Sudden profound hearing loss in the only hearing ear
A 47-year-old right-handed woman presents to the emergency department with a 4-day history of sudden right-sided hearing loss and constant right tinnitus. She reports no vertigo, no otorrhea, and no recent noise exposure. Otoscopy is normal. She has a left cochlear implant from age 12 after meningitis-related profound deafness; the implant is functioning well and she relies on it for communication. Audiometry confirms a right profound sensorineural hearing loss (PTA 95 dB, word recognition score 0%) and left ear is unaided profound (implant-assisted PTA 25 dB). MRI of the internal auditory canals with gadolinium is normal. She is otherwise healthy, takes no medications, and has no contraindications to steroids. The on-call resident proposes oral prednisolone 1 mg/kg for 7 days and discharge. She asks whether 'the other ear can be saved' and whether she should stop using her implant.
1. Is the resident's plan adequate? Justify your answer with regard to prognosis and treatment escalation.
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No. A 4-day history of profound sudden sensorineural hearing loss (PTA 95 dB, WRS 0%) in the only functionally hearing ear is an otologic emergency. Oral prednisolone 1 mg/kg is a reasonable first step, but the prognosis with profound loss and 0% word recognition is poor, and the patient is functionally dependent on her contralateral cochlear implant. I would add same-day intratympanic dexamethasone (e.g., 24 mg/mL) to the systemic steroid, because combination therapy may improve salvage rates in severe/profound cases, although evidence is strongest for salvage after systemic failure. I would not discharge her without a clear follow-up in 1–2 weeks and repeat audiometry. MRI is normal, which rules out retrocochlear pathology but does not change acute management. She should continue using her implant; the implant is in the contralateral ear and is not at risk from the right-ear process. I would also check for syphilis, autoimmune markers if indicated, and consider vestibular assessment. Hyperbaric oxygen can be considered within 2 weeks in selected centres. The key is to escalate, not observe.
2. At 3-month follow-up, audiogram shows no recovery (PTA 100 dB, WRS 0%). What are her options, and how do you counsel her about cochlear implantation in the setting of a contralateral implant?
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With no recovery at 3 months (PTA 100 dB, WRS 0%), the right ear is a candidate for cochlear implantation. She already has a functioning left implant, so the discussion is about sequential bilateral cochlear implantation versus relying on the single implant. Bilateral implants can improve sound localization, speech perception in noise, and quality of life, but the evidence for sequential implantation in adults is variable, and she will need rehabilitation. I would assess her vestibular function, imaging (MRI to confirm cochlear patency), and expectations. The risks include anaesthesia, device failure, facial nerve injury, and meningitis (vaccination required). Because she communicates well with the left implant, this is an elective decision; I would not rush her. I would also discuss that if the left implant fails in the future, the right ear would be her only hearing ear, which strengthens the case for a second implant. She should continue implant use and consider a trial of a contralateral hearing aid (unlikely to help). Shared decision-making with audiology and psychology is essential.