Patients often describe a numbness or blockage as opposed to an obvious hearing loss.
The incidence is 5 to 20 per 100,000 patients per year. 3
Untreated, spontaneous recovery has been reported to range from 30-60%. 4
While a viral or microvascular aetiolgy is proposed, it is essentially a diagnosis of exclusion: cerebellopontine angle tumours, auto-immune disease, multiple sclerosis (MS), infectious aetiologies,intralabyrinthine haemorrhage 5, perilymph fistula and Meniere’s disease must be excluded. 6
Otoscopic examination is normal. Occasionally a middle ear effusion can result in toxic inner ear damage.
A Weber tuning fork test lateralising to the worst hearing ear is highly suggestive of a sensorineural hearing loss.
Audiometry with speech discrimination
The utility of any blood tests unless there is a history of systemic autioimmune type symptoms is very marginal.2
Generally the benefit to hearing occurs if therapy started within 7 days, with treatment worthwhile out to 14 days. 4
30-50% will show no response. 16
The general approach is to treat for a week and then repeat a hearing test.
Some supporting evidence for hyperbaric oxygen therapy as an adjunct has been published.23-26 [The Hyperbaric Unit at Prince of Wales Hospital is the only option in Sydney]
Consideration of alternative therapies such as acupuncture can at least help from a psychologic perspective.27
A repeat audiogram is arranged at the 7 day mark can assist in decisions regarding ongoing/repeated steroid or other adjuvant treatments.28
Steroid Treatment Regimes
Intratympanic Steroid: [Dexamethasone, Methylprednisolone, Triamcinolone].31 The theory is to allow higher intra-cochlear concentrations of medication via absorption through the round window membrane.
The regime is variable with options to perform a single transtympanic dose either under local anaesthetic or general anaesthesia, multiple transtympanic doses (3-4 doses 3-5 days apart2, weekly dose for 3 doses14) or to place a ventilation tube to allow self administration. 32,33 The published concentration of steroids also varies; dexamethasone ranges from 4mg/ml up to 24mg/ml.
Summary of Clinical Approach to Steroid Therapy [reproduced from American Clinical Guidelines]
Success of around 40% in selected patients, defined as at least 20dB or 20%. 32,34-36 Although not all studies have reproduced these results. It is important to note that approximately 10% will show some improvement after more than 1 month 37 when analysing results of salvage therapy. 14 4,36,38,39
Measuring Success of Treatment
It is important to realise that to experience true binaural hearing, that hearing thresholds must be within 15-20dB in both ears.
In rare cases, where there has been a response to steroids, but further decline once the prednisone is reduced, and long term high dose prednisone is required, steroid sparing agents such as methotrexate are considered.
SSNHL is an otologic emergency which requires prompt diagnosis and institution of management in order to optimise the chance of recovery. It is theorised that most cases are related to the reactivation of a virus within the inner ear, but microvascular disease, other infective agents, autoimmune processes and tumours also are occasionally implicated. The patient must be thoroughly informed as to the limitations of current treatments. Bilateral sudden sensorineural hearing loss is much rarer, but is more likely to be associated with significant systemic disease.41
It must be noted that the sudden loss of hearing can cause a significant psychologic impact and this in of itself must be addressed and managed.
Consideration must be given to hearing rehabilitation. Assistive listening devices and hearing aids are useful when there is measurable hearing. BiCross or a bone anchored hearing aid, BAHA is an option when there is a residual severe sensorineural hearing loss. It provides the sensation of binaural hearing in a majority of cases by re-routing a signal to the contra-lateral ear.42,43 The other option is cochlear implantation. This allows stimulation of the damaged cochlear allowing true binaural hearing, and is especially useful if tinnitus is a particular problem.44-47
Vestibular rehabilitation is also an important component in the management of this condition, if the balance is also affected.
- Crane RA, Camilon M, Nguyen S, Meyer TA. Steroids for treatment of sudden sensorineural hearing loss: A meta-analysis of randomized controlled trials. The Laryngoscope 2014.
- Stachler RJ, Chandrasekhar SS, Archer SM, et al. Clinical practice guideline: sudden hearing loss. Otolaryngol Head Neck Surg 2012;146:S1-35.
- Alexander TH, Harris JP. Incidence of sudden sensorineural hearing loss. Otol Neurotol 2013;34:1586-9.
- Fitzgerald DC, McGuire JF. Intratympanic steroids for idiopathic sudden sensorineural hearing loss. The Annals of otology, rhinology, and laryngology 2007;116:253-6.
- Cervantes SS, Barrs DM. Sudden Sensorineural Hearing Loss Associated With Intralabyrinthine Hemorrhage. Otol Neurotol 2014.
- Jackler RB, D. Neurotology. 2nd ed. Philadelphia: Elselvier Mosby; 2005.
- Wu CH, Wang CT, Cheng PW. Pretreatment Hearing Level-Another Prognostic Factor in Sudden Sensorineural Hearing Loss. JAMA otolaryngology– head & neck surgery 2018.
- Yu H, Li H. Association of Vertigo With Hearing Outcomes in Patients With Sudden Sensorineural Hearing Loss: A Systematic Review and Meta-analysis. JAMA otolaryngology– head & neck surgery 2018;144:677-83.
- Baird SM, Nguyen K, Bhatia DDS, Wei BPC. Inner ear and retrocochlear pathology on magnetic resonance imaging for sudden and progressive asymmetrical sensorineural hearing loss. ANZ journal of surgery 2018.
- Puccinelli C, Carlson ML. Improvement or Recovery From Sudden Sensorineural Hearing Loss With Steroid Therapy Does Not Preclude the Need for MRI to Rule Out Vestibular Schwannoma. Otol Neurotol 2019.
- Conlin AE, Parnes LS. Treatment of sudden sensorineural hearing loss: I. A systematic review. Archives of otolaryngology–head & neck surgery 2007;133:573-81.
- Wilson WR, Byl FM, Laird N. The efficacy of steroids in the treatment of idiopathic sudden hearing loss. A double-blind clinical study. Arch Otolaryngol 1980;106:772-6.
- Slattery WH, Fisher LM, Iqbal Z, Liu N. Oral steroid regimens for idiopathic sudden sensorineural hearing loss. Otolaryngol Head Neck Surg 2005;132:5-10.
- Battaglia A, Lualhati A, Lin H, Burchette R, Cueva R. A prospective, multi-centered study of the treatment of idiopathic sudden sensorineural hearing loss with combination therapy versus high-dose prednisone alone: a 139 patient follow-up. Otol Neurotol 2014;35:1091-8.
- El Sabbagh NG, Sewitch MJ, Bezdjian A, Daniel SJ. Intratympanic dexamethasone in sudden sensorineural hearing loss: A systematic review and meta-analysis. The Laryngoscope 2017;127:1897-908.
- Rauch SD. Intratympanic steroids for sensorineural hearing loss. Otolaryngologic clinics of North America 2004;37:1061-74.
- Hatano M, Uramoto N, Okabe Y, Furukawa M, Ito M. Vitamin E and vitamin C in the treatment of idiopathic sudden sensorineural hearing loss. Acta oto-laryngologica 2007:1-6.
- Nageris BI, Ulanovski D, Attias J. Magnesium treatment for sudden hearing loss. The Annals of otology, rhinology, and laryngology 2004;113:672-5.
- Tucci DL, Farmer JC, Jr., Kitch RD, Witsell DL. Treatment of sudden sensorineural hearing loss with systemic steroids and valacyclovir. Otol Neurotol 2002;23:301-8.
- Satar B, Hidir Y, Yetiser S. Effectiveness of hyperbaric oxygen therapy in idiopathic sudden hearing loss. The Journal of laryngology and otology 2006;120:665-9.
- Ibrahim I, Zeitouni A, da Silva SD. Effect of Antioxidant Vitamins as Adjuvant Therapy for Sudden Sensorineural Hearing Loss: Systematic Review Study. Audiology & neuro-otology 2018;23:1-7.
- Bravenboer de Sousa M, Cazemier S, Stegeman I, Thomeer H. Use of Vasodilators In Idiopathic Sudden Sensorineural Hearing Loss: A Systematic Review. J Int Adv Otol 2017;13:399-403.
- Rhee TM, Hwang D, Lee JS, Park J, Lee JM. Addition of Hyperbaric Oxygen Therapy vs Medical Therapy Alone for Idiopathic Sudden Sensorineural Hearing Loss: A Systematic Review and Meta-analysis. JAMA otolaryngology– head & neck surgery 2018.
- Hosokawa S, Hosokawa K, Takahashi G, et al. Hyperbaric Oxygen Therapy as Concurrent Treatment with Systemic Steroids for Idiopathic Sudden Sensorineural Hearing Loss: A Comparison of Three Different Steroid Treatments. Audiology & neuro-otology 2018;23:145-51.
- Eryigit B, Ziylan F, Yaz F, Thomeer H. The effectiveness of hyperbaric oxygen in patients with idiopathic sudden sensorineural hearing loss: a systematic review. Eur Arch Otorhinolaryngol 2018;275:2893-904.
- Cho I, Lee HM, Choi SW, et al. Comparison of Two Different Treatment Protocols Using Systemic and Intratympanic Steroids with and without Hyperbaric Oxygen Therapy in Patients with Severe to Profound Idiopathic Sudden Sensorineural Hearing Loss: A Randomized Controlled Trial. Audiology & neuro-otology 2018;23:199-207.
- Chen S, Zhao M, Qiu J. Acupuncture for the treatment of sudden sensorineural hearing loss: A systematic review and meta-analysis: Acupuncture for SSNHL. Complement Ther Med 2019;42:381-8.
- Herrera M, Garcia Berrocal JR, Garcia Arumi A, Lavilla MJ, Plaza G, Grupo de Trabajo de la Comision de Audiologia de la S. Update on consensus on diagnosis and treatment of idiopathic sudden sensorineural hearing loss. Acta otorrinolaringologica espanola 2018.
- Levy E, Sela E, Letichevsky V, Ronen O. Nationwide Survey of Intratympanic Steroids for the Management of Sudden Sensorineural Hearing Loss. The Israel Medical Association journal : IMAJ 2019;21:105-9.
- Lechner M, Sutton L, Ferguson M, Abbas Y, Sandhu J, Shaida A. Intratympanic Steroid Use for Sudden Sensorineural Hearing Loss: Current Otolaryngology Practice. The Annals of otology, rhinology, and laryngology 2019:3489419828759.
- Tarkan O, Dagkiran M, Surmelioglu O, et al. Intratympanic Methylprednisolone versus Dexamethasone for the Primary Treatment of Idiopathic Sudden Sensorineural Hearing Loss. J Int Adv Otol 2018;14:451-5.
- Gianoli GJ, Li JC. Transtympanic steroids for treatment of sudden hearing loss. Otolaryngol Head Neck Surg 2001;125:142-6.
- Slattery WH, Fisher LM, Iqbal Z, Friedman RA, Liu N. Intratympanic steroid injection for treatment of idiopathic sudden hearing loss. Otolaryngol Head Neck Surg 2005;133:251-9.
- Haynes DS, O’Malley M, Cohen S, Watford K, Labadie RF. Intratympanic dexamethasone for sudden sensorineural hearing loss after failure of systemic therapy. The Laryngoscope 2007;117:3-15.
- Plaza G, Herraiz C. Intratympanic steroids for treatment of sudden hearing loss after failure of intravenous therapy. Otolaryngol Head Neck Surg 2007;137:74-8.
- Battaglia A, Burchette R, Cueva R. Combination Therapy (Intratympanic Dexamethasone + High-Dose Prednisone Taper) for the Treatment of Idiopathic Sudden Sensorineural Hearing Loss. Otol Neurotol 2008.
- Yeo SW, Lee DH, Jun BC, Park SY, Park YS. Hearing outcome of sudden sensorineural hearing loss: long-term follow-up. Otolaryngol Head Neck Surg 2007;136:221-4.
- Yang CH, Wu RW, Hwang CF. Comparison of intratympanic steroid injection, hyperbaric oxygen and combination therapy in refractory sudden sensorineural hearing loss. Otol Neurotol 2013;34:1411-6.
- Chou YF, Chen PR, Kuo IJ, Yu SH, Wen YH, Wu HP. Comparison of intermittent intratympanic steroid injection and near-continual transtympanic steroid perfusion as salvage treatments for sudden sensorineural hearing loss. The Laryngoscope 2013;123:2264-9.
- Siegel LG. The treatment of idiopathic sudden sensorineural hearing loss. Otolaryngologic clinics of North America 1975;8:467-73.
- Sara SA, Teh BM, Friedland P. Bilateral sudden sensorineural hearing loss: review. The Journal of laryngology and otology 2014;128 Suppl 1:S8-15.
- Peters JP, Smit AL, Stegeman I, Grolman W. Review: Bone conduction devices and contralateral routing of sound systems in single-sided deafness. The Laryngoscope 2014.
- Son HJ, Choo D. Optimal management of single-sided deafness. The Laryngoscope 2013;123:304-5.
- Vlastarakos PV, Nazos K, Tavoulari EF, Nikolopoulos TP. Cochlear implantation for single-sided deafness: the outcomes. An evidence-based approach. Eur Arch Otorhinolaryngol 2014;271:2119-26.
- Tokita J, Dunn C, Hansen MR. Cochlear implantation and single-sided deafness. Current opinion in otolaryngology & head and neck surgery 2014;22:353-8.
- Nawaz S, McNeill C, Greenberg SL. Improving sound localization after cochlear implantation and auditory training for the management of single-sided deafness. Otol Neurotol 2014;35:271-6.
- Hansen MR, Gantz BJ, Dunn C. Outcomes after cochlear implantation for patients with single-sided deafness, including those with recalcitrant Meniere’s disease. Otol Neurotol 2013;34:1681-7.