What is the approach to a patient who can hear a heartbeat in her ears, associated with decreased hearing?

Approach to Pulsatile Tinnitus with Hearing Loss

This patient requires immediate comprehensive audiologic evaluation and vascular imaging, as the combination of pulsatile tinnitus (hearing heartbeat in ears) with hearing loss represents a red flag for potentially serious underlying vascular or structural pathology that demands urgent workup. 1

Initial Clinical Assessment

Targeted History and Physical Examination

• Characterize the tinnitus precisely: Confirm it is truly pulsatile (synchronous with heartbeat), determine laterality (unilateral vs bilateral), assess duration, and identify any positional changes 1
• Perform otoscopic examination to identify retrotympanic vascular masses (paragangliomas, aberrant vessels), which appear as reddish-blue masses behind the tympanic membrane 1
• Auscultate the neck, periauricular region, and temporal area for objective bruits that may indicate vascular abnormalities like arteriovenous malformations or carotid stenosis 2
• Conduct cranial nerve examination focusing on CN VIII and other focal neurological deficits that would mandate immediate imaging 1
• Assess for associated symptoms: vertigo, otalgia, otorrhea, or neurological symptoms that suggest specific etiologies 2

Critical Red Flags Requiring Urgent Evaluation

The combination of pulsatile tinnitus with hearing loss automatically triggers the need for imaging, as this represents one of the specific indications where imaging is strongly recommended rather than contraindicated 1

Mandatory Audiologic Evaluation

Obtain comprehensive audiologic examination immediately (preferably within 4 weeks), as this patient has both unilateral/asymmetric features and hearing loss 1

Components of Audiologic Testing

• Pure tone audiometry with air and bone conduction thresholds at 250-8000 Hz to characterize the type and degree of hearing loss 1
• Speech audiometry including speech recognition thresholds and word recognition scores to assess functional hearing 1
• Tympanometry and acoustic reflex testing to differentiate conductive from sensorineural components 1
• Otoacoustic emissions (OAEs) may help distinguish sensory from neural hearing loss patterns 1

The audiologic evaluation will determine if the hearing loss is unilateral, asymmetric, or associated with specific patterns that guide further workup 1

Imaging Strategy

Proceed with vascular imaging as first-line evaluation given the pulsatile nature of tinnitus combined with hearing loss 1

Recommended Imaging Approach

• CT temporal bone with CT angiography (CTA) of head and neck is the preferred initial study, as it can be performed as a single mixed arterial-venous phase examination (20-25 seconds post-contrast) to evaluate both vascular anatomy and temporal bone structures with lower total radiation 1
• High-resolution temporal bone CT reconstructions can be created from CTA source images to identify paragangliomas, aberrant vessels (high-riding jugular bulb, dehiscent carotid artery), semicircular canal dehiscence, or other bony abnormalities 1
• MRI brain with contrast may be added if vestibular schwannoma or other intracranial pathology is suspected based on asymmetric sensorineural hearing loss patterns 1

Key Differential Diagnoses to Evaluate

• Vascular masses: Glomus tympanicum or jugulare (paragangliomas) 1
• Vascular anomalies: Aberrant internal carotid artery, high-riding or dehiscent jugular bulb, persistent stapedial artery 1
• Vascular malformations: Arteriovenous malformations or dural arteriovenous fistulas 1
• Idiopathic intracranial hypertension: Can present with pulsatile tinnitus and may affect hearing 1
• Superior semicircular canal dehiscence: Can cause pulsatile tinnitus and conductive hearing loss 1

Common Pitfalls to Avoid

• Do not dismiss pulsatile tinnitus as benign primary tinnitus: Unlike nonpulsatile tinnitus, pulsatile tinnitus has identifiable causes in the majority of cases and requires imaging workup 1
• Do not delay imaging while waiting for audiometry: These evaluations should proceed in parallel given the potential for serious underlying pathology 1
• Do not obtain imaging without otoscopic examination first: A retrotympanic mass visible on otoscopy changes the differential diagnosis and imaging protocol 1
• Do not rely on patient self-report of hearing status: Objective audiometry is essential as patients cannot accurately characterize their hearing loss type or severity 1

Management Based on Findings

If Vascular Pathology Identified

• Refer to appropriate specialist (interventional neuroradiology, neurosurgery, or otolaryngology) for consideration of endovascular embolization, surgical resection, or radiation therapy depending on the specific lesion 3

If Hearing Loss Confirmed Without Treatable Vascular Cause

• Recommend hearing aid evaluation if sensorineural hearing loss is documented, as amplification improves quality of life 1, 4
• Provide education and counseling about tinnitus management strategies 1, 4
• Consider cognitive behavioral therapy to reduce tinnitus-related distress, as this is the only treatment proven to improve quality of life in persistent bothersome tinnitus 1, 4
• Offer sound therapy as an option for auditory masking 1, 4

Psychological Assessment

Screen for severe anxiety, depression, or suicidal ideation related to symptoms, as these require prompt identification and intervention 1, 3