and incus with limited mobility were removed (Fig. 3R). Due to the existing indication for surgery on the contralateral side and the expected chal-lenges in intubation and airway management, a possible single-stage bilateral ear surgery was discussed with the parents already before the operation. Due to the favour-able course of the operation single-stage ear surgery was agreed upon after intraoperative re-assessment and case discussion.

On the left side, intraoperative inspection revealed limited mobility and dysplasia of malleus and incus, no

Fig. 2 temporal bone Ct. para-axial reconstructions of the right (a) and left (b) stapes region demonstrate a present oval window (dotted arrows) and a hypoplastic right stapes (arrow)

Fig. 3 Intraoperative view after coupling the FMt to the stapes. On the right side (R), the FMt clip was directly crimped to the head of the dysplastic stapes which showed a missing anterior crus. On the

left side (L), a pORp vibroplasty was performed connecting the FMt to the stapes via a CLip coupler

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identifiable chorda tympani, limited visibility of the round window niche and normal course of the facial nerve. After malleus and incus were removed, normal mobility of the stapes was found. the filiform ear canal was opened and the bony part of the external auditory canal was enlarged. Despite maximum enlargement of the ear canal, it remained funnel shaped without an eardrum.

the following alternatives were discussed with respect to surgical hearing rehabilitation on the left side: (1) atre-sia surgery (in this case including tympanoplasty with titanium pORp) bearing the risk of re-stenosis of the newly formed external auditory canal, and of insufficient hearing with the necessity of additionally carrying con-ventional hearing aids with an earmold and of hearing deterioration with time; or (2) implantation of a partially implantable hearing aid with or without a pORp or with coupling of the FMt onto the stapes or the round win-dow membrane. Considering the poor accessibility of the round window and the sufficient dimensions of the tym-panic cavity (contrary to the right side), a pORp vibro-plasty was performed using a titanium CLip coupler, with interposition of the FMt between the stapes head and a cartilage plate of the reconstructed ear canal [7] (Fig. 3).

Audiological outcomes

the postoperative paediatric audiometric assessment 1 month after surgery showed AC thresholds on both sides of 50–55 dB HL at 0.5–1 kHz, 70–75 dB HL at 2 kHz and 90–100 dB HL at 4 kHz. At 3 months the word recogni-tion scores (German Mainzer mono- and bisyllabic word test at 65 dB spL) after activation of the VsB were 90 % on the right side, 80 % on the left side and 80 % bilaterally. the BC thresholds 6 months after surgery were 5–15 dB HL at 0.25–2 kHz and 30–40 dB HL at 4–6 kHz on both sides. Control speech audiometry at 6 months after surgery showed 100 % word recognition at 65 dB spL on both sides (see table 1).

Discussion

Our results show that active middle-ear implants are among the alternative amplification methods, which can be used in patients who are not suited medically to hearing aids or in whom hearing aids do not reach the desired effective-ness. Many clinical studies have been performed to demon-strate the benefits of VsB in adults [8–10]; however, only very few children worldwide have been fitted with them [11–13]. thus, our first experience of bilateral, single-stage vibroplasty in a 6-year-child with bilateral mixed hearing loss and meatal atresia associated with Franceschetti syn-drome encourages applying VsB to children with ear mal-formations. the speech intelligibility in quiet of 100 % at single use of the left or the right VsB demonstrates excel-lent audiological outcome. Improvements in hearing in noise [18] or directional hearing tests using bilateral VsB can be expected and will be evaluated at an appropriate age in the future.

In the past, the Baha® implant would have been used in patients with conductive hearing loss caused by the Franc-eschetti syndrome. However, the VsB overcomes the dis-advantages of the percutanoeus connection and risks of inflammations. On the other hand, the VsB is more inde-pendent of skull growth and therefore suitable for implanta-tion in children. Hearing improvement in speech and sound quality was comparable to previous report in children and adults [13–17].

In the right ear, due to the limited space in the tym-panic cavity and impaired accessibility of the round win-dow niche, the FMt was crimped directly to the dysplastic stapes. Existing data on different coupling methods are too limited to favour a specific one. However, in the left ear, we preferred a pORp vibroplasty, connecting the FMt to the stapes via a titanium CLip coupler in the presence of sufficient tympanic space and possible benefits of inter-position between stapes head and a cartilage plate of the reconstructed ear canal.

Although our decision to do a single-stage operation was mainly dictated by the higher risk of multiple anaesthesia

Table 1 pure-tone thresholds and word recognition scores for the unaided and aided conditions before and after implantation

BC bone conduction threshold, AC air conduction threshold, 4PTA pure-tone average (0.5, 1, 2, 4 kHz), dB HL decibel hearing loss, WRS word recognition score, SPL sound pressure level, BAHA bone anchored hearing aid, VSB Vibrant soundbridge

Before implantation 1 month after implantation 3 months after implantation

Right Left Binaural Right Left Binaural Right Left Binaural

BC-4ptA (dB HL) 20.0 17.5

AC-4ptA (dB HL) 66.3 68.8 68.8 66.3

Unaided WRs at 65 dB spL (%) 0 0 0

Aided WRs at 65 dB spL (%) 80BAHA 80BAHA 90BAHA 90VsB 80VsB 80VsB 100VsB 100VsB 100VsB

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and intubation, this case report demonstrates that VsB implantation in children could be safely performed and would lead to excellent results in hearing rehabilitation. Our experience may encourage other surgeons to consider a vibroplasty for surgical hearing rehabilitation in children, and in certain circumstances in a single stage. However, surgeons considering the use of these implants must take into account various aspects like the individual anatomical conditions, kind and degree of hearing loss, psychological impact, and––very importantly––the limitations for future MRI imaging after surgical hearing rehabilitation with implantable hearing aids. Ongoing studies have developed a score based on Ct scans for more precise risk stratifica-tion and decision making for AMEI candidates [19].

Acknowledgments stefan K. plontke is a consultant for Otonomy, Inc., san Diego, UsA. this work was not sponsored by Otonomy. stefan plontke and torsten Rahne also work on research projects sponsored by MedEl, Innsbruck, Austria. this work was not spon-sored by MedEl.

References

1. Jahrsdoerfer RA, Jacobson Jt (1995) treacher Collins syn-drome: otologic and auditory management. J Am Acad Audiol 6(1):93–102

2. pron G, Galloway C, Armstrong D et al (1993) Ear malforma-tion and hearing loss in patients with treacher Collins syndrome. Cleft palate Craniofac J 30(1):97–103

3. Ball GR (2010) the vibrant soundbridge: design and develop-ment. Adv Otorhinolaryngol 69:1–13

4. Colletti V, soli sD, Carner M et al (2006) treatment of mixed hearing losses via implantation of a vibratory transducer on the round window. Int J Audiol 45(10):600–608

5. Cremers CW, O’Connor AF, Helms J et al (2010) International consensus on Vibrant soundbridge® implantation in children and adolescents. Int J pediatr Otorhinolaryngol 74(11):1267–1269

6. Wollenberg B, Beltrame M, schönweiler R et al (2007) Inte-gration des aktiven Mittelohrimplantates in die plastische Ohr-muschelrekonstruktion (Integration of the active middle ear

implant Vibrant soundbridge in total auricular reconstruction). HNO 55(5):349–356

7. Mlynski R, Nguyen tD, plontke sK et al (2013) presentation of floating mass transducer and vibroplasty couplers on Ct and Cone Beam Ct. Eur Arch Otorhinolaryngol [Epub ahead of print]

8. Huber AM, Mlynski R, Müller J et al (2012) A new vibroplasty coupling technique as a treatment for conductive and mixed hear-ing losses: a report of 4 cases. Otol Neurotol 33(4):613–617

9. Beltrame AM, Martini A, prosser s et al (2009) Coupling the Vibrant soundbridge to cochlea round window: auditory results in patients with mixed hearing loss. Otol Neurotol 30(2):194–201

10. Colletti L, Mandalà M, Colletti V (2013) Long-term outcome of round window Vibrant soundBridge implantation in exten-sive ossicular chain defects. Otolaryngol Head Neck surg 149(1):134–141

11. Frenzel H, Hanke F, Beltrame M et al (2010) Application of the Vibrant soundbridge in bilateral congenital atresia in toddlers. Acta Otolaryngol 130(8):966–970

12. Lesinskas E, stankeviciute V, petrulionis M (2012) Application of the Vibrant soundbridge middle-ear implant for aural atre-sia in patients with treacher Collins syndrome. J Laryngol Otol 126(12):1216–1223

13. Roman s, Nicollas R, triglia J (2010) Middle ear implant for mixed hearing loss with malformation in a 9-year-old child. Eur Ann Otorhinolaryngol Head Neck Dis 127(1):11–14

14. Frenzel H, schönweiler R, Hanke F et al (2012) the Lübeck flowchart for functional and aesthetic rehabilitation of aural atre-sia and microtia. Otol Neurotol 33(8):1363–1367

15. Clarós p, pujol Mdel C (2013) Active middle ear implants: Vibro-plasty™ in children and adolescents with acquired or congenital middle ear disorders. Acta Otolaryngol 133(6):612–619

16. Roman s, Denoyelle F, Farinetti A et al (2012) Middle ear implant in conductive and mixed congenital hearing loss in chil-dren. Int J pediatr Otorhinolaryngol 76(12):1775–1778

17. Nadaraja Gs, Gurgel RK, Kim J, Chang KW (2013) Hearing outcomes of atresia surgery versus osseointegrated bone conduc-tion device in patients with congenital aural atresia: a systematic review. Otol Neurotol 34(8):1394–1399

18. Garin p, schmerber s, Magnan J, truy E et al (2010) Bilateral Vibrant soundbridge implantation: audiologic and subjec-tive benefits in quiet and noisy environments. Acta Otolaryngol 130(12):1370–137818

19. Frenzel H, sprinzl G, Widmann G et al (2013) Grading system for the selection of patients with congenital aural atresia for active middle ear implants. Neuroradiology 55(7):895–911