Hearing Loss in Wegener’s Granulomatosis

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					Otology & Neurotology
25:833–837 © 2004, Otology & Neurotology, Inc.

                         Hearing Loss in Wegener’s Granulomatosis

                          *Sivasanker Bakthavachalam, *Mark S. Driver, *Clarke Cox,
                       *Jeffrey H. Spiegel, *Kenneth M. Grundfast, and †‡Peter A. Merkel

                   *Department of Otolaryngology–Head and Neck Surgery and the Sections of †Rheumatology and
                      ‡Clinical Epidemiology, Department of Medicine, Boston University School of Medicine,
                                                  Boston, Massachusetts, U.S.A.

Objective: To describe the frequency, type, and clinical course                  Seven patients had hearing loss requiring amplification. Five of
of hearing loss in Wegener’s granulomatosis and assess hearing                   35 (14%) patients had established hearing loss months to years
loss as an indicator of disease activity.                                        before diagnosis of Wegener’s granulomatosis. Hearing loss
Study Design, Setting, and Patients: Retrospective cohort re-                    occurred both on initial presentation and with disease relapse.
view of all patients with Wegener’s granulomatosis seen in 1                     The rates of conductive hearing loss (38%) and sensorineural
year at an academic medical center.                                              hearing loss (31%) were also high in the subset of patients 65
Main Outcome Measures: Hearing loss documented by pure-                          years of age or younger and without history of noise exposure.
tone audiogram.
                                                                                 Conclusions: Both sensorineural hearing loss and conductive
Results: Thirty-six patients were included in the analysis: 20
                                                                                 hearing loss are common in Wegener’s granulomatosis,
men and 16 women, with a mean age of 55.5 years (range,
                                                                                 may result in significant morbidity, and may precede the diag-
22–87 yr); 30 (83%) were antineutrophil cytoplasmic autoan-
                                                                                 nosis of Wegener’s granulomatosis by years. Both types of
tibodies–positive, and the mean disease duration was 47
                                                                                 hearing loss in patients with Wegener’s granulomatosis may be
months (range, 2–196 mo). Twenty patients (56%) had docu-
                                                                                 used as an indicator of disease. These data suggest that it may
mented hearing loss: there were 17 (47%) cases of sensorineu-
                                                                                 be appropriate to obtain screening audiograms in all patients
ral hearing loss and 12 (33%) cases of conductive hearing loss.
                                                                                 with newly diagnosed or relapsing Wegener’s granulomatosis.
Seven of 12 cases of conductive hearing loss improved with
                                                                                 Key Words: Audiograms—Hearing loss—Vasculitis—
immunosuppressive treatment of Wegener’s granulomatosis, 2
                                                                                 Wegener’s granulomatosis.
worsened, and 3 remained stable. Of 17 cases of sensorineural
hearing loss, 3 improved, 4 worsened, and 10 remained stable.                    Otol Neurotol 25:833–837, 2004.

   Head and neck complaints are extremely common                                 loss (CHL) (4). The reported incidence of SNHL in WG
among patients with Wegener’s granulomatosis (WG),                               varies, with recent reports citing rates of 2.8% (5) and
and are often present early in the disease course (1–3).                         13% (6). SNHL is especially significant, because cranial
This places the otolaryngologist in a pivotal role both in                       nerve impairment suggests a neurodegenerative process
diagnosis of WG and in the detection of exacerbations.                           that might warrant aggressive therapy (5). Furthermore,
Although the nasal, tracheal, and middle ear findings of                         SNHL is believed to be largely irreversible, therefore
WG are well recognized by most otolaryngologists, sen-                           potentially adding to the patient’s cumulative disability
sorineural hearing loss (SNHL) is less appreciated.                              (7). Interpreting audiologic data in patients with WG can
   Approximately 50% of WG patients have otologic                                be problematic, however. For example, it is difficult to
findings, including both SNHL and conductive hearing                             determine whether SNHL in a patient with WG is attrib-
                                                                                 utable to the autoimmune disorder itself or to other
                                                                                 causes such as presbycusis or noise-induced hearing loss
   Address correspondence and reprint requests to Peter A. Merkel,
M.D., M.P.H., Vasculitis Center, E5, Boston University School of                 (NIHL). In addition, the usefulness of SNHL in guiding
Medicine, 715 Albany Street, Boston, MA 02118, U.S.A.; Email:                    treatment decisions is unclear. This retrospective study                                                                   of all patients with WG evaluated in 1 year at an aca-
   Supported in part by research grants from The National Institute of           demic medical center was undertaken to assess the sig-
Arthritis, Musculoskeletal and Skin Diseases. Dr. Merkel is supported
in part by a Mid-Career Development Award in Clinical Investigation
                                                                                 nificance and clinical spectrum of hearing loss in WG.
(National Institutes of Health–National Institute of Arthritis, Musculo-
skeletal and Skin Diseases Grant K24 AR2224-01A1); the National                               PATIENTS AND METHODS
Center for Research Resources; Boston University General Clinical
Research Center Program Grant (National Institutes of Health–National                        Study patient eligibility criteria
Center for Research Resources Grant MO 1RR00533); and the Boston                   Patients with WG were cared for by one rheumatologist (P.
University Wegener’s Granulomatosis Research Fund.                               A. M.) through the Vasculitis Center at Boston University

834                                                  S. BAKTHAVACHALAM ET AL.

Medical Center during 2002. The Boston University Vasculitis                 bilized,” or “worsened”), were also recorded. All patients un-
Center is a tertiary referral center for inflammatory vasculitis             derwent testing for ANCA by both immunofluorescence and
and serves patients from the entire United Sates. The study was              ELISA techniques. Patients were considered ANCA-positive if
approved by the Boston University Institutional Review Board.                at any time they had positive tests of cytoplasmic pattern (C-
Thirty-seven patients were initially considered eligible for the             ANCA) coupled with antibodies to proteinase 3 (anti–PR-3) by
study. However, one patient died during the study period, and                ELISA.
data from that patient were not included in this analysis. Two
patients with mixed losses had a history of prolonged exposure                                             Statistics
to loud noise, and they were counted as having conductive loss,                 Group comparisons were made with Student’s t test for con-
but were excluded from analysis of sensorineural deafness in an              tinuous variables and Fisher’s exact test for categorical vari-
attempt to exclude non-WG causes of hearing loss. Diagnosis                  ables. All tests were two-tailed with a significance level ( )
of WG was based on a modification of the 1990 American                       of 0.05.
College of Rheumatology classification criteria for WG with a                                          Data presentation
fifth criterion of antineutrophil cytoplasmic autoantibodies                    In reporting the patient baseline data, disease duration was
(ANCA) positivity for by measurement of antibodies to pro-                   defined as the time from diagnosis of WG to the end of the
teinase-3 (PR-3) using enzyme-linked immunosorbent assay                     study period (December 31, 2002). Patients with MHL were
(ELISA) (8).                                                                 counted as having one case each of SNHL and CHL, leading to
                         Hearing loss                                        the number of cases of hearing loss exceeding the number of
   “Hearing loss” was defined using the criteria set forth by the            patients with hearing loss. Hearing loss was classified as a head
American National Standard Specification for Audiometers (9)                 and neck manifestation. “Severe” disease manifestations are
as follows: CHL was defined as the presence of an air-bone gap               defined as those that impart risk of permanent damage to an
of greater than 10 dB. SNHL was defined as both air and bone                 organ, necessitating cyclophosphamide therapy (5,8). Ex-
conduction below 15 dB with no significant air-bone gap (>10                 amples of severe manifestations include alveolar hemorrhage,
dB). Mixed hearing loss (MHL) was defined as both SNHL and                   renal disease, scleritis, gangrene, nervous system involvement,
CHL components on the audiogram. To ensure that CHL and                      or SNHL. Statistical analysis was performed on the entire co-
SNHL were treated as separate entities, patients with MHL                    hort as well as a subset of patients aged 65 years or younger
were included by assigning then to both sensorineural and con-               without a history of noise exposure. This subset was identified
ductive hearing loss categories. All audiograms were reviewed                as that most likely to have hearing loss unrelated to NIHL or
and interpreted by three authors of this article who were trained            presbycusis, because the prevalence of age-induced hearing
in interpreting audiograms: a medical student (S. B.), an oto-               loss dramatically increases above the age of 65 (11). Audio-
laryngology resident (M. S. D.), and the Director of the Divi-               gram series were analyzed to define progression as “improve-
sion of Audiology (C. C.) in the Department of Otolaryngolo-                 ment,” “no change,” or “worsening.”
gy–Head and Neck Surgery at Boston Medical Center.
                        Data collection
   Data obtained from patients’ medical records included de-                    Table 1 displays baseline data of all patients. For
mographic information, clinical detail, and laboratory studies.              analysis of air conduction, 36 patients, 20 men and 16
Additional audiograms were obtained from outside institutions                women, diagnosed with WG were included. Thirty-four
when available. Data were collected beginning with patients’                 were included in the group assessed for sensorineural
initial presentations of disease at Boston Medical Center or an              loss (18 men and 16 women). Ages ranged from 22 to 87
outside institution and for each documented WG flare. Mani-                  years, with a mean of 55.5 years. The mean disease du-
festations of disease other than hearing loss were tallied ac-               ration was 35.7 months. Thirty patients (83%) tested
cording to the Birmingham Vasculitis Activity Score for
Wegener’s Granulomatosis (BVAS/WG) (10). The major cat-
                                                                             positive for antibodies to the PR-3 antigen. Twenty-three
egories in the BVAS/WG evaluation form are organized by                      patients underwent at least one audiogram and 20 pa-
major organ systems. A “flare” was defined as any recurrence                 tients (56%) had a documented hearing loss on at least
of signs or symptoms of disease after a period of remission                  one audiogram.
(8,10). The first flare represented initial presentation with WG.               Allowing for 9 patients who had mixed losses, the
Treatment dates and responses to treatment (“improved,” “sta-                incidence of SNHL and CHL was 17 (47%) and 12

                                                    TABLE 1. Data on all study patients
                                 All (n       36)     All SNHL (n      17)      All CHL (n       12)      Any HL (n       20)   No HL (n     16)
Mean age (yr) (range)            55.5 (22–87)            62 (24–87)                 58 (39–81)               60 (24–87)           48 (22–65)
Men (%)                           20 (56)                19 (59)                     5 (41.7)                10 (50)              10 (63)
Women (%)                         16 (44)                 8 (47)                     7 (58.3)                10 (50)               6 (38)
Disease duration (mo) (range)     36 (3–120)             35 (12–108)                31 (6–84)                33 (6–108)           39 (3–120)
ANCA/anti-PR-3 (%)                30 (83)                14 (77)                     9 (75)                  16 (80)              14 (88)
Medication usage (ever)
  Glucocorticoids (%)              36 (100)              18 (100)                   12 (100)                 20 (100)             16 (100)
  Methotrexate (%)                 26 (72)               13 (72)                     9 (75)                  15 (75)              11 (69)
  Azathioprine (%)                  8 (22)                5 (27)                     4 (33)                   5 (23)               3 (19)
  Cyclophosphamide (%)             32 (89)               16 (88)                    11 (91)                  17 (85)              15 (94)

  HL, hearing loss.

Otology & Neurotology, Vol. 25, No. 5, 2004
                                   HEARING LOSS IN WEGENER’S GRANULOMATOSIS                                                                835

(33%), respectively. The patients with hearing loss were                  one had fluctuating hearing loss throughout his disease
significantly older than those without hearing loss (60                   course that never fully resolved, and the other two had
versus 48 years old, p       0.014). Two patients had au-                 stable hearing loss.
diometric patterns consistent with NIHL (characterized                       Table 4 depicts the changes in hearing loss in all cases.
by a deficit at 4,000 Hz), and two of these had a history                 Although there appears to be a trend indicating cases
of noise exposure. Seven had audiograms suggestive of                     of CHL showing improvement more often than cases
presbycusis (characterized by high-frequency loss). Sev-                  of SNHL (58% versus 18%), this difference was not
enteen of 20 patients (85%) with hearing loss tested posi-                statistically significant. All of these patients except one
tive for C-ANCA/anti–PR-3 antibodies. Sixteen of 20                       with CHL had been treated with cyclophosphamide.
patients (80%) had bilateral hearing loss. Seven patients                 Fourteen of 17 (82%) cases of SNHL were unremitting
used hearing amplification. Five of 36 (14%) patients                     despite therapy.
had established hearing loss months to years before di-                      In analyzing the subgroup of patients younger than 65
agnosis of WG. Hearing loss occurred both on initial                      years old with no noise exposure (Table 5), similar trends
presentation and with disease relapse. Comparative sta-                   in hearing outcome exist compared with the entire group.
tistics between the subgroup of patients with SNHL and                    There are fewer cases of SNHL, as expected, but still a
those with CHL are not possible because patients with                     low incidence of improvement in SNHL (13%). Al-
MHL, of which there were many, contribute data to                         though more cases of CHL showed improvement (40%)
both groups.                                                              than SNHL (13%), this difference was also not statisti-
   Table 2 displays data for patients aged 65 years or                    cally significant.
younger with no known history of noise exposure. In this                     All eight of the patients who presented with hearing
subgroup, cases of SNHL are highly likely to be second-                   loss as their initial complaint had SNHL at some point in
ary to WG alone. This restriction yielded 26 patients,                    their course. Five of the eight had stable deficits, whereas
with a mean age of 45 years (range, 22–63 yr). Eleven of                  two worsened and one improved. Five patients also had
26 patients (42%) in the younger group had hearing loss,                  CHL and, of these, two improved, two worsened, and
compared with 9 of 10 patients (90%) in the group older                   one remained stable. Of these eight patients who initially
than 65 (p     0.022). SNHL and CHL prevalences were                      complained of hearing loss, four were younger than 60
similar (31% versus 38%) in the younger group. Of the                     years of age, with no known history of noise exposure.
10 patients who were older than 65 years old, 9 had                       Three of these four patients had hearing loss that pro-
SNHL (90%) and 2 had CHL (20%).                                           gressed and one had stable hearing loss. None of the four
   Table 3 shows the nonotologic manifestations of WG                     patients’ hearing improved.
in the entire cohort. These are organized by major sys-
tems according to the BVAS/WG categorization (10).                                                 DISCUSSION
Thirty-three patients (92%) had a nonotologic head and
neck manifestation of the disease, and many had pulmo-                       Although the nasal, upper airway, pulmonary, and re-
nary (69%) and renal manifestations (67%). SNHL was                       nal manifestations of WG are well known, the otologic
associated with renal disease in 67% of cases and with                    ones are less appreciated (12–15). In the current study,
CHL in 42% of cases. Of 29 patients classified as having                  20 (56%) patients with WG had some form of hearing
severe disease, 17 (59%) had SNHL and 16 (55%) of the                     loss, which compares to the upper limit of the previously
patients had bilateral hearing loss. Seven patients had                   reported prevalences [19–61% (16), 47% (13)]. The
SNHL as their only severe manifestation and five had                      prevalence of SNHL in our patients with WG (47%) is
bilateral hearing loss. Four of the seven patients were put               higher than previously reported (2.8–43%) (4–6). This is
on cyclophosphamide for their severe WG. Two of these                     possibly explained by the comprehensive review of
had worsened hearing, one remained stable, and one had                    medical records for evidence of hearing loss used in the
hearing that improved. Subsequently, of the remaining                     current study.
three patients who were not given cyclophosphamide,                          Hearing loss can be an early indicator of WG. Sixteen

                      TABLE 2. Data on study patients aged       65 years and without a history of noise exposure
                                 All (n      26)   All SNHL (n       8)     All CHL (n      10)      Any HL (n        11)   No HL (n       15)
Mean age (yr) (range)            45.0 (22–63)         53 (24–63)               45.0 (24–55)              47 (24–63)            44 (22–63)
Males (%)                          14 (54)              4 (57)                    5 (50)                  5 (45)                9 (60)
Females (%)                        12 (46)              3 (38)                    5 (50)                  6 (55)                6 (40)
Disease duration (mo) (range)      40 (12–119)       43.0 (24–186)               41 (6–186)              39 (6–84)             42 (4–120)
ANCA/anti-PR-3 (%)                 20 (77)              4 (50)                    6 (60)                  7 (64)               14 (93)
Medication usage (ever)
  Glucocorticoids (%)             26 (100)             8 (100)                   10 (100)                 8 (100)              15 (100)
  Methotrexate (%)                16 (62)              6 (75)                     8 (80)                  9 (82)                8 (53)
  Azathioprine (%)                 5 (26)              2 (25)                     2 (20)                  3 (27)                1 (0.07)
  Cyclophosphamide (%)            21 (81)              5 (63)                     6 (60)                  9 (82)               12 (80)

  HL, hearing loss.

                                                                                                  Otology & Neurotology, Vol. 25, No. 5, 2004
836                                                S. BAKTHAVACHALAM ET AL.

                              TABLE 3. Manifestations of Wegener’s granulomatosis in study group
                            All (n   36)      SNHL (n      17)    CHL (n    12)   Any HL (n       20)   No HL (n       16)   Bilateral HL (n     16)
General                       27 (75.0)         15 (83.3)          10 (83.3)         16 (80.0)            11 (68.8)                12 (75.0)
Cutaneous (%)                 10 (27.8)          2 (11.1)           1 (8.33)          2 (10.0)             8 (50.0)                 1 (6.25)
Mucous membrane/eye (%)       19 (52.8)          8 (42.1)           6 (50.0)         10 (50.0)             9 (56.2)                 7 (43.7)
Head and neck (%)             33 (91.7)         18 (100.0)         12 (100.0)        20 (100.0)           11 (68.8)                16 (100.0)
Cardiovascular (%)             1 (2.8)           1 (5.6)            1 (8.3)           1 (5.00)             0 (100.0)                1 (6.3)
Gastrointestinal (%)           0 (0.00)          0 (0.00)           0 (0.00)          0 (0.00)             0 (100.0)                0 (0.00)
Pulmonary (%)                 25 (69.4)         12 (66.7)          10 (83.3)         15 (75.0)            10 (62.5)                10 (62.5)
Renal (%)                     24 (66.7)         12 (66.7)           5 (41.7)         12 (60.0)            12 (75.0)                11 (68.8)
Neurologic (%)                 6 (16.7)          5 (27.8)           4 (33.3)          5 (25.0)             1 (6.3)                  4 (25.0)

patients had hearing loss at presentation, and in eight it                  a screening audiogram in all patients newly diagnosed
was the primary complaint. The most common otologic                         with WG and subsequent serial audiograms for those
manifestations of WG are secondary to granuloma                             with hearing loss and/or hearing complaints.
formation within the middle ear, eustachian tube, or                           Conductive hearing loss has long been known to occur
nasopharynx. These pathologic changes can result in                         when granulomatous disease involves the middle ear, but
CHL, serous otitis, chronic otorrhea, and otomastoiditis,                   the extent to which hearing can be improved for patients
a painful, draining ear, sometimes associated with facial                   with conductive hearing loss from WG is unclear. Cer-
nerve paralysis. The evaluation of new hearing loss,                        tainly, if a patient with WG has only a middle ear effu-
either CHL or SNHL, in an adult should, therefore, in-                      sion as the reason for conductive hearing loss in an af-
clude audiometry, followed by upper airway endoscopy                        fected ear, then removing the fluid from the middle ear
in the presence of serous otitis to search for inflamma-                    and inserting a ventilating tube might result in sustained
tory lesions.                                                               hearing improvement. However, when the patient with
   Hearing loss in WG can result in permanent disability                    WG has middle ear or mastoid involvement, usually as-
as evidenced by the use of amplification in seven patients                  sociated with a disease flare, then surgery without con-
(19%) in our cohort. Several other patients would have                      comitant remission of the extratemporal bone manifesta-
undoubtedly benefited from hearing aids, and many pa-                       tions tends to be unsuccessful in achieving long-term
tients reported deficits affecting their daily activities.                  improvement in hearing.
   SNHL is a significant finding in WG, and its detection                      WG-related audiometric patterns have been described
is therefore important for appropriate patient manage-                      as typically flat, sometimes with additional high tone
ment. The presence of SNHL can be a warning of severe                       losses (4), findings that are sometimes difficult to distin-
WG and is thought to necessitate initial treatment with                     guish from those caused by noise exposure or age. In an
cyclophosphamide rather than either methotrexate or                         attempt to minimize the influence of these confounders,
azathioprine. Furthermore, SNHL is significant in that it                   we list the audiometric findings for those 26 patients who
can be associated with other severe manifestations. Al-                     were younger than 65 years old and with no history of
though 7 patients had SNHL as their only severe mani-                       noise exposure. This group had a SNHL incidence of
festation, 11 of 22 (50%) of the patients with severe                       31%, an exceptional rate when compared with the 8%
disease had SNHL associated with other severe disease                       reported incidence of SNHL in laborers who comply
manifestations. For example, 12 patients (67%) with                         fully with workplace recommendations (17). Among the
SNHL also had renal disease. Of 29 patients with other                      study patients older than 65 years, the prevalence of
manifestations of severe disease, 17 (59%) had SNHL.                        SNHL (90%) was higher than would be expected to be
This emphasizes the importance of SNHL as a potential                       caused by presbycusis alone. The accepted prevalence of
indicator of severe disease. Finally, fewer patients with                   presbycusis is 25% in those aged 65 to 75 years and 40
SNHL than with CHL had improvement, but these find-                         to 50% in those older than 75 years (17). Therefore, these
ings were not statistically significant in this small sub-                  data further support the findings that SNHL in patients
sample and can only be considered a possible trend. To-                     with WG is likely attributable to the vasculitis and
gether, these findings provide arguments for performing                     should not readily be dismissed as age- or noise-related.

  TABLE 4. Hearing outcome for all study patients with                      TABLE 5. Hearing outcome in study patients with hearing
                   hearing loss                                             loss aged 65 years and without a history of noise exposure
                                SNHL                      CHL                                                SNHL                        CHL
                              (n   17)               (n     12)                                             (n  8)                  (n     10)
      Improved (%)              3 (18)                  7 (58)                    Improved (%)               1 (13)                   4 (40)
      Stable (%)               10 (59)                  3 (25)                    Stable (%)                 4 (50)                   4 (40)
      Worse (%)                 4 (24)                  2 (17)                    Worse (%)                  3 (38)                   2 (20)

Otology & Neurotology, Vol. 25, No. 5, 2004
                                 HEARING LOSS IN WEGENER’S GRANULOMATOSIS                                                      837

   This study has several strengths. The cohort of 36           screening audiograms should be performed in all patients
patients is the largest reported with detailed audiometric      with WG; (2) all patients with documented hearing loss
data. The patients were comprehensively followed up             should have follow-up audiometric assessment; and (3)
with clinical data collected with the most current meth-        audiometric data should be considered when making
odology. Furthermore, multiple audiograms were ob-              treatment decisions. Further research focusing on dis-
tained for most patients, and these were reviewed by            cerning the cause and developing more effective treat-
multiple assessors using a uniform system of interpretation.    ment of hearing loss in WG is needed.
   This study also has certain limitations. Although we
found a high incidence of both CHL and SNHL among                                      REFERENCES
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                                                                                       Otology & Neurotology, Vol. 25, No. 5, 2004

Tags: hearing, loss
Description: To describe the frequency, type, and clinical course of hearing loss in Wegener’s granulomatosis and assess hearing loss as an indicator of disease activity.