Otological Manifestations in Adults with Primary Ciliary Dyskinesia: A Controlled Radio-Clinical Study
Abstract
:1. Introduction
2. Materials and Methods
3. Results
3.1. Patients
3.2. Clinical Examination
3.3. Hearing Evaluation
3.4. Temporal Bone CT-Scan Exploration
4. Discussion
5. Conclusions
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
References
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PCD 1 Group N = 17 | Control Group N = 17 | p-Value | |
---|---|---|---|
Age, median (Q1–Q3) | 39 (33–54) | 39 (33–54) | 0.89 |
Gender male, n (%) | 8 (47%) | 6 (35%) | 0.49 |
Ciliary ultrastructure defect, n (%) | |||
nEM 2 | 5 (29%) | 17 (100%) | - |
IDA + MTD 3 | 3 (18%) | 0 | - |
ODA 4 | 6 (35%) | 0 | - |
IDA + ODA | 3 (18%) | 0 | - |
CC 5 | 0 | 0 | - |
Kartagener syndrome, n (%) | 3 (18%) | 0 | 0.22 |
Consanguinities, n (%) | 8 (47%) | 3 (18%) | 0.07 |
Otitis media history, n (%) | 10 (59%) | 3 (18%) | 0.01 |
Rhinosinusitis history, n (%) | 17 (100%) | 13 (76%) | 0.1 |
CRSwNP 6, n(%) | 7 (41%) | 2 (12%) | 0.1 |
SNOT-22 7 score, median (Q1–Q3) | 58 (46.5–69) | 33.5 (14.2–46) | 0.01 |
Sinus hypo or aplasia, n (%) | 15 (88%) | 3 (18%) | 0.0001 |
Modified Lund–Mackay score, median (Q1–Q3) | 13.5 (10–19.2) | 6 (2.7–10.2) | 0.01 |
More Affected Ear | PCD 1 Group N = 17 | Control Group N = 17 | p-Value |
---|---|---|---|
Abnormal otoscopy, n (%) | 13 (76%) | 4 (24%) | 0.002 |
| 7 | 3 | |
| 4 | 1 | 0.04 |
| 2 | 0 | |
Hearing loss, median (Q1–Q3) (dB) | 18.75 (13.75–42.5) | 12.5 (11.25–18.75) | 0.03 |
Hearing loss, n(%) | |||
None | 6 (35%) | 12 (71%) | |
Sensorineural | 3 (18%) | 2 (12%) | 0.51 |
Conductive | 4 (24%) | 2 (12%) | |
Mixed | 4 (24%) | 1 (6%) | |
Air-bone gap 250–500 Hz, median (Q1–Q3) (dB) | 5 (5–17.5) | 2.5 (0–10) | 0.02 |
Air-bone gap 1000–2000–4000 Hz, Median (Q1–Q3) (dB) | 10 (2.5–12.5) | 0 (0–5) | 0.01 |
SRT 5, median (Q1–Q3) | 25 (22.5–48.5) | 23.5 (17–26) | 0.06 |
Intelligibility 100%, median (Q1–Q3) | 40 (35–65) | 30 (25–40) | 0.01 |
Hearing threshold: | |||
500 Hz, median (Q1–Q3) | 20 (15–35) | 15 (10–20) | 0.04 |
1000 Hz, median (Q1–Q3) | 20 (10–40) | 10 (10–20) | 0.07 |
2000 Hz, median (Q1–Q3) | 15 (10–35) | 10 (10–15) | 0.08 |
4000 Hz, median (Q1–Q3) | 35 (15–45) | 20 (15–20) | 0.04 |
Less Affected Ear | PCD 1 Group N = 17 | Control Group N = 17 | p-Value |
---|---|---|---|
Abnormal otoscopy, n (%) | 12 (71%) | 4 (24%) | 0.006 |
| 6 | 0 | |
| 5 | 2 | 0.01 |
| 1 | 2 | |
Hearing loss, median (Q1–Q3) (dB) | 17.5 (10–33.75) | 8.75 (6.25–13.75) | 0.03 |
Type of hearing loss, n (%) | |||
None | 8 (47%) | 12 (71%) | |
Sensorineural | 3 (18%) | 3 (18%) | |
Conductive | 4 (24%) | 2 (12%) | 0.49 |
Mixed | 2 (12%) | 0 | |
Air-bone gap 250–500 Hz, median (Q1–Q3) | 5 (0–17.5) | 0 (0–2.5) | 0.04 |
Air-bone gap 1000–2000–4000 Hz, Median (Q1–Q3) | 5 (0–10) | 0 (0–0) | 0.002 |
SRT 5, median (Q1–Q3) (dB) | 25 (20–40) | 22 (20–26) | 0.10 |
Intelligibility 100%, median (Q1–Q3) | 40 (30–55) | 30 (25–40) | 0.02 |
Hearing thresholds: | |||
500 Hz, median (Q1–Q3) | 15 (10–25) | 10 (10–15) | 0.50 |
1000 Hz, median (Q1–Q3) | 15 (10–20) | 5 (5–10) | 0.03 |
2000 Hz, median (Q1–Q3) | 10 (10–30) | 5 (5–10) | 0.03 |
4000 Hz, median (Q1–Q3) | 20 (15–35) | 15 (10–15) | 0.01 |
PCD 1 Group N = 34 N (%) | Control Group N = 34 N (%) | p-Value | |
---|---|---|---|
Abnormal CT scan, | 32 (94%) | 11 (32%) | <0.0001 |
Mastoid opacification | 13 (38%) | 8 (24%) | 0.19 |
Mastoid condensation | 21 (62%) | 8 (24%) | 0.01 |
Middle ear inflammation | 22 (65%) | 7 (21%) | 0.0002 |
Ossicular anomaly | 12 (35%) | 3 (9%) | 0.01 |
Other: | |||
No other anomaly | 28 (85%) | 27 (79%) | |
Gusher syndrome | 0 | 2 (6%) | 0.76 |
SCC 2 dehiscence | 3 (9%) | 3 (9%) |
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Alexandru, M.; de Boissieu, P.; Benoudiba, F.; Moustarhfir, M.; Kim, S.; Bequignon, É.; Honoré, I.; Garcia, G.; Mitri-Frangieh, R.; Legendre, M.; et al. Otological Manifestations in Adults with Primary Ciliary Dyskinesia: A Controlled Radio-Clinical Study. J. Clin. Med. 2022, 11, 5163. https://doi.org/10.3390/jcm11175163
Alexandru M, de Boissieu P, Benoudiba F, Moustarhfir M, Kim S, Bequignon É, Honoré I, Garcia G, Mitri-Frangieh R, Legendre M, et al. Otological Manifestations in Adults with Primary Ciliary Dyskinesia: A Controlled Radio-Clinical Study. Journal of Clinical Medicine. 2022; 11(17):5163. https://doi.org/10.3390/jcm11175163
Chicago/Turabian StyleAlexandru, Mihaela, Paul de Boissieu, Farida Benoudiba, Malik Moustarhfir, Sookyung Kim, Émilie Bequignon, Isabelle Honoré, Gilles Garcia, Rana Mitri-Frangieh, Marie Legendre, and et al. 2022. "Otological Manifestations in Adults with Primary Ciliary Dyskinesia: A Controlled Radio-Clinical Study" Journal of Clinical Medicine 11, no. 17: 5163. https://doi.org/10.3390/jcm11175163
APA StyleAlexandru, M., de Boissieu, P., Benoudiba, F., Moustarhfir, M., Kim, S., Bequignon, É., Honoré, I., Garcia, G., Mitri-Frangieh, R., Legendre, M., Crestani, B., Taillé, C., Escudier, E., Maitre, B., Papon, J.-F., & Nevoux, J. (2022). Otological Manifestations in Adults with Primary Ciliary Dyskinesia: A Controlled Radio-Clinical Study. Journal of Clinical Medicine, 11(17), 5163. https://doi.org/10.3390/jcm11175163