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ORIGINAL ARTICLE
Korean J Audiol 2011;15:8-13 pISSN 2092-9862 / eISSN 2093-3797

Clinical and Audiologic Characteristics


of Acute Low-Tone Sensorineural Hearing Loss:
Therapeutic Response and Prognosis
Hyeog Gi Choi, Kyoung Ho Park, Jae-Hyun Seo,
Dong Kee Kim, Sang Won Yeo and Shi-Nae Park
Department of Otolaryngology-Head & Neck Surgery, The Catholic University of Korea College of Medicine, Seoul, Korea

Objectives: This study was designed to observe the clinical and audiologic characteristics of
patients diagnosed as low tone hearing loss (LHL) and to evaluate the treatment response and
prognosis. Subjects and Methods: A retrospective chart review of 61 patients who were di-
agnosed with LHL was performed. Result of various audiologic studies and therapeutic re-
sponses of medication have been evaluated. Data were statistically analyzed with variables
such as age, sex, interval between onset and treatment, initial hearing levels, findings of elec-
Received February 10, 2011 trocochleargraphy, and initial concomitant symptoms according to their treatment response.
Revised March 31, 2011 Results: Frequent chief complaints of LHL were tinnitus and earfullness. Tinnitus was usually
Accepted April 1, 2011 matched at lower frequencies. Treatment with low-dose steroids, diuretics, and betahistine
Address for correspondence produced a high response rate of 79.4%. Complete response occurred in 49 of the 68 ears
Shi-Nae Park, MD, PhD (72.1%) and partial response occurred in five of the 68 ears (7.4%). Fourteen of the 68 ears
Department of Otolaryngology-Head (20.6%) were in the non-responder group. Patients treated within 7 days after the onset of symp-
& Neck Surgery, The Catholic toms all showed complete response. Statistically significant prognostic factors affecting treat-
University of Korea College of
ment response were age and intervals between onset and treatment (p<0.05). Conclusion:
Medicine, 505 Banpo-dong,
Patients with acutely onset tinnitus or ear fullness should be carefully evaluated and promptly
Seocho-gu, Seoul 137-701, Korea
Tel +82-2-2258-6215 treated under the diagnosis of LHL. Korean J Audiol 2011;15:8-13
Fax +82-2-595-1354 KEY WORDS: Low-tone hearing loss · Sensorineural · Prognosis.
E-mail snparkmd@catholic.ac.kr

Introduction been established. Even though LHL might be associated with


sudden deafness (SD) or Meniere’s disease (MD), it also
Patients who complain of acute onset low-tone hearing loss could be an entirely different clinical disease entity. To un-
(LHL) are relatively commonly encountered in daily clinical derstand the disease entity of LHL more precisely and to
practice. They may frequently complain of diverse symptoms stimulate further interest of this little known disease, we in-
such as tinnitus, earfullness, autophony, and hearing impair- vestigated the clinical and audiologic characteristics of 61
ment. The pure tone audiogram of these patients reveals sen- patients diagnosed with LHL and evaluated the responses to
sorineuronal hearing loss restricted to the low frequency range. treatments that included low-dose steroids, hydrochlorothia-
Abe presented the first detailed clinical review of patients with zide, betahistine mesylate, ginko biloba extract, and nicergo-
acute LHL.1 Studies of the pathophysiologic characteristics line. Literature review concerning LHL is presented as well.
of acute LHL using the glycerol test and electrocochleogram
led to the hypothesis that LHL might be caused by endolym- Subjects and Methods
phatic hydrops.2,3 Another hypothesis posited that the possi-
ble pathogenesis of LHL might be endolymphatic hydrops Study design, treatment, and criteria
underlying autonomic imbalance and insufficient blood cir- This study was designed as a retrospective chart review of
culation.4 But a definite pathophysiology of LHL has not yet 61 patients who visited our tertiary hospital between July

8 Copyright © 2011 The Korean Audiological Society


Choi HG, et al.

2001 and July 2006. It has been approved by the institutional (ECoG) were initially performed.
review board of Seoul St. Mary’s hospital (KC09RISI0287).
They were diagnosed as LHL according to the following di- Statistical analysis
agnostic criteria adopted from an established definition 5: 1) Data were statistically analyzed with variables such as age,
hearing loss is purely sensorineural in nature with normal tym- sex, interval between onset and treatment, initial hearing lev-
panogram (type A); 2) average hearing threshold at the three els, findings of ECoG, and initial concomitant symptoms be-
low frequencies (125, 250 and 500 Hz) is 30 dB or more, and tween groups. Significance was determined using t-test, Chi
the average hearing threshold at the three higher frequencies square test, and correlation analysis. The SPSS version 15.0
(2,4, and 8 KHZ) is 20 dB or less; and, 3) the patient has no software was used for analyses. A level of significance 0 .05
known etiologic factors that may cause low-tone sensorineu- was indicative of significance.
ronal hearing loss as detected by routine clinical and radiolog-
ic examinations. The patients received their medical treatments Results
at an outpatient clinic. Treatments consisted of prednisolone
(SOLONDO®, Yuhan Medica, Gunpo, Korea; 30 mg/day for A total of 61 LHL patients were evaluated. Their ages ranged
7 days) and the other drugs; hydrochlorothiazide (Dichlozid®, from 18-83 years with a mean age of 43.2±15.2 years.
Yuhan Medica; 50 mg/day), betahistine mesylate (Meniace®, Follow-up period was 3-12 months. The patients were main-
Dong Koo Pharmaceutical. Seoul, Korea; 30 mg/day), and ly female (n=43). The peak age of incidence was in the twen-
microcirculation enhancers including ginko biloba extract ties for female and thirties for male (Fig. 1). The affected ear
(GINEXIN-F®, SK Chemical Life Science, Gyeonggi-Do, Ko-
rea; 160 mg/day) and nicergoline (Sermion®, Il Dong Pharma- 18
Male Female
ceutical, Ansung, Korea; 30 mg/day). 16

Combination therapy were continued for seven days and 14


Number of the patients (%)

then switched into the medication without prednisolone with 12


the regular follow up of their hearing at 1-2 week interval 10
until they showed improvement of hearing. The patients whose 8
hearings were improved within three months were placed 6
into the responder group and the others whose hearings did 4
not show any improvement within three months were placed 2
into the non-responder group. The responder group was sub-
0
divided into complete responder group who had normaliza- 10-19 20-29 30-39 40-49 40-59 60-69 70-79 80-89
Years of age
tion of hearing and partial responder group who showed 10
dB or more improvement at the three lower frequencies but Fig. 1. Age distribution of the patients with low-tone sensorineural
hearing loss. The peak age of incidence was in the twenties for fe-
did not reach normal hearing level at the end of treatment. To male and the thirties for male.
analyze the treatment response according to time interval be-
tween onset of disease and treatment, we grouped the patients 45
41.2
39.7
as group 1 who received the treatment within 7 days of the on- 40
Percentage of the patients (%)

set of disease; group 2, whose time interval between onset and 35

treatment were between 7 days and 1 month and; group 3 who 30


had received the treatment more than 1month after the onset 25
of disease. 20

15
Audiologic study 8.8
10.3
10
Pure-tone air- and bone-conduction thresholds were mea-
5
sured at octave frequencies between 125 and 8,000 Hz in a
0
soundproof chamber. Audiometry was performed at the time Hearing Tinnitus Earfullness Autophony
of first examination, and followed up at the first week, second impairment

week and 1 month. Long-term follow-up was done at 1–3 month Fig. 2. Percentage of chief complaints in the patients with low-tone
sensorineural hearing loss. Two of the most frequent chief com-
intervals. Other audiologic studies including tympanogram, plaints were tinnitus and ear fullness and other chief complaints
speech audiometry, tinnitogram, and electrocochleargraphy were autophony and hearing impairment.

www.audiology.or.kr 9
Clinical Characteristics of Low-Tone Hearing Loss

was the right ear in 32 cases, left ear in 28 cases and bilateral in shown in Table 1. Only one patient showed an increment in
four cases, for a total of 68 ears. One patient had multiple re- the summating potential/action potential (SP/AP) ratio of
currences (four times) after treatment. Two patients were di- ECoG (>0.4); the patient met the criteria of MD and was sub-
agnosed with MD during the follow-up period. The time in- sequently diagnosed with MD during the follow up period, 7
terval between the onset of ear symptoms and diagnosis months after initial symptom of hearing loss.
ranged from 1-360 days. Four patients visited our clinic quite Treatment with low-dose steroids, diuretics, and betahis-
late after the onset of their symptoms which made the diag- tine showed the relatively high response rate (79.4%, 54 of
nosis range so wide, but the average time interval between 68 ears)(Table 2). Complete responders comprised 49 of the
onset of ear symptoms and our diagnosis of LHL in this 68 ears (72.1 %). Partial responders constituted five of 68 ears
study was 32.0 days. An interval of ≤1 month was observed (7.4%). Fourteen of 68 ears cases (20.6 %) were allocated to
in 79.1% of the cases and ≥1 month or more in 20.9 % of the non-responder group. Clinical factors affecting the results
the cases. Frequent chief complaints were tinnitus (41.2%) of therapeutic response were investigated between the re-
and earfullness (39.7%), followed by autophony (10.3%) and sponders and non-responders (Table 3). Statistically signifi-
hearing impairment (8.8%) (Fig. 2). Tinnitus was usually cant prognostic factors affecting therapeutic response to LHL
matched at lower frequencies (Table 1). Initial mean air con- were age and time interval between onset and treatment (p<
duction hearing levels of the patients with LHL were 44.1± 0.05). Age showed a positive correlation with the state of fi-
6.0 dB at 125 Hz, 46±6.0 dB at 250 Hz, and 35.9±6.8 dB at nal hearing loss at each frequency (125, 250, and 500 Hz, r=
500 Hz (Fig. 3). The results of other audiologic studies such 0.43, 0.33, and 0.27 respectively, p<0.05) and the time interval
as speech discrimination score, ECoG, and tinnitogram are between onset and treatment was positively correlated with
the state of final hearing loss at 125 and 250 Hz (r= 0.51 and
Table 1. Results of audiologic studies; speech discrimination score, r=0.43, p<0.05)(Table 4). Group 1, 2 and 3 showed 100%,
electrocochleography, and tinnitogram
72.3% and 53.3% response rate respectively, which was sig-
Audiologic study Results
nificantly different prognosis according to time interval be-
Speech discrimination score (n=64) 97.6±7.0% (72-100)
tween onset of disease and treatment (p<0.01, Table 5).
SP/AP ratio 0.25±0.13
Electrocochleography (n=18)
SP/AP>0.04 1/18
Pitch Number of patients Discussion
125 Hz 2
250 Hz 9 Low frequency sudden sensorineural hearing loss is a com-
500 Hz 2 monly encountered finding in otology practice. Although
Tinnitogram (n=19)
1,000 Hz 2 there have been many studies regarding the pathophysiology
3,000 Hz 2 of LHL, the exact mechanism of LHL has remained elusive,
4,000 Hz 2 and no international consensus on the general treatment of
8,000 Hz 2 LHL has been reached. Some similarities of LHL to SD or MD
such as an abrupt onset of hearing loss, accompanying ear
fullness and tinnitus are apparent during course of the diseas-
Frequency (Hz)
es, but the lack of vertigo and high recovery rate of hearing
125 250 500 1,000 2,000 4,000 8,000
0 still differentiates LHL from SD or MD. However, diagnostic
or therapeutic guidelines and consensus on this disease entity
10 13.2 13.4
17.2 16.8 has not been established.6-10)
21.3 20.1 15.1
20
19.3
23.5 Clinical features of LHL include a predominance of female
20.1
Hearing level (dB)

30 sufferers; peak incidence during the fourth decade of life;


27.5
high incidence of tinnitus, earfullness, and/or autophony; and
40 36.0
Table 2. Therapeutic response of medical treatment
44.1
50 46.0
Response rate of all patients (68 ears) 79.4% (54 ears)
Affected ear Responder (n=54) Complete (n=49) 72.1%
60
Unaffected ear Partial (n=5) 07.4%
70 Non-responder (n=14) 20.6%
Duration of treatment 51.2±64.2 days
Fig. 3. Mean air conduction hearing levels of the affected and unaf-
fected ear of the patients with low-tone sensorineural hearing loss. Response time 27.1±57.5 days

10 Korean J Audiol 2011;15:8-13


Choi HG, et al.

Table 3. Analysis of clinical factors on therapeutic response


Clinical factors Responder (n=54) Non-responder (n=14) p value
Age (years)* 41.7±15.3 52.3±11.7 0.030
Sex M : F 18 : 35 2 : 12 0.440
Onset-Treatment interval (days)* 21.1±52.1 77.4±99.3 0.028
Concomitant symptoms Tinnitus 34/54 10/14 0.310
Earfullness 43/54 11/14 0.063
Autophony 47/54 11/14 0.069
Unsteadiness 7/54 1/14 0.550
Initial hearing level 125 Hz 43.5±11.6 49.6±11.2 0.440
250 Hz 45.5±11.8 51.7±10.7 0.110
500 Hz 34.6±13.0 43.3±14.5 0.070
SDS (%) 97.8±6.90 96.8±7.70 0.710
ECoG SP/AP 0.22±0.10 0.36±0.19 0.080
The factors marked as ‘*’ had the statistically significant difference between responder and non-responders. SDS: speech discrimi-
nation score

Table 4. Analysis of prognostic factors on therapeutic response


Prognostic factors Frequency Correlation coefficient p value
Age vs. final HL 125 Hz 0.51 <0.0001
250 Hz 0.43 <0.0001
500 Hz 0.43 <0.0001
Onset-Tx interval vs. final HL 125 Hz 0.43 <0.0001
250 Hz 0.33 0.0080
500 Hz 0.27 0.0300
HL: hearing loss, Tx: treatment

Table 5. Therapeutic response rate according to time interval be- consistent with a previous report.4) The feeling of earfullness
tween onset of disease and treatment
occurs frequently in patients with acute LHL. For example,
Reponsder (ears) Non-responder (ears)
earfullness has been a cited initial symptom in 61.0% of pa-
Group 1 31 0
tients with MD,11) 63.5% with acute low-tone sensorineural
Group 2 16 6
hearing loss,4) and 40.2% with sudden deafness.12) At present,
Group 3 08 7
the mechanism resulting in earfullness is unknown, and even
Group 1: patients who received the treatment within 7 days of
the onset of disease, Group 2: Patients who has been treated the relationship between earfullness and the audiogram has
between 7 days and 1 month after the onset of disease, yet to be elucidated. An association of earfullness and the low-
Group 3: Patients who had received the treatment more than
1month after the onset of disease. Chi-square test, p<0.01 frequency region was described, where hearing loss is relative-
ly mild, and which disappear after the hearing threshold sta-
suspicion of bilateral involvement.8) In our study, patients bilized.13) These authors hypothesized that earfullness might
with LHL were predominantly female preponderance and the originate from some functional factor rather than an organic
peak age of incidence was in the twenties for females and in lesion of the cochlea. In our study, since the two most frequent-
the thirties for males. But, bilateral involvement was observed ly reported symptoms were earfullness and tinnitus, we sug-
only in four cases. Interestingly, the symptoms of LHL varied gest that the patients with acute onset earfullness and tinnitus
widely, and the chief complaint was not always hearing im- should be carefully and promptly evaluated for their hearing
pairment. One study reported that over half of the patients loss at lower frequencies, and properly treated thereafter.
had tinnitus, which often took the form of a low-pitched, mo- Many otolaryngologists have tried to explain the patho-
tor-like tone.4) A sensation of earfullness and autophony is also physiology of LHL with the results of audiologic examina-
frequently observed.4) Another study reported that the pa- tions.2,3,8,14) In addition to these, the glycerol test, vestibular
tient’s complaints include ear fullness (89%), tinnitus (80%), evoked myogenic potentials, and the orthostatic test have
autophony (63%), hearing loss (58%), and hyperacusis been used to discover the pathophysiology and etiology of
(43%).3) In our study, the frequent chief complaints were tinni- LHL.4,9) Especially, ECoG and the glycerol test are useful test
tus and earfullness; tinnitus was usually at lower frequencies, tools for some study groups.3,5,14,15) In one of these studies, the

www.audiology.or.kr 11
Clinical Characteristics of Low-Tone Hearing Loss

SP/AP ratio was abnormally increased in 63% of LHL pa- dose steroids, diuretics, betahistine and microcirculating en-
tients.3) In another study, the mean SP/AP ratio in LHL patients hancers, since possible mechanisms of LHL include endo-
was 0.35±0.13 and the mean detection threshold of the co- lymphatic hydrops and insufficient blood circulation. The same
chlear microphonics was 32.0±9.4 dB normal hearing level, therapeutic regimen in our study enabled the analysis of prog-
prompting the suggestion that the pathogenesis of LHL might nostic factors for this combination therapy, which may help
arise from an endolymphatic hydrops with little or no impair- clarify the scientific clinical guidelines for the treatment of
ment of hair cells that resembled early-stage MD.8) Presently, LHL. In this study, overall response rate of the combination
we did not observe the same findings of elevated SP/AP ratio treatment was as high as 80%, with no side effects or adverse
in ECoG as in previous studies. Only one patient among 18 pa- effects. Limitation of this study is lacking proper control
tients who has been checked ECoG during the symptomatic group, but, analysis of therapeutic efficacy of combination
period displayed an elevated ECoG finding. Even though our therapy here in our study will be an important basic data for
follow-up period may not have been long enough to show the future study in the patients with LHL. Presently, we eval-
the progression of LHL to MD, the observation of ECoG find- uated prognostic factors affecting treatment response in pa-
ings within normal range at the initial stage of LHL lead us to tients with LHL which were age and onset-treatment inter-
seek other possible pathomechanisms of LHL beyond endo- vals. The treatment results were much better in younger
lymphatic hydrops. Only one of our patients who showed ab- patients and in patients with shorter intervals between onset
normal ECoG finding subsequently progressed to MD within and treatment. Our observations support the view that pa-
a short period following LHL onset. Our results indicate that tients with acute tinnitus and earfullness should be consid-
LHL might be a different disease entity from MD. However, ered as LHL patients and with LHL, early combination treat-
we must not rule out the possible progression of LHL to MD ment being more effective than delayed treatment.
once there is an abnormally elevated SP/AP ratio.
Since there is no international consensus on the treatment Conclusion
of LHL, various regimens and diverse modalities have been
reported for the therapeutic strategies of LHL. One study re- Although LHL frequently shows somewhat similar clinical
ported that more than 70% of examined female patients and and audiologic characteristics to mild form of sudden deafness
80% of the male patients showed complete or partial hearing or early stage MD, it is thought to be a different entity with
recovery when treated with vitamin B12 and adenosine tri- better medical treatments response and prognosis compare to
phosphate disodium, with only a few patients taking prednis- SD or MD. For the patients with acute ear fullness and tinni-
olone at various dosages.4) Prednisolone forms the basis of an tus, LHL should always be considered. Its early diagnosis and
accepted regimen consisting of 40 mg/d on days 1-3; 30 prompt treatment might be the one approach guideline for
mg/d on days 4-6; 20 mg/d on days 7-9; and 10 mg/d on this less known disease entity. Further studies on pathogenesis
days 10-12).3) Diuretics or vasodilators can also be used.3) of LHL are needed to build up the consensus on the disease
In a study that examined the treatment outcome of intratym- entity and on the standard treatment modality. 
panic dexamethasone and hyaluronidase injection for 18
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www.audiology.or.kr 13