Sudden Hearing Loss:

- Definition:
 SNHL of >30 dB in 3 consecutive frequencies within 3 days.
- Median age 40-54 years.
- Mostly unilateral.
- Bilateral in 1-5%
- Acute tinnitus accompanies the hearing loss in most cases.
- Vestibular symptoms are present in 25-50% of patients.

Etiology:
Divided into categories of

- Idiopathic sudden SNHL (90%) :

 Theories includes:
 Viral
 Vascular
 Intracochlear membrane rupture
 Autoimmune.

-Defined causes that must be exclude (10%) :

 Infections:
meningitis, encephalitis , labyrinthitis , cochleitis

Viral : Mumps , herpes zoster, , rubella

Epstein barr , HIV , CMV , Influenza , enterovirus

Bacterial : lyme disease, syphilis ,

 Trauma:
Head injury, temporal bone fractures , ear operations, noise trauma,
barotrauma, spontaneous rupture of cochlear
membranes.
Large vestibular aqueduct syndrome is associated
with SNHL, after minor head trauma.

 Vascular:
Hemorrhage, embolism or thrombosis of labyrinthine or cochlear artery or their vasospasm.
They may be associated with diabetes, hypertension, polycythaemia, macroglobinaemia or
sickle cell trait.

 Cardiovascular:
Thromboembolism, Macroglobulinemia, Sickle cell disease, severe acute hypotension

 Genetic:
englarged vestibular aqueduct in pendred syndrome, brachio-otorenal syndrome.

 Otologic:
Meniere's disease, Cogan's syndrome,
Perilymphatic Fistula.

 Toxic:
Ototoxic drugs. Aminoglycosides, aspirin, cisplatin , radiation therapy

 Neoplastic:
Acoustic neuroma. Meningioma ,temporal bone mets

Neurologic:
 Multiple sclerosis

Hypothyroidism, sarcoidosis ,

Investigations:

Must be done early , faster treatment improves prognosis

Detailed history , physical examination

 Hearing assessment
 CBC and ESR; urea and electrolytes;
 lipid profile; glucose;
 thyroid function;
 syphilitic serology;
 auto-antibodies;
 ± MRI (depending on availability).

Bad prognostic factors:

1. Vestibular symptoms (vertigo)
2. total deafness
3. delay in treatment initiation
4. advanced age
5. down sloping audiogram (high- frequency loss)
6. associated vascular risk factors
Good Prognostic factors:
1. minimal hearing loss
2. low-frequency hearing loss
3. no change in ECoG N1 latency
4. younger patients below 40

Treatment :

 Oral steroids:
- Main stay treatment for sudden SNHL.
- Prednisone 1 mg/kg/d (maximal dose is 60 mg/d).
then do PTA if there is improvement tapper the steroid
If no improvement give steroid for another week then tapper

 Intra-tympanic steroids:
Indications:
-Contraindications to oral steroids.
- Salvage after failed oral steroids.
Hyperbaricoxygen:

Oxygen that is given at a pressure that is higher than the pressure of the atmosphere at
sea level , increases the amount of oxygen in the body.

Hyperbaric oxygen can increase perilymph oxygen tension and restore hearing in a
significant number of patients with sudden sensorineural hearing loss. Efficacy may
depend on many factors including the degree of patency of the labyrinthine artery and
the ability to increase oxygen tension in the perilymph.

 Younger patients respond better to hyperbaric oxygen

therapy (HBOT) than older patients (50-60 years).
 Early HBOT from 2 weeks to 3 months is better than late
HBOT.
 Patients with moderate to severe hearing loss benefit
more from HBOT than those with mild hearing loss.

Other treatments:
Should not routinely prescribed.
Includes:
 Antivirals
 Thrombolytics
 Vasodilators
 Vasoactive substances
 Antioxidants

Monitoring and prognosis:

 Regular follow-up audiometric evaluation should be done within

6 months of diagnosis.
 50% showed recovery within a 10-day course of steroid
therapy.
 Final hearing levels is reached by 1 month in 80% of patients
and by 3 months in 97% of patients.
 30% of patients return to normal hearing.
 30% of patients end up with profound hearing loss.

Recovery after treatment:

  Complete:
 PTA within 10 dB of initial hearing level or within
10 dB of the hearing level of the unaffected ear.

  Partial:
 PTA within 50% of initial hearing level or > 10 dB
improvement of hearing level.

  No recovery:
 < 10 dB improvement in hearing level relative to
the initial hearing level

.
Sudden SNHL & Acoustic neuroma:
▪ It is not at all uncommon for sudden SNHL to be the initial manifestation of a vestibular
schwannoma (acoustic neuroma).
▪ 10% of acoustic neuromas initially present with sudden SNHL.
▪ The prevalence of acoustic neuroma among patients with sudden SNHL ranges from a 0.8% -
3%

▪ Clues that may suggest the presence of vestibular neuroma:

1. The presence of tinnitus in the ipsilateral ear before the sudden SNHL
2. mid- and high-frequency hearing loss are more commonly associated with Acoustic
neuroma than low-frequency losses
3. electronystagmographic (ENG) abnormalities are more common with Acoustic
neuroma.

▪ There is no relationship between tumor size and SNHL

▪ Responsiveness of the hearing loss to treatment with steroids is not a reliable indicator
that a retrocochlear lesion is not present.

▪ There have been many reported cases of steroid-responsive SNHL and SNHL with
spontaneous recovery, which have been found to be caused by acoustic neuroma.

▪ It should be emphasized that one should have a high level of suspicion for acoustic
neuroma in any patient with SNHL

▪ So evaluate all patients with sudden SNHL with gadolinium-enhanced MRI.

Prognosis rule of 3:
• 1/3 return to normal hearing;
• 1/3 result in 40-60 Db;
• 1/3 result in total loss of hearing loss

Investigation of choice in children is CT with contrast, 2nd choice is MRI

DDX of SSNHL IN PEDIATRICS: ( that is why radiology of choice is CT > MRI)
1. Mondini syndrome incomplete cochlea ( one and half turn )
2. Dilated cochlear aqueduct
3. Cochlear dysplasia