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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,

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).

TABLE I.

Data Pertaining to Patients With EVA.

Characteristic

Unilateral, n

¼

74

Bilateral, n

¼

70

P

Male

38 (51%)

29 (41%)

.26

Age at identification of hearing loss, mo*

70 (0–324.5) [39.5–106]

54.5 (0–208) [32.5–69.5]

.01

Follow-up time, mo

32.8 (0.5–164) [13.5–69.5]

56.3 (0.5–812.5) [33.8–76.8]

.02

3 months of follow-up

55 (74.3%)

61 (87.1)

.05

Unilateral hearing loss at initial assessment

34 (46.0%)

11 (15.7%)

<

.0001

Pure tone average in better-hearing ear, dB

37.9 (10–130) [27.5–65]

50 (6.25–130) [32.5–70]

.19

Hearing loss severity in better-hearing ear

.48

Mild

30 (40.5%)

21 (30.0%)

Moderate

11 (14.9%)

15 (21.4%)

Moderately severe

11 (14.9%)

12 (17.1%)

Severe

3 (4.1%)

7 (10%)

Profound

11 (14.9%)

10 (14.3%)

Normal/borderline

8 (10.8%)

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.

TABLE II.

Vestibular Measurements of Ears With Hearing Loss.

Bilateral EVA,

129 Ears*

Unilateral EVA and

Ipsilateral Hearing

Loss, 73 Ears

Non-EVA Ears and

Contralateral Hearing

Loss, 41 Ears

EVA Ears With Normal

Hearing, 12 Ears

Midpoint, mm (range)

1.7 (0.1–4.1)

1.5 (0.1–3.5)

0.41 (0.1–0.9)

1.25 (0.1–3.8)

Operculum, mm (range)

2.7 (0.68–7.6)

2.3 (0.1–7.5)

1.37 (0.1–1.9)

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

136