presbycusis
Summary
Presbycusis is age-related, progressive, bilateral, symmetric sensorineural hearing loss that begins with high-frequency sounds. It is the most common cause of hearing loss in adults and results from cumulative degeneration of cochlear hair cells at the basal turn. Patients typically have trouble understanding speech in noisy environments.
Detail
**Definition:** Age-related sensorineural hearing loss (SNHL), usually bilateral and symmetric, gradually progressive, and affecting high frequencies first.
**Pathophysiology:** - Loss of cochlear hair cells (especially outer hair cells) at the basal turn, which encodes high frequencies. This is why high frequencies are lost first. - Contributing factors: cumulative noise exposure, oxidative stress and mitochondrial damage, atrophy of the stria vascularis (metabolic presbycusis), spiral ganglion neuron loss (neural presbycusis), and reduced basilar membrane compliance (cochlear conductive presbycusis). - Risk factors: older age, male sex, genetics, smoking, diabetes, hypertension and atherosclerosis, ototoxic drugs (aminoglycosides, loop diuretics, cisplatin, salicylates), and chronic noise exposure. - Hair cells do not regenerate in humans, so the loss is permanent.
**Clinical features:** - Gradual, bilateral hearing loss, typically after age 60. - Difficulty understanding speech, especially in noisy settings (the "cocktail party" problem). Consonants (s, f, th, k) are lost first, so patients say "I can hear but can't understand." - Poor speech discrimination out of proportion to pure-tone loss, along with loudness recruitment. - Tinnitus is common.
**Diagnosis:** - Weber: no lateralization (symmetric loss). - Rinne: air conduction > bone conduction bilaterally (normal pattern, as in all SNHL). - Audiometry: bilateral symmetric high-frequency SNHL with a sloping downward audiogram. Speech discrimination scores are reduced. - Asymmetric hearing loss should prompt evaluation for a retrocochlear lesion (vestibular schwannoma) with MRI.
**Management:** - Hearing aids are first-line. - Cochlear implants for severe to profound loss. - Assistive listening devices, communication strategies, and avoidance of noise and ototoxins. - Untreated hearing loss is associated with social isolation, depression, and cognitive decline or dementia.
**Differentiating high-yield points:** - Noise-induced hearing loss: a notch at 4000 Hz on audiogram. - Otosclerosis: conductive loss with a normal tympanic membrane, a negative Rinne (BC > AC), and a Carhart notch at 2000 Hz. - Ménière disease: episodic vertigo, tinnitus, aural fullness, and low-frequency fluctuating SNHL. - Vestibular schwannoma: unilateral SNHL and tinnitus, with bilateral tumors in NF2. - Sudden SNHL: loss over <72 hours, treated with urgent steroids.
Sources
- First Aid for the USMLE Step 1 (Ear/ENT and Neurology sections)
- Robbins & Cotran Pathologic Basis of Disease
- Harrison's Principles of Internal Medicine (Disorders of Hearing)
- Cummings Otolaryngology: Head and Neck Surgery
- UpToDate: Presbycusis (age-related hearing loss)
- Pathoma / Boards and Beyond (ENT)
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