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TABLE I. Data Pertaining to Patients With EVA.

Characteristic

Unilateral, n ¼ 74

Bilateral, n ¼ 70

P

Male

38 (51%)

29 (41%)

.26 .01 .02 .05

Age at identification of hearing loss, mo*

70 (0–324.5) [39.5–106] 32.8 (0.5–164) [13.5–69.5]

54.5 (0–208) [32.5–69.5] 56.3 (0.5–812.5) [33.8–76.8]

Follow-up time, mo †

3 months of follow-up

55 (74.3%) 34 (46.0%)

61 (87.1)

Unilateral hearing loss at initial assessment Pure tone average in better-hearing ear, dB Hearing loss severity in better-hearing ear

11 (15.7%)

< .0001

37.9 (10–130) [27.5–65]

50 (6.25–130) [32.5–70]

.19 .48

Mild

30 (40.5%) 11 (14.9%) 11 (14.9%)

21 (30.0%) 15 (21.4%) 12 (17.1%)

Moderate

Moderately severe

Severe

3 (4.1%)

7 (10%)

Profound

11 (14.9%) 8 (10.8%)

10 (14.3%)

Normal/borderline ‡

5 (7.1%)

*Range in parentheses and interquartile range (25th and 75th percentile) in brackets. † Among 126 patients with follow-up. ‡ High-frequency loss in 6 of 8 patients with unilateral EVA and 1 of 5 with bilateral EVA. EVA ¼ enlarged vestibular aqueduct.

14–130], respectively; P ¼ .9). This finding was consistent with the final audiometric assessment of ipsilateral and contralateral PTA values (52.5 [range, 7.5–121.25] vs. 50 [range, 12.5–130], respectively; P ¼ . 6). The severity of hearing loss at the initial assessment was categorized as borderline to mild in 37.4% of ears with hearing loss, moderate to severe in 45.3% of affected ears, and profound in the remaining 17.3% of affected ears. There was no difference in hearing loss severity between patients with bilateral vs. unilateral EVA ( P ¼ .48; Table I). There was no statistical relationship ( P ¼ .21) between levels of hearing loss severity and whether the loss was ipsilateral or contralateral to the EVA at ei- ther the initial or the final audiometric assessment. Vestibular Aqueduct Measurements Hearing loss and vestibular aqueduct phenotypes were compared regarding temporal bone measurements at the midpoint and the operculum (Table II). No significant differences were found in the temporal bone measure- ments between the ears of patients with unilateral EVA and ipsilateral hearing loss and all ears with EVA and normal hearing ( P ¼ .4). Operculum measurements were significantly greater in the ears of patients with bilateral EVA than in those with unilateral EVA ( P ¼ .025).

months; P ¼ .02) and a bilateral EVA group (75.0 vs. 48 months; range, 38–102.5 vs. 0–208 months; P ¼ .008).

Hearing Loss Severity The median PTAs at the initial and final audiometric assessments for all ears with hearing loss (n ¼ 243) were 50 (range, 10–130) and 57 (range, 7.5–122.5), respectively. Nine ears had an isolated HFPTA (median HFPTA was 35.8; range, 20–57.5). Patients with hearing loss and uni- lateral EVA had slightly better hearing than those with bilateral EVA, although this difference was not statisti- cally significant (47.5 [range, 10–130] vs. 55.6 [range, 12– 130], respectively; P ¼ .075). This finding was consistent when analyzing the better-hearing ear in both groups. At initial audiometric assessment, there was no statistical difference between the median PTA of patients with unilateral hearing loss (n ¼ 45) and the better-hear- ing ear of patients with bilateral loss (n ¼ 99; 48.75 vs. 41.25; P ¼ .4). This finding was consistent when our cohort was stratified into unilateral and bilateral EVA groups. Among the 74 patients with unilateral EVA (n ¼ 114 ears with hearing loss), there was no significant dif- ference in the median PTA at the initial audiometric assessment between ipsilateral ears (n ¼ 73) and contra- lateral ears (n ¼ 41; 47.5 [range, 10–130] vs. 41 [range,

TABLE II. Vestibular Measurements of Ears With Hearing Loss.

Unilateral EVA and Ipsilateral Hearing Loss, 73 Ears †

Non-EVA Ears and Contralateral Hearing Loss, 41 Ears

Bilateral EVA, 129 Ears* 1.7 (0.1–4.1) 2.7 (0.68–7.6)

EVA Ears With Normal Hearing, 12 Ears ‡

Midpoint, mm (range) Operculum, mm (range)

1.5 (0.1–3.5) 2.3 (0.1–7.5)

0.41 (0.1–0.9) 1.37 (0.1–1.9)

1.25 (0.1–3.8) 2.0 (0.8–4.2)

* versus †: midpoint P ¼ .069, operculum P ¼ .025 † versus ‡: midpoint P ¼ .4, operculum P ¼ .4. EVA ¼ enlarged vestibular aqueduct.

Laryngoscope 123: June 2013

Greinwald et al.: Unilateral Enlarged Vestibular Aqueduct

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