THE PATIENT CASE REPORT 48-year-old man SIGNS & SYMPTOMS – Acute hearing loss, tinnitus, and fullness in the left ear Dennerd Ovando, MD; J. Walter Kutz, MD; Weber test lateralized to the – Sergio Huerta, MD right ear Department of Surgery (Drs. Ovando and Huerta) – Positive Rinne test and and Department of normal tympanometry Otolaryngology (Dr. Kutz), UT Southwestern Medical Center, Dallas; VA North Texas Health Care System, Dallas (Dr. Huerta) Sergio.Huerta@ THE CASE UTSouthwestern.edu The authors reported no A healthy 48-year-old man presented to our otolaryngology clinic with a 2-hour history of potential conflict of interest hearing loss, tinnitus, and fullness in the left ear. He denied any vertigo, nausea, vomiting, relevant to this article. otalgia, or otorrhea. He had noticed signs of a possible upper respiratory infection, including a sore throat and headache, the day before his symptoms started. His medical history was unremarkable. He denied any history of otologic surgery, trauma, or vision problems, and he was not taking any medications. The patient was afebrile on physical examination with a heart rate of 48 beats/min and blood pressure of 117/68 mm Hg. A Weber test performed using a 512-Hz tuning fork lateral- ized to the right ear. A Rinne test showed air conduction was louder than bone conduction in the affected left ear—a normal finding. Tympanometry and otoscopic examination showed the bilateral tympanic membranes were normal. THE DIAGNOSIS Pure tone audiometry showed severe sensorineural hearing loss in the left ear and a poor speech discrimination score. The Weber test confirmed the hearing loss was sensorineu- ral and not conductive, ruling out a middle ear effusion. Additionally, the normal tympa- nogram made conductive hearing loss from a middle ear effusion or tympanic membrane perforation unlikely. The positive Rinne test was consistent with a diagnosis of idiopathic sudden sensorineural hearing loss (SSNHL). DISCUSSION SSNHL is defined by hearing loss of more than 30 dB in at least 3 consecutive frequen- cies with acute onset of less than 72 hours.1,2 The most common symptoms include acute hearing loss, tinnitus, and fullness in the affected ear.1 The majority of cases of SSNHL are unilateral. The typical age of onset is in the fourth and fifth decades, occurring with equal distribution in both sexes. The annual incidence of SSNHL is 5 to 20 cases per 100,000 indi- viduals worldwide.1,2 z Etiology. Identifiable causes of SSNHL include viral infections, vascular events, co- chlear hydrops, head trauma, tumors (eg, vestibular schwannoma), and demyelinating dis- orders. Bilateral SSNHL can be seen in autoimmune diseases and rarely can be caused by medications, such as aminoglycosides or certain chemotherapy medications. However, 90% of cases of SSNHL are considered idiopathic because the etiology cannot be determined.1 z Diagnosis. The initial evaluation should include an otoscopic examination, tuning fork tests, and pure tone audiometry.1-3 Weber and Rinne tests are essential when evaluat- ing patients for unilateral hearing loss and determining the type of loss (ie, sensorineural MDEDGE.COM/FAMILYMEDICINE VOL 68, NO 6 | JULY/AUGUST 2019 | THE JOURNAL OF FAMILY PRACTICE 355 CASE REPORT vs conductive). The Weber test (ideally us- clear efficacy of corticosteroid treatment for ing a 512-Hz tuning fork) can detect either the management of idiopathic SSNHL.7,8 conductive or sensorineural hearing loss. In Because of the potential systemic adverse a normal Weber test, the patient should hear effects associated with oral corticosteroids, the vibration of the tuning fork equally in both intratympanic (IT) corticosteroids have been ears. The tuning fork will be heard in both advocated as an alternative treatment option. ears in conductive hearing loss but will only A prospective, randomized, noninferiority be heard in the unaffected hear if sensorineu- trial comparing the efficacy of oral vs IT cor- ral hearing loss is present. So, for instance, if ticosteroids for idiopathic SSNHL found IT a patient has a perforation in the right tym- corticosteroids to be noninferior to systemic panic membrane causing conductive hearing treatment.9 IT treatment also has been advo- loss in the right hear, the tuning fork would be cated as a rescue therapy for patients who do heard in both ears. If the patient has sensori- not respond to systemic treatment.10 neural hearing loss in the right ear, the tuning A combination of oral and IT cortico- fork would only be heard in the left ear. steroids was investigated in a retrospective The Rinne test compares the percep- study analyzing multiple treatment modali- tion of sound waves transmitted by air con- ties.10 Researchers first compared 122 pa- duction vs bone conduction and serves as a tients receiving one of 3 treatments: (1) IT The most rapid screen for conductive hearing loss. In a corticosteroids, (2) oral corticosteroids, and common positive Rinne test, the patient should be able (3) combination treatment (IT + oral corti- symptoms to hear the vibrating tuning fork next to the costeroids). There was no difference in hear- of sudden pinna louder than when placed against the ing recovery among any of the treatments. sensorineural mastoid bone (ie, air conduction greater than Fifty-eight patients who were refractory to hearing loss bone conduction). In a negative Rinne test, initial treatment were then included in a sec- include not only bone conduction is greater than air conduc- ond analysis in which they were divided into acute hearing tion, and a conductive hearing loss is present. those who received additional IT corticoste- loss, but also In our patient, pure tone audiometry, tym- roids (salvage treatment) vs no treatment tinnitus and panometry, and speech audiometry results (control). There was no difference in hearing fullness in the were consistent with SSNHL. recovery between the 2 groups. The authors affected ear. Magnetic resonance imaging (MRI) of concluded that IT corticosteroids were as ef- the brain and brainstem with gadolinium fective as oral treatment and that salvage IT contrast can reveal vascular events (throm- treatment did not add any benefit.10 botic or hemorrhagic), demyelinating dis- The American Academy of Otolaryngology- orders, or retrocochlear lesions such as Head and Neck Surgery (AAO-HNS) recently vestibular schwannoma and is indicated published guidelines on the diagnosis and in all cases of suspected SSNHL.4,5 An MRI management of SSNHL.11 The guidelines should be obtained within 6 weeks of the ini- state that IT steroids should be considered tial presentation—even if the patient’s hear- in patients who cannot tolerate oral steroids, ing returns to normal after treatment.4 such as patients with diabetes. It is important z Treatment and management. The to note, however, that the high cost of IT treat- current standard of care for treatment of ment (~$2000 for dexamethasone or methyl- idiopathic SSNHL is systemic steroids.1,2 prednisolone vs < $10 for oral prednisolone) Although the gold standard currently is oral is an issue that needs to be considered as prednisolone or methylprednisolone (1 mg/ health care costs continue to rise. kg/d for 10 to 14 days with a taper,1,2 the evi- z Antivirals. Because an underlying viral dence for this regimen stems from a single etiology has been speculated as a potential placebo-controlled trial (N = 67) that dem- cause of idiopathic SSNHL, antiviral agents onstrated greater improvement in the ste- such as valacyclovir or famciclovir also are roid group compared with the placebo group potential treatment agents.12 Antiviral medi- (61% vs 32%).6 A Cochrane review and other cations have minimal adverse effects and systematic analyses have not demonstrated are relatively inexpensive, but the benefits 356 THE JOURNAL OF FAMILY PRACTICE | JULY/AUGUST 2019 | VOL 68, NO 6 have not yet been proven in randomized con- THE TAKEAWAY trolled trials, and they currently are not en- SSNHL is a medical emergency that requires dorsed by the AAO-HNS in their guidelines prompt recognition and diagnosis. The steps for the management of SSNHL.11 in evaluating sudden hearing loss include: z Spontaneous recovery occurs in up (1) appropriate history and physical exami- to 40% of patients with idiopathic SSNHL. As nation (eg, otoscopic examination, tuning many as 65% of those who experience recov- fork tests), (2) urgent audiometry to confirm ery do so within 2 weeks of the onset of symp- hearing loss, (3) immediate referral to an oto- toms, regardless of treatment.1,2 Treatment laryngologist for further testing (eg, tympa- beyond 2 weeks after onset of symptoms is nometry, blood tests, MRI), and (4) initiation unlikely to be of any benefit, although some of treatment. otolaryngologists will treat for up to 6 weeks If a specific etiology is identified (eg, ves- after the onset of hearing loss. tibular schwannoma), the patient should be re- A substantial number of patients with ferred to a specialist for appropriate treatment. SSNHL may not recover. Management of If there is no identifiable cause (idiopathic these patients begins with referral to an ap- SSNHL), the patient should be treated with oral propriate specialist to initiate counseling and/or intratympanic steroids. Patients who and lifestyle changes. Depending on the do not recover following treatment should be degree of hearing loss, audiologic rehabili- offered audiologic rehabilitation. JFP Ninety percent tation may include use of a traditional or of sudden CORRESPONDENCE bone-anchored hearing aid or a frequency- Sergio Huerta, MD, UT Southwestern Medical Center, 4500 sensorineural modulation system.1,2,11 Tinnitus retraining S Lancaster Road #112L, Dallas, TX 75216; Sergio.Huerta@ hearing loss UTSouthwestern.edu therapy might be of benefit for patients with cases are persistent tinnitus.11 considered z Our patient. After a discussion of his idiopathic.