Diagnosis and Management of Unilateral Sensorineural Hearing Loss with Unsteadiness and Essential Tremor
The primary concern is vestibular schwannoma (acoustic neuroma), which must be excluded with urgent MRI brain with internal auditory canal sequences using gadolinium contrast, while simultaneously initiating systemic corticosteroid therapy if the hearing loss is sudden in onset. 1, 2
Differential Diagnosis
The combination of unilateral sensorineural hearing loss and unsteadiness is the classic presentation of vestibular schwannoma, occurring in 94% and 17-75% of cases respectively. 1 The essential tremor is likely coincidental and unrelated to the audiovestibular symptoms, as it is not a typical feature of retrocochlear pathology.
Key Diagnostic Considerations:
- Vestibular schwannoma is the most common cerebellopontine angle tumor (>80% of cases) and presents with this exact symptom constellation 1
- Brainstem/cerebellar stroke (particularly AICA territory) can present identically and requires urgent exclusion 2, 3
- Idiopathic sudden sensorineural hearing loss (ISSNHL) with vestibular involvement if onset was acute 1, 2
Immediate Diagnostic Workup
Audiometric Testing
Perform comprehensive audiometry to confirm sensorineural hearing loss (≥30 dB loss at 3 consecutive frequencies) and distinguish from conductive causes. 1 This is a strong recommendation from the American Academy of Otolaryngology-Head and Neck Surgery.
Neuroimaging - Critical Step
MRI brain with internal auditory canal sequences (with gadolinium contrast) is mandatory to evaluate for retrocochlear pathology. 1, 2 This is the gold standard imaging modality and superior to CT for detecting:
- Vestibular schwannomas (even small intracanalicular tumors) 1
- AICA territory stroke 2, 3
- Demyelinating disease 2
Red Flags Requiring Urgent Neuroimaging:
- Severe vertigo/unsteadiness lasting >24 hours with hearing loss 2
- Downbeating or gaze-evoked nystagmus 1, 2
- Focal neurologic symptoms (facial numbness, diplopia, dysarthria, hemiataxia) 1, 3
- Severe imbalance with inability to walk 2
Treatment Algorithm
If Hearing Loss is Sudden in Onset (<2 weeks):
Initiate oral corticosteroids immediately while awaiting MRI results:
- Prednisone 1 mg/kg/day (maximum 60 mg daily) for 7-14 days with or without taper 2
- This is the only evidence-based treatment for sudden sensorineural hearing loss 2
- Treatment should begin within 2 weeks of symptom onset for optimal outcomes 2
Intratympanic dexamethasone can be offered as:
- Initial therapy if oral steroids are contraindicated 2
- Salvage treatment if hearing recovery is incomplete after 2-4 weeks of initial corticosteroid therapy 1, 2
Symptomatic Management of Unsteadiness:
Short-term vestibular suppressants for the first 2-3 days only:
- Meclizine 25 mg TID, or
- Dimenhydrinate 50 mg q6h, or
- Ondansetron 4-8 mg q8h 2
Critical caveat: Do not use vestibular suppressants beyond 72 hours as they impair central vestibular compensation. 2
If Vestibular Schwannoma is Confirmed:
Management depends on tumor size and patient factors, with options including:
- Observation with serial MRI (for small, asymptomatic tumors)
- Microsurgical resection
- Stereotactic radiosurgery 1
The essential tremor should be managed separately and is not related to the audiovestibular pathology.
Follow-up Strategy
- Audiometric reassessment at 7 days to document response to corticosteroids (if sudden hearing loss) 2, 4
- Follow-up audiometry within 6 months to document final hearing status 1, 5
- MRI results review to determine definitive diagnosis and guide long-term management 1
If Hearing Does Not Recover:
Counsel about amplification options including:
- Conventional hearing aids if usable residual hearing remains 5
- CROS hearing aids for single-sided deafness 5
- Osseointegrated bone conduction devices 5
- Cochlear implantation in select cases of profound loss 5
Prognostic Considerations
The presence of unsteadiness/vertigo at onset significantly worsens prognosis for hearing recovery, with substantially lower complete recovery rates compared to sudden hearing loss without vestibular symptoms. 2 Approximately one-third to two-thirds of sudden sensorineural hearing loss patients recover some hearing within 2 weeks, but those with vertigo have poorer outcomes. 2, 6
Common pitfall: Assuming idiopathic sudden hearing loss without obtaining MRI imaging—4-6% of unilateral vestibular schwannomas can present with sudden hearing loss, and missing this diagnosis has significant implications for morbidity. 1, 7