THEME ENT

Jessica Prasad Vincent C Cousins

MBBS(Hons), is a surgical registrar, BMedSci, MBBS, FRACS, is Clinical Associate Professor, Monash
The Alfred Hospital, Melbourne, University Department of Surgery, and an otologist-neurotologist,
Victoria. [email protected] The Alfred Hospital, Melbourne, Victoria.

Asymmetrical
hearing loss
Hearing loss is the most prevalent sensory deficit reported
Background
by patients. Ten to 20% of Australians have some hearing
General practitioners are usually the first point of contact for
impairment and approximately 50% of those over 65 years of age
patients with hearing loss. Asymmetrical sensorineural hearing
are hearing impaired. In 2005–2006, 2.6% of all general practice
loss can be a symptom of a wide range of diseases. A correct
diagnosis is essential for appropriate treatment and limitation of encounters in Australia were for ear symptoms with an
the progression of hearing loss. additional 0.8% for vertigo and dizziness.1 Hearing loss can be
conductive, sensorineural or mixed. The most common type of
Objective
hearing impairment in adults is sensorineural.2
This article provides an outline for an approach to a patient

presenting with asymmetrical sensorineural hearing loss, and
also provides a brief summary of four disease processes which Sensorineural hearing loss (SNHL) indicates defects in either the
may present with asymmetrical hearing loss. cochlea or in the neural transmission to the central nervous system.
Table 1 demonstrates the wide range of possible aetiologies of SNHL.
Discussion
The more common causes of SNHL include presbycusis, noise induced
Asymmetrical sensorineural hearing loss may be secondary to
hearing loss, Meniere disease, drug induced (ie. aminoglycosides) and
the process of aging or simply be related to excessive noise
infectious causes.
exposure. It can however, be the only presenting symptom of a
vestibular schwannoma or an intracranial tumour. A high level Asymmetrical sensorineural hearing loss (ASNHL) is defined as
of clinical suspicion is required to ensure that these pathologies binaural difference in bone conduction thresholds of >10 dB at two
are not missed. consecutive frequencies or >15 dB at one frequency (0.25–8.0 kHz)3
(Figure 1). Poorer speech perception will often accompany poorer
hearing and may be the reason for the patient’s presentation. A
difference of >15% in the maximum speech discrimination score is
also significant.

An approach to patient presenting with ASNHL

Asymmetrical sensorineural hearing loss may occur as a result of the
common causes of bilateral sensorineural hearing loss such as age
related or noise related hearing loss. However, ASNHL may also be:
• the only presenting symptom of a vestibular schwannoma or an
intracranial tumour
• an indicator of another serious underlying pathological process such
as an immune disorder or demyelinating disease, or
• associated with conditions such as idiopathic sudden SNHL or
Meniere disease which may require specialist referral.

312 Reprinted from Australian Family Physician Vol. 37, No. 4, April 2008
Dual pathology is not uncommon in the causation of SNHL, and it is
Table 1. Examples of pathologies associated with SNHL
the asymmetry of the loss that may indicate an additional pathological
process in the worse hearing ear. Therefore, the management of Hereditary and • Syndromic (1/3)
a patient with ASNHL requires a high level of clinical suspicion to developmental – Usher syndrome
ensure that these pathologies are not missed. – Pendred syndrome
Unilateral tinnitus, with or without associated hearing loss, has – Alport syndrome
the same diagnostic implications as ASNHL. • Nonsyndromic (2/3)
A detailed history can give valuable clues to the aetiology of the – autosomal dominant inheritance
hearing loss. It is important to establish: – autosomal recessive inheritance
• the pattern of the hearing loss (unilateral or bilateral symptoms, – x lined or maternal mitochondrial
progressive or stepwise, sudden or gradual in onset) – cochlear otosclerosis
• associated symptoms such as tinnitus, aural fullness, vertigo, • Inner ear anomalies
imbalance, otalgia and otorrhoea – large vestibular aqueduct
• history of previous ear infections, noise exposure, otic barotrauma • Cochlea anomalies
or ear surgery – Mondini
• history of trauma to the ear or head Infection* • Meningitis, mumps, rubella, syphilis
• previous intracranial surgery • Other viral infections
• general health (conditions such as cerebrovascular disease, Immune • Systemic lupus erythromatosus
diabetes, other metabolic and autoimmune disorders can affect disorders • Rheumatoid arthritis
hearing) • Polymyositis
• family history of hearing loss or ear related tumours • Ulcerative colitis
• use of medications such as antibiotics (especially aminoglycosides), • Scleroderma
antimalarial drugs, anti-inflammatory agents (nonsteroidal anti- • HIV
inflammatory agents [NSAIDs], salicylates), chemotherapeutic Neurological • Multiple sclerosis
agents and diuretics. • Cerebral ischaemia/stroke
• Migraine
Clinical examination
Neoplasms* • Benign
The clinical examination includes examination of: • Vestibular schwannomas (acoustic neuroma)
• the auricles – skin lesions, pre-auricular sinuses, oedema, erythema, • Meningiomas/other
tragal tenderness • Malignant
• external auditory canals – mass lesion, discharge • Local primary tumours
• tympanic membranes – colour, thickness, scarring, effusion, • Metastatic lesions
perforations Ototoxins* • Aminogylcosides
• head and neck • Loop diuretics
• cranial nerves, and • Quinine
• postnasal space. • Chemotherapy agents (eg. cisplatin)
Gross testing of hearing can be carried out using whisper tests. • NSAIDs
Tuning fork tests generally allow the physician to determine whether Systemic • Paget disease
there is a conductive component to the hearing loss (Table 2). • Diabetes mellitus
All patients with ASNHL require investigations including • Hypertriglyceridemia/hypercholesterolemia
audiology and detailed diagnostic imaging (usually magnetic
Trauma* • Noise induced
resonance imaging [MRI]) to determine aetiology. In some cases
• Acoustic trauma
various blood tests will assist. The clinical examination and history
• Head injury
may often provide little help in differentiating patients with more
• Previous cranial/ear surgery
common minor ear conditions from those with significant or sinister
Vascular/ • Coagulopathies
diagnosis. Cranial nerve palsies on examination are a worrying haematological • Leukaemia
sign. All patients with ASNHL require specialist ear, nose and
Idiopathic* • Presbycusis
throat (ENT) referral and assessment due to the complex nature of
• Meniere disease
the involved pathologies. It is preferable for this to happen within
• Idiopathic sudden sensorineural hearing loss
6 weeks; but sooner if there are other associated neurological
* Indicates a more common/important condition
symptoms or signs.

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 Asymmetrical hearing loss THEME

Investigation may lead to a means of treating or limiting the hearing Signs of demyelinating lesions of central nervous system may also
loss, such as in Meniere disease. It may indicate the presence of lesions be identified with this technique. The use of gadolinium contrast
that need treatment such as large cerebellopontine angle tumours. It increases the sensitivity of this study. Computerised tomography
may simply provide a diagnosis of cause and explanation to the patient (CT) scanning may be used if inner ear pathology or a developmental
which may not be specifically treatable, or which may have implications anomaly such as an inner ear dysplasia or large vestibular aqueduct is
for other family members where an inherited cause is identified. suspected. Computerised tomography scanning, preferably with the
use of contrast, will also be used if an MRI is contraindicated such as
Audiology
when the patient has implanted electrical devices (cochlear implant,
Routine audiology testing includes pure tone audiometry (PTA), cardiac pacemaker) or a cerebral aneurysm clip.4
speech discrimination tests and impedance tympanometry. Evoked Magnetic resonance imaging do not attract a Medicare rebate
potential audiometry, which includes auditory brainstem response and unless ordered by a specialist. If there are concerns about a major
electrocochleography (ECoG), may be used in particular circumstances. space occupying lesion, a contrast enhanced CT scan can be arranged
Pure tone audiometry is a measure of hearing levels by air and early by the GP first to exclude any lesion >1.5 cm in maximum
bone conduction using pure tone stimuli (at 250, 500, 1000, 2000, diameter. If clear, then it is reasonable to wait for ENT assessment.
4000 and 8000 Hz) at selected intensities. Speech audiometry utilises
Serology, haematology, biochemistry
spoken voice as a sound stimulus at selected intensities.
The speech discrimination score is calculated as the highest Haematological, biochemical and serological tests may be useful
percentage of phonemes or word parts repeated correctly when in investigating hearing loss and choice of investigation will be
presented at 40 dB or more above the average response levels determined by the history and examination findings. These include:
obtained on the pure tone air conduction test. A disproportionate loss • fluorescent treponemal antibody for syphilis
of speech discrimination compared to PTA results may be indicative of • fasting blood sugar levels for diabetes
a problem with the cochlear nerve (retrocochlear pathology). • erythrocyte sedimentation rate, antinuclear antibodies and
rheumatoid factor in patients with a suspected immune cause of
Imaging
hearing loss.3
A finding of ASNHL will generally necessitate the use MRI of the Other specialised investigations such as haemolytic component and
inner ear and brain to exclude retrocochlear pathology such as a antiheat shock protein 70 antibodies5,6 are sometimes used in the
vestibular schwannoma, meningioma or other intracranial tumours. specialist setting.
An overview of four pathologies, which may present as ANSHL, is
Figure 1A, B. Audiograms showing mild (A) and severe (B) presented below.
asymmetrical sensorineural hearing loss
Noise induced sensorineural hearing loss
A
Hearing impairment secondary to excessive noise exposure is one of
the most common causes of new presentations with sensorineural
hearing impairment7 and may account for approximately 30–50%
of hearing loss presentations. Prolonged or cumulative exposure to
noise levels exceeding 75–80 dB such as may occur with loud music,
machinery and gun shooting, overwhelms the reparative capacity
of the cochlear hair cells and result in permanent hearing loss.8 The
pattern of hearing loss is usually bilateral and equal but may be
asymmetrical, particularly if one ear is preferentially exposed to the
noise source, such as with gun shooting.
B
The diagnosis is indicated by a history of excessive or prolonged
noise exposure and an audiogram finding of high frequency SNHL
occurring maximally at 3–6 KHz. Other causes of ASNHL will need to
be excluded.
The management of noise induced sensorineural hearing loss
(NISNHL) is primarily preventive with education concerning avoidance
of noisy environments and use of appropriate protective equipment
such as ear plugs or ear muffs. Noise that precludes one hearing
another speak is likely to be >80 dB in intensity. Some machinery is
labelled with intensity data as a guide.

Reprinted from Australian Family Physician Vol. 37, No. 5, May 2008 315
 theme Asymmetrical hearing loss

Table 2. Tuning fork tests

The Rinne test compares air conduction (AC) with bone conduction (BC) using a 256 Hz or 512 Hz tuning fork which is struck and placed
on the mastoid process. When the patient can no longer hear the sound conducted via the bone, the fork is moved to beside the external
auditory canal (EAC). AC is greater than BC (AC>BC) if the patient still can hear the sound at the EAC. This is called a positive Rinne test
and is consistent with normal hearing or ipsilateral SNHL. A negative Rinne test (BC>AC) indicates ipsilateral conductive hearing loss
(CHL) or profound SNHL with crossover
The Weber test utilises a 512 Hz tuning fork. The resonating fork is placed on the vertex and the patient is asked to identify the ear
where the sound is heard louder. In a conductive loss, the Weber lateralises to the affected ear. In SNHL, the sound lateralises to the
better hearing ear. Central perception indicates symmetrical hearing

Weber test Rinne test left ear Rinne test right ear
Normal hearing Central Positive (AC>BC) Positive (AC>BC)
CHL left ear Localises to left Negative (BC>AC) Positive (AC>BC)
CHL right ear Localises to right Positive (AC>BC) Negative (BC>AC)
Symmetrical SNHL Central
ASNHL left ear affected Localises to right Positive Positive
(negative if profound SNHL on left)
ASNHL right ear affected Localises to left Positive Positive
(negative if profound SNHL on right)

Once NISNHL has been diagnosed, the use of hearing aids may The incidence of vestibular schwannoma is about 2% in all patients
be necessary to aid communication. Affected subjects should also with ASNHL.10
be advised to protect their ears from all noxious influences, including The mean age of diagnosis is in the mid 40s. It is uncommon in
further noise exposure. younger patients and may be associated with the rare condition of
neurofibromatosis type 2 in these patients.
Vestibular schwannoma
Presentation is usually with asymmetrical hearing loss. Typically
Vestibular schwannoma (acoustic neuroma) is a benign tumour hearing loss is gradual, but sudden onset of hearing loss has been
arising from the Schwann cells in the vestibular portion of eighth found in 12–16% of affected patients.11,12 Patients may also have
cranial nerve. It usually begins growing in the internal auditory unilateral tinnitus, vertigo and balance disturbance. Facial and
canal and extends into the cerebellopontine angle of the posterior trigeminal nerve function may also be altered.8
cranial fossa with time. The clinical incidence rate is approximately Examination findings are generally few. Tuning fork tests should
1 per 100 000 per year and the incidence may have increased in be performed (Table 2). Facial numbness, especially a reduced corneal
recent years increase due to the increased use of MRI.9 This tumour reflex, and facial weakness may be seen with larger tumours.
comprises greater than 85% of all cerebellopontine angle tumours. Audiogram findings include some pattern of ASNHL and decreased
speech discrimination scores. Vestibular function tests may indicate
Figure 2. MRI of left sided vestibular schwannoma in patient reduced responses. Auditory brainstem responses are frequently
presenting with asymmetrical sensorineural hearing loss
abnormal, however they are affected in many other conditions leading
to high false positive and false negative rates.13,14 Magnetic resonance
imaging with gadolinium is sensitive for tumours from 2 mm in size
and is used in most patients presenting with ASNHL to ensure that
vestibular schwannomas are diagnosed (Figure 2).
High resolution CT scanning of the temporal bones provides
excellent middle ear cleft and inner ear detail. It does however have
limitations in assessing intracranial tumours <1.5 cm. A contrast
enhanced MRI should be performed when significant pathology
is suspected.4
Management of the tumour depends on tumour size, patient
symptoms, preference and comorbidities, and availability of treatment
facilities and expertise. Management options include observation,
radiation or surgery.

316 Reprinted from Australian Family Physician Vol. 37, No. 4, April 2008
 Asymmetrical hearing loss THEME

Close observation using serial MRI can be an option for small presents at 40–60 years of age. The aetiology of the disease is not
tumours or patients with significant medical comorbidities. Treatment fully understood. The disorder is thought to be related to raised
may then be undertaken if there is tumour growth or increasing endolymph volume in the membranous labyrinth of the inner ear,
patient symptoms. Microsurgical excision of the tumour is still termed ‘endolymphatic hydrops’. Hearing loss is sensorineural, low
regarded as the gold standard of treatment. This may entail sacrifice frequency and usually unilateral. Bilateral disease has been reported
of hearing (translabyrinthine approach) in large tumours or where in various series as having a prevalence of 2–78% and development
there is poor pre-operative hearing. Hearing preservation approaches of bilateral disease may occur in time.28 With disease progression,
(middle and posterior cranial fossa approaches) may be used in high frequency hearing loss occurs as well and the patient is left
smaller tumours with good presenting hearing. Stereotactic radiation with a moderate to severe SNHL affecting all frequencies, indicated
therapy can be used for tumours up to 3 cm in maximum dimension, by a flat pure tone audiogram
particularly in the elderly and the infirm. The aim of surgery is total The diagnostic guidelines developed by the American Academy
tumour removal while minimising morbidity. Radiation is used to of Otolaryngology29 stipulate that at least two spontaneous episodes
halt tumour growth. All treatment is aimed at limiting morbidity and of rotational vertigo lasting at least 20 minutes, audiological
preventing mortality caused by tumour growth. Cure rates range confirmation of sensorineural hearing loss and tinnitus, or perception
90–99% in various series for either modality of treatment.9,15,16 of aural fullness are required for a diagnosis.
As these patients present with ASNHL, most have imaging
Idiopathic sudden sensorineural hearing loss
studies to exclude retrocochlear pathology. Electrocochleography and
Idiopathic sudden sensorineural hearing loss (ISSNHL) of unknown vestibular function tests are also usually performed.
aetiology is a loss of significant hearing (>30 dB in at least three Management is challenging due to incomplete understanding of
adjacent frequencies) that occurs over <72 hours. The incidence the disease process and the paroxysmal nature of the symptoms.
is 8–15 per 100 000 per year. 17 Proposed causes include viral Treatment aims to reduce the frequency and severity of vertiginous
inflammation of the cochlear nerve or inner ear, vascular interruption, attacks, and ameliorate or abort hearing loss and tinnitus. Medical
rupture of intralabyrinthine membranes and autoimmune diseases. treatments, which are usually first line treatment, include a low salt
The average age of onset is 40–50 years. Hearing loss is unilateral diet, diuretics (thiazides) and a betahistine. In patients with symptoms
in 95–100% of patients. Bilateral loss tends to occur in an older and signs suggesting Meniere disease, it is very reasonable for the
population with lower recovery rates.9,18 Overall recovery rates are GP to institute these medical measures, particularly if there is delay
50–65% with a high rate of spontaneous recovery. in availability of ENT assessment. The efficacies of these treatments
A diagnosis of ISSNHL necessitates exclusion of mass lesions and have not been proven.27,30
other discrete conditions causing ASNHL. However, the acute onset of Pressure pump therapy, surgical modalities including endolymphatic
hearing loss and pattern seen on audiogram findings are nonspecific sac surgery, labyrinthectomy and vestibular nerve section, and chemical
for any particular diagnosis. ablation using intratympanic gentamicin31,32 are treatments used to
Treatment is controversial as the aetiology remains obscure. Oral alleviate vertigo in the 15–20% of patients who have failed medical
steroids are used frequently, however evidence for their effectiveness treatment. Intratympanic steroids have been used for treatment of
is lacking.19 In the acute setting, it is reasonable to institute a SNHL associated with Meniere disease with variable success.33–36
1 week reducing course of prednisolone if there are no medical
contraindications. All patients will need ENT review and MRI, but
Summary of important points
a good quality CT scan can be done early to exclude a major space • ASNHL can be a presenting symptom of a range of disease
occupying lesion. If the hearing loss is in the better hearing or only processes.
hearing ear, referral should be made on the day of presentation. • Thorough history and examination are invaluable in directing further
There is some evidence that steroids injected into the middle ear may investigations.
have a role in the treatment of ISSNHL,20–24 particularly if oral steroid • The use of tuning forks can differentiate SNHL from CHL.
treatment has failed. Hyperbaric oxygen may improve hearing in early • All ASNHL must be investigated.
presentation ISSNHL.25 Vasodilators and antivirals have also been used, • Important conditions not to be missed include vestibular
however evidence of efficacy is lacking. Patients may need hearing aids schwannoma and other CPA tumours.
and other rehabilitation where there is inadequate recovery.
Conflict of interest: none declared.
Meniere disease
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correspondence [email protected]

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