Bergstrom
(
13
13 Bergstrom L. Osteogenesis imperfecta: otologic and maxilla facial aspects. Laryngoscope. 1977;87(9 Pt 2 Suppl 6):1-42. PMid:330992.
)
|
n=: 32 pediatric patients, mean age 5 years |
Cross-sectional |
Absent Features audiograms |
CHL:14% SHL:9% MHL: 4.5% |
Carruth et al.
(
14
14 Carruth JA, Lutman ME, Stephens SD. Anaudiological investigation of osteogenesis imperfecta. J Laryngol Otol. 1978;92(10):853-60. http://dx.doi.org/10.1017/S0022215100086229. PMid:712217. http://dx.doi.org/10.1017/S0022215100086...
)
|
n=22 GCa n=10 GCo: n=12 |
Case control |
Absent |
In the study group, 6 people had conductive hearing loss, ranging from 20 dB to 50 dB, 1 of which was a result of otitis media. |
Riedner et al.
(
15
15 Riedner ED, Levin LS, Holliday MJ. Hearing patterns in dominant osteogenesis imperfecta. Arch Otolaryngol. 1980;106(12):737-40. http://dx.doi.org/10.1001/archotol.1980.00790360015006. PMid:7436848. http://dx.doi.org/10.1001/archotol.1980....
)
|
n= 70, 13 families, 5 to 48 years |
Cross-sectional |
Hearing loss: >25 dBHL between 250 and 8 kHz CHL: gap of 5 dBNA (500-4 kHz) |
OI hearing loss begins in the second or third decade. In general, younger patients have CHL and the older MHL or SHL. |
Shea and Postma
(
16
16 Shea JJ, Postma DS. Findings and long-term surgical results in the hearing loss of osteogenesis imperfecta. Arch Otolaryngol. 1982;108(8):467-70. http://dx.doi.org/10.1001/archotol.1982.00790560005002. PMid:7103822. http://dx.doi.org/10.1001/archotol.1982....
)
|
n=43 (62 ears) age: 2 to 50 years |
Follow-up |
Absent |
84% bilateral CHL. |
Shapiro et al.
(
17
17 Shapiro JR, Pikus A, Weiss G, Rowe DW. Hearing and Middle Ear Function in Osteogenesis Imperfecta. JAMA. 1982;247(15):2120-6. http://dx.doi.org/10.1001/jama.1982.03320400032030. PMid:7062527. http://dx.doi.org/10.1001/jama.1982.0332...
)
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n=55 OI patients, 92 family members 43 controls |
Case control |
NHL: drop below 15 dBHL in consecutive frequencies CHL: GAP≥ 15 dBHL in one or more frequencies AN: without gap presence, drop greater than 15 dBHL CHOI: SHL at high frequencies (6 and 8) |
1. not only typical SHL occurs more than CHL or MHL, an atypical and characteristic SHL pattern occurs with significant frequency in OI patients and their relatives; 2. Middle ear function is also abnormal in OI, probably due to the involvement of the ligament and ossicle. The middle ear may also be abnormal in many first-degree relatives with no history of fracture; 3. These abnormalities are influenced by age. |
Cox and Simmons
(
18
18 Cox JR, Simmons CL. Osteogenesis imperfecta and associated hearing loss in five kindreds. South Med J. 1982;75(10):1222-6. http://dx.doi.org/10.1097/00007611-198210000-00016. PMid:7123292. http://dx.doi.org/10.1097/00007611-19821...
)
|
n = 30, 5 families, ages 4 to 67 years, 57% between 4 and 20 years |
Cross-sectional |
Hearing loss: pure tone> 20 dBHL between 250 and 1 kHz and / or> 25 dBHL between 2 and 6 kHz |
11 participants with loss, mean 26 years (9 to 67 years) Of the 11 with loss: 6 mild CHL, 2 moderate MHL and 2 mild, 1 moderate CHL at high frequencies. |
Pedersen
(
19
19 Pedersen U. Hearing loss in patients with osteogenesis imperfecta. A clinical and audiological study of 201 patients. Scand Audiol. 1984;13(2):67-74. http://dx.doi.org/10.3109/01050398409043042. PMid:6463554. http://dx.doi.org/10.3109/01050398409043...
)
|
n = 201, less than 10 to more than 70 years |
Cross-sectional |
SHL:AC≥15 dBHL and gap<15 dBHL CHL:BC >15 dBHL and gap≥15 dBHL MHL: BC≥15 dBHL and gap≥15 dBHL For frequencies from 250 to 4 kHz |
NHL 50% MHL 27% CHL 12% SHL 8% Anacusia 3% |
Stewart and O’Reilly
(
9
9 Stewart EJ, O’Reilly BF. A clinical and audiological investigation of osteogenesis irnperfecta. Clin Otolaryngol. 1989;14(6):509-14. http://dx.doi.org/10.1111/j.1365-2273.1989.tb00414.x. PMid:2612030. http://dx.doi.org/10.1111/j.1365-2273.19...
)
|
n= 56, age: 10 to 60 years |
Cross-sectional |
SHL: VA≥ 30 dBHL on at least two frequencies between 250 and 8 kHz CHL: gap≥ 15 dBHL on at least two frequencies between 250 and 4 kHz MHL: BC≥ 30 dBHL and gap≥ 15 dBHL on at least two frequencies between 250 and 8 kHz |
MHL: 16% CHL: 14% SHL: 12% NHL: 4% Results for 46 ears with tympanogram. |
Pedersen et al.
(
20
20 Pedersen U, Melsen F, Elbrond O, Charles P. Histopathology of the stapes in osteogenesis imperfecta. J Laryngol Otol. 1985;99(5):451-8. http://dx.doi.org/10.1017/S0022215100097036. PMid:3998630. http://dx.doi.org/10.1017/S0022215100097...
)
|
213 patients |
Cross-sectional |
Absent |
50% NHL 27% MHL 12% CHL 8% SHL 3% Anacusia |
Garretsen et al.
(
21
21 Garretsen AJ, Cremers CW, Huygén PL. Hearing loss (in nonoperated ears) in relation to age in osteogenesis imperfecta type I. Ann Otol Rhinol Laryngol. 1997;106(7 Pt 1):575-82. http://dx.doi.org/10.1177/000348949710600709. PMid:9228859. http://dx.doi.org/10.1177/00034894971060...
)
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142 participants with type I OI, divided into children and under 30 years of age |
Case control |
CHL: mean gap in 500, 1 and 2 or 4 and 8 ≥ 15 dBHL and BC <15 dBHL SHL: AC ≥ 15 dBHL, gap below 15 dBHL MHL: GAP≥15 dBHL and BC≥15 dBHL CHOI: Shapiro et al.(1717 Shapiro JR, Pikus A, Weiss G, Rowe DW. Hearing and Middle Ear Function in Osteogenesis Imperfecta. JAMA. 1982;247(15):2120-6. http://dx.doi.org/10.1001/jama.1982.03320400032030. PMid:7062527. http://dx.doi.org/10.1001/jama.1982.0332...
)
|
MHL:<30 years:37% ≥30 years:68% NHL:<30 years:34% ≥ 30 years:6% SHL:<30 years:18% ≥30 years:19% CHOI:<30 years:7% ≥30 years:5% CHL:<30 years: 2% ≥ 30 years:0% Disabling:<30 years:2% ≥30 years:2% |
Kuurila et al.
(
22
22 Kuurila K, Grénman R, Johansson R, Kaitila I. Hearing loss in children with osteogenesis imperfect. Eur J Pediatr. 2000;159(7):515-9. http://dx.doi.org/10.1007/s004310051322. PMid:10923226. http://dx.doi.org/10.1007/s004310051322...
)
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45 children, average age: 10 years, maximum age: 16 years |
Cross-sectional |
NHL: AC ≤ 20 dBHL CHL: gap≥ 15 dBHL and BC<15 dBHL SHL: BC ≥ 15 dBHL and gap<15 dBHL MHL: gap>15 dBHL and BC ≥ 15 dBHL For mean 500-2 kHz |
NHL: 93.3% The three cases of loss were 2 CHL and 1 SHL from birth, probably of etiology unrelated to OI. |
Imani et al.
(
23
23 Imani P, Vijayasekaran S, Lannigan F. Is it necessary to screen for hearing loss in the paediatric population with osteogenesis imperfecta? Clin Otolaryngol Allied Sci. 2003;28(3):199-202. http://dx.doi.org/10.1046/j.1365-2273.2003.00685.x. PMid:12755755. http://dx.doi.org/10.1046/j.1365-2273.20...
)
|
n = 22, mean age: 9.64 years |
Cohort |
Not described. Presents altered audiometry results |
Incidence of loss: 77.3% Losses were of conductive component that was solved and 5 children with permanent loss, 3 SHL and 2 CHL. |
Paterson et al.
(
24
24 Paterson CR, Monk EA, McAllion SJ. How common is hearing impairment in osteogenesis imperfecta? J Laryngol Otol. 2001;115(4):280-2. http://dx.doi.org/10.1258/0022215011907442. PMid:11276328. http://dx.doi.org/10.1258/00222150119074...
)
|
n: 1.394 0 to 70 years |
Cross-sectional |
Absent |
The onset of loss between the second and fourth decade of life was more common. At age 50, approximately 50% had loss. In the next 20 years, there was little increase. Hearing loss was significantly lower in type IV OI than in type I OI. |
Pillion and Shapiro
(
7
7 Pillion JP, Shapiro J. Audiological findings in osteogenesis imperfecta. J Am Acad Audiol. 2008;19(8):595-601. http://dx.doi.org/10.3766/jaaa.19.8.3. PMid:19323351. http://dx.doi.org/10.3766/jaaa.19.8.3...
)
|
n=41 Groups: >20 years (n=21) <20 years (n=20) Mean age: 26.54 (2 to 68 years) |
Cross-sectional |
SHL: AC>20 dBNA 250 to 8 kHz and gap<10 dBNA from 250 to 4 kHz CHOI: gap<10 dBNA from 250 to 4 kHz e high threshold from 6 to 8 kHz CHL: AC>20 dBNA and gap>10 dBNA from 250 to 4 kHz MHL: AC>20 dBNA and BC>15 dBNA with gap>10 dBNA from 250 to 4 kHz |
loss of 62% ears; prevalence> 20 years: 88%; <: 38% SHL or MHL: 41% CHL: 21% In children (mean age 9.87 SD: 4.33) CHL is predominant and in adults (mean age: 44.05 SD: 12.44) MHL is predominant. |
Swinnen et al.
(
4
4 Swinnen FK, Coucke PJ, De Paepe AM, Symoens S, Malfait F, Gentile FV, Sangiorgi L, D’Eufemia P, Celli M, Garretsen TJ, Cremers CW, Dhooge IJ, De Leenheer EM. Osteogenesis imperfecta: the audiological phenotype lacks correlation with the genotype. Orphanet J Rare Dis. 2011;6(1):88. http://dx.doi.org/10.1186/1750-1172-6-88. PMid:22206639. http://dx.doi.org/10.1186/1750-1172-6-88...
)
|
n = 184, mean age: 30.5 (3 to 89 years). |
Cohort |
NHL: AC<15 dBNA in 500, 1 and 2 CHL: BC<15 dBNA and gap≥15 dBNA on average of 500, 1 and 2 SHL: AC≥15 dBNA and gap<15 dBNA on average of 500, 1 and 2 or AC>30 dBNA on average of 4, 6 and 8 MHL: BC≥15 dBNA and gap≥15 dBNA on average of 500, 1 and 2 |
Loss in 52.7% 44% bilateral 8.7% unilateral |
Swinnen et al.
(
8
8 Swinnen FKR, De Leenheer EMR, Goemaere S, Cremers CWRJ, Coucke PJ, Dhooge IJM. Association between bone mineral density and hearing loss in osteogenesis imperfecta. Laryngoscope. 2012;122(2):401-8. http://dx.doi.org/10.1002/lary.22408. PMid:22252604. http://dx.doi.org/10.1002/lary.22408...
)
|
n=56 |
Cross-sectional |
NHL: AC<15 dBNA CHL: BC<15 dBNA and gap>15 dBNA MHL: BC>15 dBNA and gap>15 dBNA SHL: AC>15 dBNA and gap<15 dBNA Deafness: AC>20 dBNA |
39% NHL 21% MHL 11% SHL 4% SHL high frequency 3% CHL |