V Couloigner1, M Gratacap2, E Ambert-Dahan3, S Borel3, V Ettienne2, A Kerouedan2, D Bouccara3, M Zerah4, M Kalamarides5, O Sterkers3. 1. Service d'ORL pédiatrique, université Paris Descartes, hôpital Necker-Enfants-Malades, AP-HP, 149, rue de Sèvres, 75015 Paris, France. Electronic address: vincent.couloigner@nck.aphp.fr. 2. Service d'ORL pédiatrique, université Paris Descartes, hôpital Necker-Enfants-Malades, AP-HP, 149, rue de Sèvres, 75015 Paris, France. 3. Service d'ORL, université Pierre-et-Marie-Curie, hôpital de la Pitié-Salpêtrière, AP-HP, Paris, France. 4. Service de neurochirurgie, université Paris Descartes, hôpital Necker-Enfants-Malades, AP-HP, Paris, France. 5. Service de neurochirurgie, université Pierre-et-Marie-Curie, hôpital de la Pitié-Salpêtrière, AP-HP, Paris, France.
Abstract
AIM OF THE STUDY: To present three pediatric cases of auditory brainstem implantation (ABI) and review literature data concerning this topic. PATIENTS: The first two children had a neurofibromatosis type II with bilateral sensorineural deafness; in both cases, the implant was inserted during the surgical removal of a vestibular schwannoma; the third patient had profound deafness due to bilateral cochlear nerve insufficiency associated with inner ear malformation. RESULTS: Two postoperative complications were observed: patient 1 had a persistent fever which required the replacement of the fat graft used to seal the translabyrinthine approach; patient 3 had a CSF leakage requiring additional surgery and lumbar external drainage. In our three patients, the numbers of active electrodes were 6/22 (Cochlear ABI 24M ABI), 11/12 (Medel Opus II ABI) and 11/12 (implant Medel), respectively. Due to additional major surgical procedures and to disappointing functional results of the ABI, patient 1 stopped wearing her implant 18 months after implantation. Nine months after surgery, patient 2 achieved open-set speech recognition and was very satisfied with the implant. Six months after implantation, patient 3 (cochlear nerve deficiency), who was 3.5 years-old at the time, clearly reacted to some environmental sounds but was not yet able to achieve speech recognition. CONCLUSIONS: ABI has now entered the list of treatments that can be proposed in pediatric profound sensorineural deafness. Its major risks of complications are CSF leakage and non-auditory side effects. Its outcomes are worse and less predictable than cochlear implants. Thus, its indications must remain restricted to cases meeting the following conditions: absence of alternative option to restore hearing, patients and parents high level of motivation and realistic expectations.
AIM OF THE STUDY: To present three pediatric cases of auditory brainstem implantation (ABI) and review literature data concerning this topic. PATIENTS: The first two children had a neurofibromatosis type II with bilateral sensorineural deafness; in both cases, the implant was inserted during the surgical removal of a vestibular schwannoma; the third patient had profound deafness due to bilateral cochlear nerve insufficiency associated with inner ear malformation. RESULTS: Two postoperative complications were observed: patient 1 had a persistent fever which required the replacement of the fat graft used to seal the translabyrinthine approach; patient 3 had a CSF leakage requiring additional surgery and lumbar external drainage. In our three patients, the numbers of active electrodes were 6/22 (Cochlear ABI 24M ABI), 11/12 (Medel Opus II ABI) and 11/12 (implant Medel), respectively. Due to additional major surgical procedures and to disappointing functional results of the ABI, patient 1 stopped wearing her implant 18 months after implantation. Nine months after surgery, patient 2 achieved open-set speech recognition and was very satisfied with the implant. Six months after implantation, patient 3 (cochlear nerve deficiency), who was 3.5 years-old at the time, clearly reacted to some environmental sounds but was not yet able to achieve speech recognition. CONCLUSIONS: ABI has now entered the list of treatments that can be proposed in pediatric profound sensorineural deafness. Its major risks of complications are CSF leakage and non-auditory side effects. Its outcomes are worse and less predictable than cochlear implants. Thus, its indications must remain restricted to cases meeting the following conditions: absence of alternative option to restore hearing, patients and parents high level of motivation and realistic expectations.
Keywords:
Cochlear nerve deficiency; Deafness; Dysplasie du nerf cochléaire; Hearing loss; NF2; Neurofibromatose de type 2; Neurofibromatosis type II; Surdité
Authors: Eric P Wilkinson; Laurie S Eisenberg; Mark D Krieger; Marc S Schwartz; Margaret Winter; Jamie L Glater; Amy S Martinez; Laurel M Fisher; Robert V Shannon Journal: Otol Neurotol Date: 2017-02 Impact factor: 2.311
Authors: John Ka Keung Sung; Betty Pui Ki Luk; Terence Ka Cheong Wong; Jiun Fong Thong; Hoi Tung Wong; Michael Chi Fai Tong Journal: Audiol Neurootol Date: 2018-09-18 Impact factor: 1.854