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Causes of sensorineural hearing loss: presbycusis, sudden loss, Ménière's, asymmetric loss and genetic causes
Most sensorineural losses in adults are age-related, but some patterns demand immediate action: sudden loss (an emergency), unilateral or asymmetric loss with tinnitus (suspect retrocochlear) and fluctuating loss with vertigo (Ménière's). In children the common genetic cause is GJB2.
Why it matters in the exam
Presbycusis is the leading sensorineural diagnosis in adults: bilateral, symmetrical, sloping toward the highs, with marked trouble in noise; it is permanent and progressive, not fluctuating. Sudden loss is a drop of at least 30 dB at three adjacent frequencies within 72 hours; in most cases it is idiopathic, and it is treated urgently with steroids (oral or into the middle ear) and investigated with MRI to rule out a vestibular schwannoma. A unilateral or asymmetric loss with one-sided tinnitus, especially with poor word recognition on that side, requires referral for contrast MRI.
In Ménière's disease (endolymphatic hydrops) there are attacks of spinning vertigo lasting 20 minutes to 12 hours, a documented low-to-mid-frequency sensorineural loss, and fluctuating tinnitus and fullness. The early low-frequency loss arises because the apical cochlea is wide and floppy and is hit first by the pressure. The disease usually starts in one ear, and only a minority become bilateral over the years; hearing fluctuates. Middle-ear effusion, an enlarged vestibular aqueduct and autoimmune disease also make hearing vary. Genetics: GJB2 (connexin 26) — the common cause of non-syndromic recessive loss, usually congenital and stable; Usher — congenital loss with retinitis pigmentosa that shows as night blindness in adolescence; Pendred — enlarged vestibular aqueduct and goitre; Alport — kidney disease; Jervell and Lange-Nielsen — long QT.
Key points
- Presbycusis — the common adult cause, symmetrical, sloping.
- Sudden: 30 dB, 3 frequencies, 72 hours; steroids and MRI.
- Asymmetric with tinnitus: MRI.
- Ménière's: vertigo, lows, fluctuation, fullness.
- GJB2 is common; Usher with retinitis pigmentosa.
Summary table
| Cause | Pattern | What to do or remember |
|---|---|---|
| Presbycusis | Bilateral, symmetrical, sloping to the highs | The common cause in adults; not fluctuating |
| Sudden loss | At least 30 dB at 3 adjacent frequencies within 72 hours | Most cases idiopathic; steroids quickly; MRI |
| Asymmetric with tinnitus | One-sided, poor recognition on that side | Contrast MRI to rule out schwannoma |
| Ménière's | Low-mid, fluctuating, vertigo 20 minutes to 12 hours | Hydrops; ECochG; usually one-sided at first |
| GJB2 (connexin 26) | Congenital, non-syndromic, recessive | The common genetic cause; good implant results |
| Usher | Congenital with retinitis pigmentosa | Night blindness in adolescence |
| Other syndromes | Pendred, Alport, Jervell and Lange-Nielsen, Waardenburg | Goitre, kidneys, long QT, pigment |
A free exam question
Which of the following can be characterized by fluctuating hearing?
- A. Presbycusis
- B. Noise-induced hearing loss
- C. Otosclerosis
- D. Meniere's disease
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Sources
- Chandrasekhar et al., Otolaryngology–Head and Neck Surgery, 2019 — Clinical Practice Guideline: Sudden Hearing Loss (update)
- Lopez-Escamez et al., Journal of Vestibular Research, 2015 — Diagnostic criteria for Menière's disease (Bárány Society)
- Smith, Shearer, Hildebrand & Van Camp, GeneReviews, updated 2023 — Hereditary hearing loss and deafness overview
Sources are given for further reading; the text is written afresh and not copied from them.
Reviewed by the Examqo AI Professor · 2026-11-02. The text may contain errors; when in doubt, check the source.