Hearing rehabilitation with bone conduction implant in patient with primary ciliary dyskinesia – a particular indication
DOI:
https://doi.org/10.34631/sporl.2087Keywords:
primary ciliary dyskinesia, bone conduction implant, mixed deafness, transmission deafnessAbstract
Primary ciliary dyskinesia (PCD) is a rare genetic disease characterized by an abnormality in the function or structure of the cilia, coursing with pulmonary, otological and nasosinusal manifestations. Hypoacusis affects 60% of patients with PCD and results mainly from otitis media with effusion (OME) and otological complications such as simple or cholesteatomatous chronic otitis media (COM).Clinical case: Female patient, 52 years old, with PCD, followed in the Otorhinolaryngology department for bilateral COM and chronic rhinosinusitis. Her surgical history includes nasosinusal endoscopic surgery and repeated myringotomies with placement of transtympanic ventilation tubes (TVTT), bilaterally. Despite the placement of TVTT, the simple tone audiogram revealed bilateral severe chronic mixed deafness, with mean bone thresholds of 36.25 dB on the right and 30 dB on the left. Ear tomography excluded erosion or discontinuity of ossicular chains, presence of cholesteatoma and pathology of the inner ear. The patient underwent surgery to place a bone conduction implant in her right ear. After 4 weeks, the processor was activated. The free field audiogram revealed a mean threshold of 26.25dB.
Conclusion: In PCD patients, insertion of TVTT is still a controversial treatment for OME. Persistent postoperative otorrhea is a frequent side effect and may complicate the use and tolerance of hearing aids. Bone conduction implants should be considered in auditory rehabilitation, with excellent results.
References
Takeuchi K, Kitano M, Sakaida H, Usui S, Masuda S, Ogawa S. et al. Analysis of otologic features of patients with primary ciliary dyskinesia. Otol Neurotol. 2017 Dec;38(10):e451-e456. doi: 10.1097/MAO.0000000000001599.
Alexandru M, de Boissieu P, Benoudiba F, Moustarhfir M, Kim S, Bequignon É. et al. Otological manifestations in adults with primary ciliary dyskinesia: a controlled radio-clinical study. J Clin Med. 2022 Aug 31;11(17):5163. doi: 10.3390/jcm11175163.
O’Callaghan C, Chilvers M, Hogg C, Bush A, Lucas J. Diagnosing primary ciliary dyskinesia. Thorax. 2007 Aug;62(8):656-7. doi: 10.1136/thx.2007.083147.
Takeuchi K, Kitano M, Ishinaga H, Kobayashi M, Ogawa S, Nakatani K. et al. Recent advances in primary ciliary dyskinesia. Auris Nasus Larynx. 2016 Jun;43(3):229-36. doi: 10.1016/j.anl.2015.09.012.
Luo W, Yi H, Taylor J, Li JD, Chi F, Todd NW. et al. Cilia distribution and polarity in the epithelial lining of the mouse middle ear cavity. Sci Rep. 2017 Mar 30;7:45870. doi: 10.1038/srep45870.
Majithia A, Fong J, Hariri M, Harcourt J. Hearing outcomes in children with primary ciliary dyskinesia—a longitudinal study. Int J Pediatr Otorhinolaryngol. 2005 Aug;69(8):1061-4. doi: 10.1016/j.ijporl.2005.02.013.
Prulière-Escabasse V, Coste A, Chauvin P, Fauroux B, Tamalet A, Garabedian EN. et al. Otologic features in children with primary ciliary dyskinesia. Arch Otolaryngol Head Neck Surg. 2010 Nov;136(11):1121-6. doi: 10.1001/archoto.2010.183.
Sommer JU, Schäfer K, Omran H, Olbrich H, Wallmeier J, Blum A. et al. ENT manifestations in patients with primary ciliary dyskinesia: prevalence and significance of otorhinolaryngologic co-morbidities. Eur Arch Otorhinolaryngol. 2011 Mar;268(3):383-8. doi: 10.1007/s00405-010-1341-9.
Bequignon E, Dupuy L, Zerah-Lancner F, Bassinet L, Honoré I, Legendre M. et al. Critical evaluation of sinonasal disease in 64 adults with primary ciliary dyskinesia. J Clin Med. 2019 May 7;8(5):619. doi: 10.3390/jcm8050619.
Randel A, Editor. AAO–HNSF releases guideline on tympanostomy tubes in children [Internet]. 2014. Available from: https://www.aafp.org/pubs/afp/issues/2014/0501/p754.html
Wolter NE, Dell SD, James AL, Campisi P. Middle ear ventilation in children with primary ciliary dyskinesia. Int J Pediatr Otorhinolaryngol. 2012 Nov;76(11):1565-8. doi: 10.1016/j.ijporl.2012.07.011.
Zawawi F, Shapiro AJ, Dell S, Wolter NE, Marchica CL, Knowles MR. et al. Otolaryngology manifestations of primary ciliary dyskinesia: a multicenter study. Otolaryngol Head Neck Surg. 2022 Mar;166(3):540-547. doi: 10.1177/01945998211019320.
Campbell RG, Birman CS, Morgan L. Management of otitis media with effusion in children with primary ciliary dyskinesia: a literature review. Int J Pediatr Otorhinolaryngol. 2009 Dec;73(12):1630-8. doi: 10.1016/j.ijporl.2009.08.024.
Hadfield PJ, Rowe-Jones JM, Bush A, Mackay IS. Treatment of otitis media with effusion in children with primary ciliary dyskinesia. Clin Otolaryngol Allied Sci. 1997 Aug;22(4):302-6. doi: 10.1046/j.1365-2273.1997.00020.x.
Alexandru M, Veil R, Rubbo B, Goutaki M, Kim S, Lam YT. et al. Ear and upper airway clinical outcome measures for use in primary ciliary dyskinesia research: a scoping review. Eur Respir Rev. 2023 Jul 12;32(169):220200. doi: 10.1183/16000617.0200-2022.
Edmiston RC, Aggarwal R, Green KMJ. Bone conduction implants – a rapidly developing field. J Laryngol Otol. 2015 Oct;129(10):936-40. doi: 10.1017/S0022215115002042.
Noone PG, Leigh MW, Sannuti A, Minnix SL, Carson JL, Hazucha M. et al. Primary ciliary dyskinesia. Am J Respir Crit Care Med. 2004 Feb 15;169(4):459-67. doi: 10.1164/rccm.200303-365OC.
Ah-Tye C, Paradise JL, Colborn DK. Otorrhea in young children after tympanostomy-tube placement for persistent middle-ear effusion: prevalence, incidence, and duration. Pediatrics. 2001 Jun;107(6):1251-8. doi: 10.1542/peds.107.6.1251.
Golz A, Netzer A, Joachims HZ, Westerman ST, Gilbert LM. Ventilation Tubes and Persisting Tympanic Membrane Perforations. Otolaryngol Head Neck Surg. 1999 Apr;120(4):524-7. doi: 10.1177/019459989912000401. Otolaryngology–Head and Neck Surgery. 1999 Apr 7;120(4):524–7.
Bush A, Cole P, Hariri M, Mackay I, Phillips G, O’Callaghan C. et al. Primary ciliary dyskinesia: diagnosis and standards of care. Eur Respir J. 1998 Oct;12(4):982-8. doi: 10.1183/09031936.98.12040982.
den Besten CA, Monksfield P, Bosman A, Skarzynski PH, Green K, Runge C. et al. Audiological and clinical outcomes of a transcutaneous bone conduction hearing implant: six‐month results from a multicentre study. Clin Otolaryngol. 2019 Mar;44(2):144-157. doi: 10.1111/coa.13248.
Sprinzl GM, Wolf‐Magele A. The Bonebridge bone conduction hearing implant: indication criteria, surgery and a systematic review of the literature. Clin Otolaryngol. 2016 Apr;41(2):131-43. doi: 10.1111/coa.12484.
Mylanus EAM, Hua H, Wigren S, Arndt S, Skarzynski PH, Telian SA. et al. Multicenter clinical investigation of a new active osseointegrated steady-state implant system. Otol Neurotol. 2020 Oct;41(9):1249-1257. doi: 10.1097/MAO.0000000000002794.
Edmiston RC, Aggarwal R, Green KMJ. Bone conduction implants – a rapidly developing field. J Laryngol Otol. 2015 Oct;129(10):936-40. doi: 10.1017/S0022215115002042.
Dimitriadis PA, Farr MR, Allam A, Ray J. Three-year experience with the cochlear BAHA attract implant: a systematic review of the literature. BMC Ear Nose Throat Disord. 2016 Oct 1;16:12. doi: 10.1186/s12901-016-0033-5.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2024 Portuguese Journal of Otorhinolaryngology and Head and Neck Surgery
This work is licensed under a Creative Commons Attribution-ShareAlike 4.0 International License.
