Creutzfeldt-Jakob disease presenting with bilateral hearing loss:A case report

BACKGROUND Sporadic Creutzfeldt-Jakob disease(s CJD)is a prion disease characterized as a fatal transmissible neurodegenerative disorder.Dizziness is often the first presenting symptom of s CJD,but hearing loss as an early manifestation is very rare.CASE SUMMARY A 76-year-old man presented with bilateral sudden hearing impairment and dizziness for 10 d.He was taking medications for hypertension and diabetes.He denied any difficulty with activities of daily living or hearing impairment before the onset of symptoms.Pure tone audiometry showed bilateral severe hearing impairment.Brain magnetic resonance imaging(MRI)and laboratory tests were within normal limits.Given his diagnosis of sudden sensory hearing loss,the patient received corticosteroid treatment but it was ineffective.Two weeks later,he complained of aggravated gait impairment,disorientation,and cognitive impairment.Repeat brain MRI showed diffuse cortical high signal intensities on diffusion-weighted imaging.In cerebrospinal fluid analysis,the real-time quaking-induced conversion assay was positive,and 14-3-3 protein was detected in the by western blotting.Considering all the data,we diagnosed probable s CJD,and the patient’s symptoms rapidly progressed into akinetic mutism.CONCLUSION For patients with abrupt bilateral hearing impairment,especially in the elderly,various differential diagnoses,including s CJD,should be considered.

Comparison between Bilateral and Unilateral Sudden Sensorineural Hearing Loss

Background：Bilateral sudden sensorineural hearing loss （BSSHL） is rare and assumed to be a different clinical entity compared to unilateral SSHL （USSHL）.This study examined the differences between the idiopathic BSSHL and USSHL.Methods：Forty-six sequential BSSHL patients （Se-BSSHL） and 68 simultaneous BSSHL （Si-BSSHL） were consecutively admitted between June 2008 and December 2015.Two sets of patients served as control groups：（1） USSHL patients with healthy contralateral ear and （2） USSHL patients with contralateral preexisting hearing loss （USSHLwCHL）.We retrospectively analyzed differences among four cohorts using analysis of variance,Kruskal-Wallis test,Welch＇s t-test,and Chi-square test as appropriate before and after propensity score matching （PSM） based on age,gender,and body mass index （BMI）.Results：The prevalence of idiopathic BSSHL was 8.6% （114/1329） among the total SSHL patients.In the total cohort,USSHL patients tended to be younger,female,and tended to have lower BMI,renal parameters,and total cholesterol in addition to higher high-density lipoprotein compared to the other three groups.Most routine blood indicators,some coagulation markers,and immunoglobulin M （H =13.4,P =0.004） were significantly different among the study groups.After PSM,the major significant differences were found in audiometric characteristics.Si-BSSHL and Se-BSSHL patients demonstrated similar hearing thresholds as USSHL but were significantly better than the USSHLwCHL patients across most frequencies before and after treatment （H =30.0,P 〈 0.001 for initial hearing and H =12.0,P =0.007 for final hearing）.Moreover,the BSSHL patients showed different hearing loss distribution patterns （more descending type,x2 =33.8,P =0.001） with less hearing gain （H =17.5,P 〈 0.001） compared to the USSHL patients.Conclusions：Idiopathic BSSHL is a relatively rare subtype of SSHL with a higher rate of descending audiogram type and inferior hearing outcome rather than being classified as a completely different disease entity compared to USSHL.