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Bone-anchored hearing aid (Baha) in patients with Treacher Collins syndrome: tips and pitfalls.
Int J Pediatr Otorhinolaryngol. 2011 Oct; 75(10):1308-12.IJ

Abstract

OBJECTIVES

Treacher Collins syndrome, also known as mandibulofacial dysostosis, is an autosomal dominant disorder of the cranio-facial morphogenesis affecting 1 of 50,000 live newborns. Most children with this disease present with bilateral, severe conductive hearing loss due to bilateral aural atresia. Auditory rehabilitation of these children can be effectively carried out with bone-anchored hearing aids (Baha). The aim of this retrospective study is to review the "Bambino Gesù" Children's Hospital's experience with Baha in Treacher Collins patients, highlighting the tips and pitfalls of Baha surgery in this particular population.

METHODS

The clinical charts were reviewed of all children with a Treacher Collins syndrome diagnosis receiving a Baha in the "Bambino Gesù" Children's Hospital from January 1995 to January 2010. Data were collected concerning patients' anagraphics and medical history, comorbidities, surgical technique, complications and functional outcome.

RESULTS

23 Treacher Collins children were included. A two-stage surgery was adopted in 51% (n=12) cases, while a one-stage approach was chosen in 49% (n=11). 2 children underwent 1st stage surgery as they were younger than 5. There were neither cases of osseointegration failure, nor cases of traumatic implant loss. Overall, the local complication rate was not different in the one-stage and in the two-stage group. The functional gain was significantly better with the Baha than with the conventional bone-conduction hearing aids (p<0.0002). There was no difference in terms of functional outcome between the 7 patients receiving Baha at an age younger than 5 years and the rest (p=0.23).

CONCLUSIONS

Baha can provide effective auditory rehabilitation for children with Treacher Collins syndrome, as long as it is performed in a tertiary care center where a multidisciplinary approach to the frequent comorbidities is possible. The main challenge of Baha surgery in this population is the poor or irregular thickness of the patient's calvarial bone, which often makes it necessary to drill additional holes, to place the fixture in contact with the dura or to use materials for bone augmentation.

Authors+Show Affiliations

"Bambino Gesù" Children's Hospital, Rome, Vatican City State.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

21839526

Citation

Marsella, Pasquale, et al. "Bone-anchored Hearing Aid (Baha) in Patients With Treacher Collins Syndrome: Tips and Pitfalls." International Journal of Pediatric Otorhinolaryngology, vol. 75, no. 10, 2011, pp. 1308-12.
Marsella P, Scorpecci A, Pacifico C, et al. Bone-anchored hearing aid (Baha) in patients with Treacher Collins syndrome: tips and pitfalls. Int J Pediatr Otorhinolaryngol. 2011;75(10):1308-12.
Marsella, P., Scorpecci, A., Pacifico, C., & Tieri, L. (2011). Bone-anchored hearing aid (Baha) in patients with Treacher Collins syndrome: tips and pitfalls. International Journal of Pediatric Otorhinolaryngology, 75(10), 1308-12. https://doi.org/10.1016/j.ijporl.2011.07.020
Marsella P, et al. Bone-anchored Hearing Aid (Baha) in Patients With Treacher Collins Syndrome: Tips and Pitfalls. Int J Pediatr Otorhinolaryngol. 2011;75(10):1308-12. PubMed PMID: 21839526.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Bone-anchored hearing aid (Baha) in patients with Treacher Collins syndrome: tips and pitfalls. AU - Marsella,Pasquale, AU - Scorpecci,Alessandro, AU - Pacifico,Concettina, AU - Tieri,Luigi, Y1 - 2011/08/11/ PY - 2011/05/12/received PY - 2011/07/18/revised PY - 2011/07/19/accepted PY - 2011/8/16/entrez PY - 2011/8/16/pubmed PY - 2012/4/28/medline SP - 1308 EP - 12 JF - International journal of pediatric otorhinolaryngology JO - Int J Pediatr Otorhinolaryngol VL - 75 IS - 10 N2 - OBJECTIVES: Treacher Collins syndrome, also known as mandibulofacial dysostosis, is an autosomal dominant disorder of the cranio-facial morphogenesis affecting 1 of 50,000 live newborns. Most children with this disease present with bilateral, severe conductive hearing loss due to bilateral aural atresia. Auditory rehabilitation of these children can be effectively carried out with bone-anchored hearing aids (Baha). The aim of this retrospective study is to review the "Bambino Gesù" Children's Hospital's experience with Baha in Treacher Collins patients, highlighting the tips and pitfalls of Baha surgery in this particular population. METHODS: The clinical charts were reviewed of all children with a Treacher Collins syndrome diagnosis receiving a Baha in the "Bambino Gesù" Children's Hospital from January 1995 to January 2010. Data were collected concerning patients' anagraphics and medical history, comorbidities, surgical technique, complications and functional outcome. RESULTS: 23 Treacher Collins children were included. A two-stage surgery was adopted in 51% (n=12) cases, while a one-stage approach was chosen in 49% (n=11). 2 children underwent 1st stage surgery as they were younger than 5. There were neither cases of osseointegration failure, nor cases of traumatic implant loss. Overall, the local complication rate was not different in the one-stage and in the two-stage group. The functional gain was significantly better with the Baha than with the conventional bone-conduction hearing aids (p<0.0002). There was no difference in terms of functional outcome between the 7 patients receiving Baha at an age younger than 5 years and the rest (p=0.23). CONCLUSIONS: Baha can provide effective auditory rehabilitation for children with Treacher Collins syndrome, as long as it is performed in a tertiary care center where a multidisciplinary approach to the frequent comorbidities is possible. The main challenge of Baha surgery in this population is the poor or irregular thickness of the patient's calvarial bone, which often makes it necessary to drill additional holes, to place the fixture in contact with the dura or to use materials for bone augmentation. SN - 1872-8464 UR - https://www.unboundmedicine.com/medline/citation/21839526/Bone_anchored_hearing_aid__Baha__in_patients_with_Treacher_Collins_syndrome:_tips_and_pitfalls_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0165-5876(11)00345-4 DB - PRIME DP - Unbound Medicine ER -