Sudden sensory neural hearing loss

A subset of patients with SNHL develops hearing loss rapidly, awakening with it in the
morning or developing a progressive loss over 12 hours or less. This commonly is a
frightening experience for the patient, who might assume that this is a life threatening
disorder or that it will lead to profound bilateral deafness.
Definition is controversial and different guidelines exist.

Wilson et al 30dB sensorineural

in ≥3 contiguous frequencies
in <3 days
Haberkamp and Tanyeri >20dB hearing loss
in ≥3 contiguous frequencies
in <3 days
Kronenberg et al >20dB sensorineural hearing impairment in
one or both ears occurring abruptly or in a
time interval not exceeding 1 week
Arellano et al Generally unilateral sensorineural hearing
loss which develops in <72 hours

AETIOLOGY OF SUDDEN SENSORY NEURAL DEAFNESS

Infectious Disorders
Viral Infection: Viral neuritis or cochleitis has long been thought to be the most common
cause of sudden SNHL. SNHL can complicate clinically evident infections with mumps,
measles, herpes zoster, and infectious mononucleosis and with congenital rubella and
CMV. With the possible exception of mumps parotitis and herpes zoster infections,
however, the clinical diagnosis of viral infections is unreliable.The mumps virus has been
isolated from the perilymph of patients with sudden SNHL and experimental mumps
labyrinthitis has been reproduced in hamsters by inoculation of the subarachnoid space with
mumps virus. Lassa fever, an arenavirus infection endemic in West Africa, has been shown
to be associated with sudden SNHL in approximately two thirds of the patients. The time
course, the results of audiometric testing, and the patterns of recovery in Lassa fever are
very similar to that in idiopathic sudden SNHL. Measles and rubella also are well-
documented causes of labyrinthitis but these cases rarely manifest in a manner typical of
sudden SNHL. Herpes zoster oticus also can cause sudden SNHL, although it is a clinical
entity distinct from idiopathic sudden SNHL. The evidence that herpes zoster may be
associated with idiopathic sudden SNHL is limited to viral seroconversion studies. Sudden
hearing loss associated with infectious mononucleosis is rare but has been reported.
Meningitis. Meningitis is a well-recognized and common etiology of acquired severe to
profound SNHL. It is possible that rare cases of idiopathic sudden SNHL may be caused by
subclinical meningoencephalitis.
Syphilis. It has been estimated that the incidence of syphilis in patients with sudden SNHL is
2% or less. Syphilitic hearing loss may manifest at any stage of the disease, and it may be
associated with other manifestations of syphilis, with vestibular symptoms, or alone. It may
also manifest with unilateral or bilateral sudden SNHL. It is important to consider the
possibility of reactivation of syphilis in patients with HIV infection.
Lyme Disease. Lyme disease is a well-established etiology of acute facial paralysis, and it
would not be unreasonable to assume that it could also cause SNHL. Hearing loss has not
been strongly associated with Lyme disease, however.
Acquired Immunodeficiency Syndrome: It is not surprising that sudden SNHL may be
associated with HIV infection; it is not a common manifestation of AIDS, but it has been well
documented in the literature. In the presence of HIV infection, sudden SNHL may occur with
or without the presence of opportunistic infection, and it may occur without clinical
evidence of AIDS. Sudden SNHL caused by reactivation of latent syphilis may complicate any
stage of HIV infection. Some cases of sudden SNHL associated with AIDS may be the result of
reactivation of latent CMV infection.
Neoplasms
Acoustic Neuroma. It is common for sudden SNHL to be the initial manifestation of a
vestibular schwannoma (acoustic neuroma). The presence of tinnitus in the ipsilateral ear
before sudden SNHL is suggestive, but it is not present in most cases. In addition,
midfrequency and highfrequency hearing loss are more commonly associated with acoustic
neuroma than are low-frequency losses, and electronystagmography abnormalities are
more common with acoustic neuroma. Responsiveness of the hearing loss to treatment with
steroids is not a reliable indicator that a retrocochlear lesion can be ruled out.
Neoplasms of the CPA or internal auditory canal other than acoustic neuromas have also
been associated with SNHL. These include meningioma,cholesteatoma, hemangioma,
arachnoid cyst, and metastatic neoplasms.
Skull base neoplasms that erode into the inner ear can rarely manifest with SNHL.
Trauma and Membrane Ruptures Head Injury: Sensorineural hearing loss of any degree can
occur after closed or open head injury. The mechanism of injury in such patients has been
shown pathologically to vary from mild loss of outer or inner hair cells or cochlear
membrane breaks to fracture across the labyrinth or intralabyrinthine hemorrhage. Many of
these injuries are pathologically indistinguishable from injuries of acoustic trauma. Some
patients experience a variable degree of recovery from head injury–induced hearing loss, a
process probably equivalent to the temporary threshold shift seen with acoustic trauma.
Perilymphatic Fistula: Round or oval window fistulae can occur congenitally, after
stapedectomy, or after barotrauma. SNHL is well described after events that cause
barotrauma. Some investigators theorize that these fistulae can occur after heavy lifting or
straining or even spontaneously. Patients with such fistulae can have sudden or fluctuating
SNHL and varying degrees of vestibular symptoms. No test is reliable for detecting the
presence of such a fistula, and even surgical exploration is subject to error. Except in post
stapedectomy patients, it is doubtful that perilymph fistula is a significant cause of SNHL.
Intracochlear Membrane Breaks. Intracochlear membrane ruptures and fistulae have been
documented pathologically in patients with endolymphatic hydrops. Such breaks have been
proposed to be an etiology of SNHL.
Pharmacologic Toxicity : Interferon has been associated with SNHL that has been reversible
in most patients. The insecticides malathion and methoxychlor have been associated with
bilateral SNHL.
Immunologic Disorders The finding that many patients with SNHL seem to benefit from
glucocorticoid therapy and the finding of cross-reacting circulating antibodies in many
patients with sudden and rapidly progressive SNHL suggest that at least a subset of SNHL
cases are caused by inner ear autoimmunity.In addition, many well-known autoimmune
diseases have been associated with SNHL, including Cogan syndrome, systemic lupus
erythematosus, temporal arteritis, and polyarteritis nodosa.
Vascular Disorders: Sudden hearing loss can occur with occlusion of the cochlear blood
supply. Because of the abruptness of onset of SNHL and the fact that the cochlea depends
on a single terminal branch of the posterior cerebral circulation, vascular occlusion has been
thought by some authors to be an attractive hypothetic etiology for idiopathic sudden
hearing losses. Other aspects that argue against a circulatory etiology include high incidence
of spontaneous recovery, significant incidence in young patients, lack of an apparent
increased incidence in diabetics, the fact that the loss frequently is limited to just a few
frequencies, and the fact that most patients do not have vertigo. Similar to viral etiologies, a
few cases of SNHL are clearly a result of vascular occlusion, but most cases remain
idiopathic. Migraine, hemoglobin sickle cell disease, and macroglobulinemia have been
documented to be associated with sudden SNHL. Rare cases of thromboangiitis obliterans
(Buerger disease) have been associated with sudden SNHL. Small cerebellar infarctions may
mimic labyrinthine lesions, including sudden onset of hearing loss. Cardiopulmonary bypass
and noncardiac surgery have been associated with an increased risk of sudden SNHL.
Sudden SNHL has also been reported after spinal manipulation with probable injury to the
vertebrobasilar arterial system. It has long been believed that patients with diabetes have a
higher incidence of idiopathic sudden SNHL than people without diabetes. This belief has
been based on the higher incidence of other acute cranial neuropathies and on the diffuse
vascular abnormalities found in patients with diabetes
Developmental Abnormalities: Large vestibular aqueduct syndrome is associated with
SNHL and frequently occurs in a stepwise fashion associated with minor head trauma. It
seems plausible that other, as yet undefined developmental abnormalities may predispose
individuals to sudden SNHL, either spontaneously or after minor head trauma.
Idiopathic Disorders :
Meniere Disease. Some patients seen with typical sudden SNHL ultimately may develop a
history more suggestive of endolymphatic hydrops or even frank Meniere disease; this
probably constitutes only 5% of all patients with sudden SNHL
Multiple Sclerosis. MS is a demyelinating disorder of the CNS manifested by differing
neurologic lesions separated by space and time. Sudden SNHL is a rare initial manifestation
of MS. Among patients with MS, auditory abnormalities are common.
Sarcoidosis. CNS manifestations are rare (incidence of 1% to 5%), although among patients
with neurosarcoidosis, 20% have eighth cranial nerve findings. This eighth cranial nerve
involvement may manifest as sudden SNHL, although it only rarely is an isolated finding.
Psychogenic Disorders Pseudohypacusis frequently manifests as a sudden loss. In most
patients, malingering is readily apparent after initial audiologic studies.
Diagnosis
1. Full blood count and erythrocyte sedimentation rate (ESR)
2. urea and electrolytes
3. lipid profile
4. glucose
5. thyroid function
6. syphilitic serology
7. Antibody titres for viral infections
8. HIV screening
9. auto-antibodies
10. HRCT in suspected trauma to head
11. MRI (depending on availability). Gadolinium-enhanced MRI of the internal auditory
canal and CPA
12. Angiogram of cochlea blood supply to rule out vascular etiology
TREATMENT Treatment of sudden SNHL should be based on its etiology.
If an etiology is apparent, the appropriate treatment should follow:
1. antibiotics for infectious causes
2. withdrawal of the offending drug for ototoxicity.
3. Vasodilators have been used extensively in the treatment of sudden SNHL. Any
proposed vasodilator would have to cross the blood-brain barrier and have an effect
on intracranial circulation. IV histamine infusion, oral papaverine, and oral nicotinic
acid have been used most frequently. Other agents proposed to improve cochlear
blood flow include low-molecular-weight dextran, mannitol, pentoxifylline, and
heparin. It was later shown that triiodinated benzoic acid derivatives such as
diatrizoate have a specific effect on the stria vascularis, protecting the endocochlear
potential from furosemide-induced depression
4. Because endolymphatic hydrops is a common final pathology for many inner ear
injuries and may be associated with some cases of sudden SNHL, some authors have
advocated treatment with a sodium-restricted diet and a diuretic
5. Because of the evidence for a viral etiology and, specifically, evidence of
herpesvirus, treatment with oral antiviral medications has been proposed. Because
these drugs rarely have adverse side effects, many practitioners routinely treat
patients with sudden hearing loss with antivirals in addition to steroids
6. Despite the possible efficacy of carbogen treatment, it is not routinely offered to
patients with sudden SNHL; the treatment requires inpatient hospitalization, which
renders it expensive and inconvenient. Insurers typically regard carbogen as
investigational, which results in significant expense to patients. Because of these
issues and its controversial nature, most patients do not accept carbogen if it is
offered. It would be more strongly considered in exceptional situations, such as a
 patient with sudden SNHL in an only-hearing ear or in a particularly motivated
patient.
7. Most cases are idiopathic, and treatment decisions should be made on the basis of
empiric guidelines. Because of the dictum, “first, do no harm,” new or
unconventional treatment protocols should be well reasoned and carefully applied.
It seems prudent to avoid the use of potentially harmful treatment protocols
outside of controlled clinical trials, because serious complications and deaths have
been reported after treatment of idiopathic sudden SNHL.
Steroids in moderate doses have become the most widely accepted treatment option for
idiopathic sudden SNHL. Direct steroid treatment to the inner ear via middle ear instillation
or round window microcatheter has seen increasing use on an empiric basis. This treatment
has the potential advantage of very high steroid concentrations within the inner ear without
the associated systemic side effects. An alternative management regimen proposed by some
investigators involves attempts to improve blood flow or oxygenation to the inner ear. A 10-
day course of prednisone, approximately 1 mg/kg/day, is prescribed, followed by a slow
taper. If a partial recovery is noted at the end of the 10 days, the full dose is extended
another 10 days, and the cycle is repeated until no further improvement is noted.
Valacyclovir (1000 mg three times daily for 10 days) also is considered, because it may be
beneficial, and because the risks and side effects are minimal. A 2-g sodium diet is
recommended with a hydrochlorothiazide-triamterene diuretic combination
Drugs used in idiopathic sudden sensory neural hearing loss.