Sudden hearing loss in 50 y.o. man

This 50-year-old man presents with steroid-responsive sensorineural hearing loss (SNHL) on a background of reduced R auditory acuity following shingles aged 12. Background mild allergic rhinoconjunctivitis; no sinus or Eustachian tube abnormality. Coeliac gene positivity with gluten-related gut symptoms - due for review by GE to address the possibility of a seronegative form of coeliac. Daughter has proven CD. FHx: remarkably similar syndrome of sudden hearing loss affecting his mother (R>L) with vertigo, tinnitus and nausea. A paternal uncle had acoustic neuroma, requiring surgery. HPI: Starting in early 2021, he has developed episodes of ‘fullness’ in L ear with rapid onset, lasting a few hours at a time, with reduced auditory acuity in L ear + worsened auditory acuity in compromised R ear associated with tinnitus (‘white noise’) but no vertigo. Strangely, first few episodes seemed responsive to vasoconstrictive therapy (pseudoephedrine) – returned to usual baseline between episodes. By Dec 2021, no longer responsive to vasoconstrictor. Audiometry revealed SNHL in L ear, mixed conductive/SNHL in R ear. Hearing services suggested purchasing hearing aids. Trial of steroids (60mg/d then rapid wean over 5 days, started empirically by GP) normalised auditory acuity in L ear – 6 successfully-treated episodes occurred throughout 2022. Experienced mild ocular inflammation starting ~ August 2022 – ophthalmologist diagnosed marginal keratitis and blepharitis Rx: periodic FML therapy. Subsequent breakthrough hearing loss Dec 2022 whilst in midst of a course prednisolone therapy at stressful time (mother’s unexpected passing) – had weaned from 60mg prednisolone to 25mg/d. Returned to 60mg dose, hearing again normalised, but further relapse 2 days after completing a more protracted course (moving from prednisolone 60mg/d down to zero over 3 weeks). Reviewed by Sydney ENT specialist. Otoscopy normal. No URTI, vascular disease, other cranial neuropathies or focal neurological signs. SNHL in L ear. No sinus or Eustachian tube problems. MRI revealed no retrocochlear, cerebellopontine or acoustic neuroma problems. Autoimmune screen negative. ESR, CRP, FBC, TSH normal. Recent further episode 60dB hearing loss, involving higher more than lower frequencies – partially resolving with return from 25mg to 60mg/d. I feel this all fits with idiopathic SNHL and am adding MTX as steroid-sparing agent (consider cyclophosphamide if this fails). DDx = Cogan’s, but inflammatory markers normal. Any further thoughts, suggestions?

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